ENT Basics Corectat 2

Sever Pop

ENT BASICS

Coperta: Patricia Pușcaș

Editură acreditată CNCSIS (24)

© Sever Pop, 2011

ISBN

Descrierea CIP este disponibilă la Biblioteca Naţională a României

Sever Pop

ENT BASICS

Casa Cărţii de ŞtiinţăCluj-Napoca, 2011

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CONTENTS

I. EAR

1. EAR BASICS ............................................................................. 111.1. Clinical anatomy ................................................................ 111.2. Physiology of the ear .......................................................... 151.3. Symptoms of ear diseases .................................................. 181.4. Clinical examination .......................................................... 21

2. DISEASES OF THE EXTERNAL EAR .................................................. 312.1. Diseases of the auricle ........................................................ 312.2. Diseases of the external auditory canal .............................. 34

3. DISEASES OF THE MIDDLE EAR ..................................................... 423.1. Trauma ............................................................................... 423.2. Bullous myringitis .............................................................. 473.3. Acute suppurative otitis media (AOM) ............................. 483.4. Otitis media with eff usion (OME) .................................... 513.5. Chronic suppurative otitis media ....................................... 533.6. Otosclerosis ........................................................................ 60

4. DISEASES OF THE INNER EAR ....................................................... 634.1. Sudden sensorineural hearing loss (SSHL) ....................... 634.2. Progressive sensorineural hearing loss ................................ 654.3. Inner ear infections ............................................................ 714.4. Management of hearing impairment ................................. 74

5. PERIPHERAL VESTIBULAR SYNDROME ............................................. 785.1. Vertigo ............................................................................... 785.2. Examination of the vestibular function .............................. 795.3. Vertigo: clinical syndromes ................................................ 82

6. REFERENCES ............................................................................. 91

II. NOSE

1. NOSE AND PARANASAL SINUSES BASICS......................................... 951.1. Clinical anatomy ................................................................ 951.2. Physiology of the nose ..................................................... 1011.3. Symptoms of nasal diseases .............................................. 1021.4. Clinical examination ........................................................ 1041.5. Clinical investigations ...................................................... 108

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2. DISEASES OF THE EXTERNAL NOSE AND NASAL VESTIBULE ...............1122.1. Acute nasal infections ...................................................... 1122.2. Trauma ............................................................................. 1142.3. Dermatological conditions ............................................... 1152.4. Tumors ............................................................................. 116

3. RHINITIS ................................................................................1203.1. Acute rhinitis ................................................................... 1203.2. Chronic specifi c rhinitis ................................................... 1223.3. Atrophic rhinitis .............................................................. 1273.4. Nasal manifestations in systemic diseases ........................ 1283.5. Allergic rhinitis ................................................................ 1313.6. Non-allergic perennial rhinitis ......................................... 1343.7. Nasal polyposis ................................................................ 137

4. RHINOSINUSITIS ......................................................................1404.1. Acute rhinosinusitis ......................................................... 1404.2. Chronic rhinosinusitis ...................................................... 1434.3. Fungal rhinosinusitis ........................................................ 145

5. EPISTAXIS ..............................................................................1475.1. Defi nition. Classifi cation ................................................. 1475.2. Aetiology ......................................................................... 1475.3. Management .................................................................... 148

6. TUMORS OF THE NOSE AND PARANASAL SINUSES ...........................1506.1. Benign tumors ................................................................. 1506.2. Malignant tumors ............................................................ 150

7. REFERENCES ...........................................................................154

III. PHARYNX

1. PHARYNX BASICS ....................................................................1591.1. Clinical anatomy .............................................................. 1591.2. Phisiology ........................................................................ 1641.3. Symptoms of pharyngeal diseases .................................... 1651.4. Clinical examination ........................................................ 166

2. ACUTE AND CHRONIC PHARYNGEAL INFECTION .............................1702.1. Acute viral pharyngitis ..................................................... 1702.2. Acute bacterial pharyngitis (Follicular tonsillitis) ............ 1712.3. Acute adenoiditis ............................................................. 1732.4. Suppurative complications of acute bacterial pharyngitis . 1742.5. Specifi c acute bacterial pharyngitis .................................. 1772.6. Specifi c viral pharyngitis .................................................. 1802.7. Pharyngeal manifestations in blood disorders .................. 1822.8. Chronic non-specifi c conditions ...................................... 1832.9. Chronic specifi c pharyngitis............................................. 188

3. TUMOURS OF THE PHARYNX ......................................................193

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3.1. Tumors of the nasopharynx .............................................. 1933.2. Tumors of the oropharynx ................................................ 1983.3. Tumors of the hypopharynx ............................................. 202

4. NEUROLOGICAL DISORDERS OF THE PHARYNX ..............................2044.1. Sensory disorders ............................................................. 2044.2. Motor disorders ............................................................... 205

5. FOREIGN BODIES IN THE PHARYNX ..............................................2076. REFERENCES ...........................................................................208

IV. LARYNX

1. LARYNX BASICS .......................................................................2111.1. Clinical anatomy .............................................................. 2111.2. Phisiology ........................................................................ 2161.3. Symptoms of laryngeal diseases ....................................... 2171.4. Clinical examination ........................................................ 218

2. INFLAMMATORY DISEASES .........................................................2232.1. Acute laryngitis ................................................................ 2232.2. Acute edematous subglottic laryngitis.............................. 2242.3. Acute epiglottitis .............................................................. 2252.4. Laryngeal diphtheria ........................................................ 2252.5. Acute laryngeal edema ..................................................... 2262.6. Chronic nonspecifi c laryngitis.......................................... 2272.7. Chronic specifi c laryngitis ................................................ 2292.8. Pseudo-myxomatous laryngitis (Reinke’s edema) ............ 2322.9. Vocal fold polyp ............................................................... 2332.10. Vocal nodules ................................................................. 234

3. TUMORS OF THE LARYNX ..........................................................2363.1. Benign Tumors ................................................................ 2363.2. Malignant tumors ............................................................ 237

4. LARYNGEAL NERVE DISORDERS ..................................................2434.1. Laryngeal nerve palsy ....................................................... 243

5. TRACHEOSTOMY ......................................................................2475.1. Defi nition. History .......................................................... 2475.2. Indications ....................................................................... 2475.3. Techniques ....................................................................... 2485.4. Postoperative management .............................................. 2495.5. Complications .................................................................. 249

6. LARYNGEAL TRAUMA ...............................................................2516.1. Defi nition. Aetiology ....................................................... 2516.2. Symptoms ........................................................................ 2526.3. Diagnosis ......................................................................... 2526.4. Treatment ......................................................................... 252

7. FOREIGN BODIES IN THE LARYNX ................................................254

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7.1. Defi nition. Aetiology ....................................................... 2547.2. Symptoms ........................................................................ 2557.3. Diagnosis ......................................................................... 2557.4. Treatment ......................................................................... 255

8. REFERENCES ...........................................................................256

V. SALIVARY GLAND BASICS

1. SALIVARY GLAND BASICS ..........................................................2591.1. Clinical anatomy .............................................................. 2591.2. Physiology ........................................................................ 2621.3. Disorders of salivary secretion .......................................... 2631.4. Clinical examination ........................................................ 264

2. INFLAMMATORY DISORDERS ......................................................2672.1. Infectious infl ammatory disorders .................................... 2672.2. Noninfectious infl ammatory disorders ............................. 271

3. OBSTRUCTIVE DISORDERS .........................................................2743.1. Sialolithiasis ..................................................................... 2743.2. Other obstructive disorders .............................................. 275

4. SALIVARY GLANDS TUMORS ......................................................2764.1. Benign tumors ................................................................. 2764.2. Malignant tumors ............................................................ 280

5. REFERENCES ...........................................................................284

VI. NECK BASICS

1. NECK BASICS ..........................................................................2871.1. Surgical anatomy of the neck ........................................... 2871.2. Clinical examination ........................................................ 293

2. NECK MASSES .........................................................................2962.1. Congenital neck masses ................................................... 2962.2. Infl ammatory/infectious neck masses .............................. 3012.3. Benign tumors ................................................................. 3052.4. Malignant tumors ............................................................ 310

3. REFERENCES ...........................................................................318

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I. EAR

1. EAR BASICS

1.1. Clinical anatomy

1.1.1. External ear

Th e external ear comprises the auricle (pinna) and the external auditory canal (meatus). Th e auricle is composed of cartilage, covered by perichondrium and skin. Th e length of the external auditory canal is approximately 2.5 cm. In adults the canal is not straight, there is an italic S-shaped angulation. Th at’s why a certain amount of upward traction of the auricle is required in order to expose the eardrum during otoscopy. Th e external auditory canal is covered by skin and in the outer cartilaginous portion contains hair follicles and glands which secrete wax. Th e epithelium lining the canal is continued onto the surface of the eardrum. Th e narrowest part of the canal (isthmus) is located between the fi brocartilaginous and the bony canal. In the bony part, the skin is thinner and closely adherent to the periosteum and does not have hair follicles and ceruminous glands. Th at’s why furuncles occur only at the level of the outer cartilaginous meatus1. Th e sensory innervation is supplied by the auriculo-temporal branch of the Vth cranial nerve, the vagus nerve, the facial nerve and the cervical plexus.

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1.1.2. Middle ear and mastoid

1.1.2.1. Tympanic membrane:

It consists of three layers: an outer epithelial or epidermal layer, a middle layer of elastic fi brous tissue (radial and circular fi bers) and an inner layer of mucous membrane. Th e eardrum is inserted into a bony groove (tympanic sulcus) by the Gerlach’s ligament (annulus). It is slightly oval in shape, forming an angle of about 55° with the fl oor of the external auditory canal2. Th e most important anatomical landmarks which are visible at otoscopy are:

Th e handle of the malleus and the lateral or short process • of the malleusTh e Gerlach’s ligament (• annulus)Th e anterior and posterior malleo-tympanic folds • An anteroinferior light refl ex: the Politzer’s triangle (cone • of light)

Th e anterior and posterior malleo-tympanic folds divide the eardrum into a superior part called Shrapnell’s membrane (pars fl accida) and an inferior one called pars tensa.

1.1.2.2. Middle ear ossicles:

Th e ossicular chain consists of three ossicles: malleus, incus and stapes. Th e malleus is the largest one, measuring up to 9 mm in length. It comprises a head, a neck and a handle or manubrium, which is embedded in the eardrum. Th e incus has a body and two processes. Th e stapes footplate articulates with the oval window by the annular ligament.

1.1.2.3. Middle ear cavity:

Th e middle ear cavity is divided into three portions: the attic, the meso-tympanum and the hypo-tympanum. Th e middle ear extends beyond the limits of the eardrum. At the level of the anterior wall we have the eustachian tube opening. Th e posterior wall presents the communication with the mastoid antrum. Th e external wall consists of the eardrum and the outer bony wall. Th e most important anatomical landmarks

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Figure 1. External, middle and inner ear:

1 - External auditory canal; 2 - Eardrum; 3 - Middle ear cavity; 4 - Round window; 5 - Eustachian tube; 6 - Malleus; 7 - Incus; 8 - Stapes; 9 - Semicircular canals; 10 - Vestibular nerve; 11 - Cochlear nerve; 12 - Cochlea

Figure 2. Th e tympanic membrane

1 - Handle of the malleus; 2 - Gerlach’s ligament (annulus); 3 - Pars tensa; 4 - Umbo; 5 - Politzer’s triangle (cone of light); 6 - Short process of the malleus; 7 - Anterior malleo-tympanic fold; 8 - Pars fl accida (Shrapnell’s membrane); 9 - Posterior malleo-tympanic fold

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Figure 3. Middle ear cavity

1 - Malleus; 2 - Incus; 3 - Stapes; 4 - Eardrum; 5 - External auditory canal; 6 - Middle ear cavity; 7 - Eustachian tube

of the internal wall are the lateral or horizontal semicircular canal, the facial nerve, the oval window, the promontory (corresponding to the basal turn of the cochlea) and the round window. Th e facial nerve is dehiscent in its tympanic course (oval window region) in about 30% of individuals1. Under the fl oor of the middle ear lies the jugular bulb. Above the middle ear is the dura-mater of the middle cranial fossa. Th e mastoid antrum lies just behind the middle ear cavity and communicates with it via the aditus ad antrum. In most of the population the mastoid air cell system is fairly extensive with air cells extending into the mastoid tip, the zygomatic arch, the petrous apex. Sometimes the mastoid antrum is the only air-fi lled space in the mastoid. Th is condition is called sclerotic mastoid and occurs in perhaps 20% of adult temporal bones, mainly in individuals with chronic ear diseases2. Th e Eustachian tube communicates with the rhino-pharynx and may be opened by pharyngeal movements (swallowing). It has

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a bony upper part and a cartilaginous lower end. Th e pharyngeal opening has a typical triangular-shaped appearance.

1.1.3. Internal Ear

Th e inner ear labyrinth consists of a bony capsule within which is the membranous labyrinth. Th e bony capsule consists of three parts:

Posteriorly, the three semicircular canals• In the middle, the vestibule• Anteriorly, the cochlea•

Th e bony labyrinth contains perilymph. Suspended in the perilymph lies the membranous labyrinth. Th is is a complex series of sacs and tubes containing a diff erent fl uid, called the endolymph. Th e membranous labyrinth consists of three semicircular canals: horizontal, superior and posterior, the superior and the posterior share a common crus. Th e anterior end of each canal is dilated to form its ampulla, which contains a patch of neuroepithelium called crista ampullaris, the hairs of which are embedded in the overlying gelatinous cupula. Th e canals open into the utricle, this and the saccule both lie in the vestibule of the bony labyrinth. Each contains a patch of neuroepithelium known as the macula. Th is resembles the crista ampullaris, except that the overlying membrane is fl atter and contains particles of calcium carbonate called otoliths. Th e membranous cochlear duct is a simple tube situated in the bony cochlea and coiled for two and a half turns around its central bony modiolus. Th e neuroepithelium of the cochlea is arranged along the entire length of the basilar membrane and is known as the spiral organ of Corti3.

1.2. Physiology of the ear

1.2.1. Middle ear

Sound represents a combination of waves that are generated by a vibrating sound source (or sources) and propagated through the air until they reach the ear. Th e study of hearing is often concerned with measuring the minimum intensity of sound that can be detected by the ear. Th is is defi ned as the auditory threshold. Th e sound intensity is measured in decibels (dB).

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Th e external and middle ears, which together form the conductive component of the auditory apparatus, transmit sound waves from the external environment to the sensory organ of hearing, the inner ear. As they transmit sound, they also amplify it and modify its frequency spectrum. Th e ability of a wave to be transferred from one medium to another is dictated by the impedances of the media. When a wave is transferred from a low-impedance medium (ex. air) to one of high impedance (ex. water), a considerable amount of its energy is refl ected and fails to enter the liquid. Water has 3470 times the acoustic impedance of air, meaning that only 0.1% of the sound energy present in an acoustic signal traveling through air actually enters a water-fi lled medium. Moreover, because of the small surface area of the oval window, approximately 3% of the sound energy of an acoustic signal enters the cochlea. For hearing to occur, a system of amplifi cation must be present to address the impedance mismatch that exists between air and water. Th e external and middle ears provide that amplifi cation system. Th e anatomic arrangement of the structures within the middle ear, allow it to amplify sound waves delivered to it via the external ear. Sound is transferred from the large surface area of the tympanic membrane to the small surface area of the oval window. Amplifi cation of the wave occurs because of a concentration of sound energy at the level of the oval window. In addition, the long axis of the malleus is longer than that of the incus, creating a lever eff ect that further augments the amplitude of the wave. Th e amplifi cation conferred on the sound wave by the area ratio (22:1) and the lever ratio (1.3:1) substantially mitigates the eff ect of the impedance mismatch. Th e eustachian tube is responsible for maintaining the middle ear in a condition that optimizes middle ear function. Its functions include the following: to equilibrate air pressure in the middle ear with that of the external environment, to drain middle ear secretions into the nasopharynx and to protect the middle ear from potentially infectious nasopharyngeal secretions1.

1.2.2. Inner Ear

Th e inner ear functions as the sensorineural receptor organ of the auditory system, converting an acoustic waveform into an electrochemical stimulus that can be transmitted to the Central nervous system. Th e cochlea consists of 3 fl uid-fi lled ducts or scalae. Th ese ducts are functionally divided into 2 spaces. Th e scala tympani and

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Figure 4. Inner ear

1 - Superior semicircular canal; 2 - Perilymph; 3 - Endolymph; 4 - Horizontal semicircular canal; 5 - Posterior semicircular canal; 6 - Ampullae; 7 - Oval window; 8 - Utricle and saccule; 9 - Cochlear duct; 10 - Cochlea; 12 - Round window

Figure 5. Inner ear

1 - Scala vestibuli; 2 - Cochlear duct; 3 - Scala tympani; 4 - Tectorial membrane; 5 - Stria vascularis; 6 - Spiral ganglion; 7 - Cochlear nerve; 8 - Basilar membrane; 9 - Spiral organ of Corti; 10 - Spiral ligament; 11 - Vestibular membrane (Reissner)

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scala vestibuli communicate with each other and are fi lled with perilymph. Th e scala media is isolated from the perilymphatic space and contains endolymph. Th e diff erence between the electrolyte composition of the perilymphatic and endolymphatic spaces creates an electrochemical environment that makes sensorineural transduction possible. A sound wave is transmitted to the middle ear, eliciting vibration of the ossicular chain. Th e vibration of the stapes transmits the sound wave via the oval window to the scala vestibuli, generating fl uid waves in the perilymph. Th e displacement of the perilymph causes a wavelike displacement of the basilar membrane and organ of Corti, ultimately causing distention of the round window membrane. Depolarization of the hair cells generates impulses which passes along the fi bers of the cochlear nerve to reach the auditory cortex3.

1.3. Symptoms of ear diseases

1.3.1. Earache (Otalgia)

Pain in the ear is usually determined by infl ammatory conditions of the external auditory canal and the middle ear, impacted wax etc. In the majority of cases, the pain’s etiology does not belong to the ear. It is the so-called referred otalgia. In these cases, a complete ENT examination must be performed after we excluded during otoscopy a local cause.

1.3.2. Hearing Loss

Hearing loss may be unilateral or bilateral, with sudden onset or long-standing, stable or progressive, fl uctuating. Th e history is extremely important.

Ask the patient about the onset, the evolution of the • hearing loss Ask the patient about his/her job (noise exposure)• Is there a family history of hearing loss?• Is there a history of meningitis, parotitis, measles or • syphilis, head trauma?Is there a history of ototoxic drugs (e.g. streptomycin, • gentamicin, loop diuretics, cisplatin)?Is the hearing loss associated with tinnitus, vertigo, other • neurological symptoms?

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OTOSCOPIC EXAMINATION IN EARACHE

Pathological Normal

External auditory canal Tympanic membrane Dental problems• Acute tonsillitis• Peri-tonsillar abscess• Temporo-mandibular joint • syndromeCervical spine• Mucosal aero-digestive tract • malignanciesElongated stiloid process • (Eagle’s syndrome)

Trauma• Lacerations• Hematoma• Furuncle• Diff use external otitis• Otomycosis• Herpes zoster oticus • Impacted cerumen• Foreign bodies•

Traumatic perforation• Haemotympanum• Acute suppurative otitis • mediaSerous otitis media (mild)• Bullous myringitis•

Table 1. Otoscopic examination in earache

HEARING LOSS

Conductive Sensorineural

External ear Middle ear

Noise exposure• Ototoxic drugs• Presbyacusis• Meniere’s disease•

Wax• Diff use external otitis• Foreign bodies• Tumors of the EAC•

Eardrum perforation • Acute suppurative otitis media• Serous otitis media• Chronic suppurative otitis media• Otosclerosis•

Table 2. Types of hearing loss

1.3.3. Aural discharge (Otorrhoea)

Th e aural discharge may be unilateral or bilateral, acute or chronic (long-standing), continuous or intermittent. Semiologic characteristics of the discharge may give us a hint for a quick diagnosis.

Muco-purulentDiff use external otitis• Foreign bodies• Chronic suppurative otitis media•

Foul-smelling muco-purulentCholesteatoma• Malignant external otitis•

Blood-stained muco-purulentAural polyp• Carcinoma•

Creamy, fl uff y Otomycosis•

WateryCerebrospinal fl uid leak following temporal bone • trauma

Table 3. Semiologic characteristics of aural discharge

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1.3.4. Tinnitus

Tinnitus is described as an annoying perception of noises or sounds within the head or the ears. It causes a lot of distress, especially at night when the patient is trying to sleep. Th e term tinnitus derives from the Latin word tinnire, meaning to ring. Sound that only the patient hears is subjective tinnitus, while sound that others can hear as well is called objective tinnitus. Estimates of patients with tinnitus range from 10-15% of the population (30-40 million people). Of patients presenting with ear-related symptoms, 85% report experiencing tinnitus as well. Both adults and children report experiencing tinnitus. Development of tinnitus increases in incidence with age4. Tinnitus is a symptom not a disease, and therefore refl ects an underlying abnormality. In most cases is associated with sensorineural hearing loss. History is extremely important:

Is it bilateral, unilateral?• Time of onset?• Is it continuous, intermittent, pulsating, low-pitched or • high-pitched?

Objective tinnitus is a rare condition and has a muscular or vascular etiology.

1.3.5. Vertigo (Dizziness)

Vertigo is a sensation of abnormal movement of the surroundings in relation to the patient, or of the patient in relation to his surroundings. It is often accompanied by vegetative symptoms: nausea, vomiting, sweating, tachycardia, but never loss of consciousness. It has a rotatory or spinning pattern, and is related to head movements. It represents the main symptom in the peripheral vestibular syndrome. In the peripheral vestibular syndrome patients describe episodic attacks, with normal equilibrium between the crises. Th e duration of the vestibular crisis is very important for the diagnosis. We have vertigo lasting seconds (benign positional paroxysmal vertigo BPPV), hours (Meniere’s disease) or days (vestibular neuronitis)5. It is essential to collect a detailed clinical history:

Diff erentiate the true vertigo from other pathological • movement sensations (dizziness, unsteadiness)!

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Th e duration of the attack?• Does head motion impel the crises?• Th e presence of associated vegetative symptoms: nausea, • vomiting?Th e association of a hearing loss, tinnitus, aural fullness?• Th e association of other neurological symptoms?• Is there a history of head trauma, chronic ear disease, ear • surgery?

1.4. Clinical examination

1.4.1. Inspection and palpation

Inspection of the pinna will follow the anatomical landmarks determined by the cartilage prominences: the helix, the ante helix, the tragus, the anti tragus, the triangular fossa. Th e ear lobule does not contain cartilage, only adipose tissue. Gross inspection of the pinna and the retro-auricular region may reveal the presence of congenital malformations, infl ammatory processes and neoplastic diseases. Palpation of the mastoid area:

Th ree mastoid • trigger points to palpateTh e antral point: it is located posterosuperiorly to the • meatus, it corresponds to the antrum, it is sensitive in acute otomastoiditisTh e tip of the mastoid: it is sensitive in a particular form • of mastoiditis (Betzold’s), in which the pus erodes the bony cortical wall of the mastoid’s tip and accumulates beneath the sternocleidomastoid muscle’s sheath, at the level of its superior insertionTh e sinusal point is located at the level of the postero-• inferior margin of the mastoid bone, 1-1.5 cm from the mastoid’s tip, posteriorly; it is sensitive in sigmoid sinus thrombosis, a serious complication of cholesteatoma.

1.4.2. Otoscopy

Instruments:Aural speculum• Siegle’s aural speculum: squeezing of the bulb causes • the eardrum to move, by applying positive and negative pressures (pneumatic otoscopy)

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Figure 6. Th e auricle (pinna)

1 - Helix; 2 - Ante helix; 3 - Triangular fossa; 4 - Tragus; 5 - Anti tragus; 6 - Ear lobule; 7 - Concha

Figure 7. Th e mastoid points

1 - Antral point; 2 - Tip of the mastoid; 3 - Sinusal point

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Figure 8. Aural speculum

Figure 9. Otoscope

Figure 10. Otoscopy

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Electric otoscope (magnifi cation ×2.5) • Th e 0°, 4 mm tele-otoscope (ear rigid endoscopy)• Th e binocular microscope: greatest accuracy in ear • examination

Th e largest speculum which can be inserted in the meatus without pain should be used. To get a clear view, the pinna and cartilaginous canal must be pulled upwards and backwards. For the examination of the left ear, the ear speculum is held between the thumb and the forefi nger of the left hand. For the examination of the right ear the speculum is held in the right hand. Th e external auditory canal is inspected for foreign bodies, cerumen, secretions, mycosis, exostosis. If secretions are present in the external auditory canal, a careful toilette must be performed using suction. Th e appearance of a normal tympanic membrane (anatomical landmarks):

Gerlach’s ligament (• annulus): the eardrum is attached to the tympanic ringHandle of the malleus, with the short, lateral process • projecting laterally from the upper end of the handleUmbo: the central point of the tympanic membrane (at • the tip of the malleus handle)Anterior and posterior tympano-malleolar folds• Pars tensa: a pale grey tense thin membrane• Pars fl accida (Schrapnell’s membrane), covering the attic • region is loose and much thickerPolitzer triangle: a cone of light refl ex located in the • antero-inferior quadrant of the tympanic membrane

1.4.3. Examination of the Eustachian tube

Valsalva’s method of infl ation: the patient is instructed to • take a deep breath, keep the nose tightly pinched and the lips fi rmly closed, and then forcibly attempt to blow the nose. If succesfull, the drum will be seen to move outward and a click may be audiblePolitzer’s method: the olive attachement of a Politzer • bag is applied to one nostril, the other nostril is closed with a fi nger, the patient is told to say “jac” or “cucu”, at the moment of the nasopharynx closure the bag is compressed sharply and the intra-nasal pressure thereby

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raised considerably above normal, the result is to force air up to the Eustachian tubeInfl ation with a catheter placed at the level of the • pharyngeal ostium of the Eustachian tube, through the nasal cavities

1.4.4. Examination of hearing

Hearing can be assessed by the clinician during the ENT examination. Some subjective tests are available, even if they are not so accurate as a pure-tone audiometry. For example, the free-fi eld speech test will in most cases discover a hearing loss greater than 30 dB with a false-positive rate around 13%6.

1.4.4.1. The whispered voice test (Free-fi eld speech testing)

A normal ear is capable of hearing a whispered voice from a distance of nearly 6 meters. So, the examiner will be placed at 6 meters from the patient, opposite the ear to be tested, in a silent environment. It is compulsory to mask the non test ear (pushing the opposite ear tragus in and out by an assistant). Also the eyes must be covered, in order to avoid lip-reading by the patient. Th e examiner explains to the patient that a combination of numbers will be whispered and spoken in the test ear, and the patient is asked to repeat them. If the patient is capable of recognizing more than 80% of the whispered numbers, we will consider a normal hearing. If the patient doesn’t hear anything from 6 meters, we will repeat the test from 3 meters and 1 meter.

1.4.4.2. Tuning fork tests

Tuning fork tests are used to diff erentiate between a conductive and a sensorineural hearing loss. Th e most frequently used forks are the 256- and 512-Hz, as these give more reliable outcomes than the 1024-Hz fork7. Th e tuning forks are activated by striking them lightly against a bony prominence (knee, elbow). It’s very important the way you are handling the tuning fork, you are not allowed to touch the vibrating parts!

1.4.4.2.1. The Weber test

Th e examiner places the activated tuning fork centrally on the forehead, on the bridge of the nose or over the incisor teeth,

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and ask the patient to tell him if a sound or a vibration is audible. If the answer is positive, the examiner will ask the patient to describe where the sound is perceived: in the midline, in the left ear or in the right ear? We have three clinical possibilities:

Th e patient locates the sound in the midline or in both ears • (normal hearing or symmetrical bilateral hearing loss)Th e sound is perceived in the aff ected ear (conductive • hearing loss)Th e sound is perceived in the healthy ear (sensorineural • hearing loss)

Unfortunately, the Weber test has a low sensitivity and specifi city, the chance of accurately diff erentiating conductive and sensorineural hearing loss is 33%8.

1.4.4.2.2. The Rinne test

Th e examiner places the activated fork on the mastoid bone behind the auricle (bone conduction) and asks the patient to confi rm if the sound is audible. If the patient’s answer is positive, the examiner asks the patient to notice when the sound is no longer heard. Th e average time for bone conduction in a normal

Figure 11. Th e Weber test

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Figure 12 - Th e Rinne test 1

Figure 13 - Th e Rinne test 2

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ear, using the 512-Hz fork, is about 20 seconds. After the sound on the mastoid bone is no longer perceived, the examiner places the fork in front of the tested ear (air conduction), and asks the patient to tell if the sound is audible again, and if the answer is “yes”, to report when the sound is no longer audible. In normal hearing, the patient should hear the sound other 20 seconds. Th is is the positive Rinne test. What happens if the patient has a conductive hearing loss in the tested ear? Th e bone conduction is normal, so the sound will be heard on the mastoid for 20 seconds or even more, but when the examiner places the fork in front of the ear, no sound will be heard. It’s the negative Rinne test. If a sensorineural hearing loss is present, the patient will hear the sound on the mastoid less than 20 seconds, and the same period of time when the fork is placed in front of the ear (air conduction is normal). It’s a positive shortened Rinne test. Th ough many ENT doctors use a 516-Hz tuning fork, the 256-Hz fork give superior sensitivities and specifi cities9.

1.4.4.3. Pure-tone audiometry

Th e purpose of the pure-tone audiometry is to determine hearing threshold levels for pure tones. Th e hearing threshold is defi ned as the level of a sound at which, under specifi ed conditions, a person gives 50 per cent

Figure 14. Pure tone audiometry: normal hearing (—< : bone conduction right ear; — : air conduction right ear; >— : bone conduction left ear; ×— : air conduction left ear)

29

Figure 15. Pure tone audiometry: conductive hearing loss ( —< : bone conduction right ear; — : air conduction right ear; >— : bone conduction left ear; ×— : air conduction left ear)

of correct detection responses on repeated trials. “Specifi ed conditions” means the type of sound and ways of presenting the sound. Th e normal test sound is pure tone pulses at standardized frequencies in the range of 125-8000 Hz, and the normal presentation mode is monaurally by means of a standardized type of earphone10. Th e necessary equipment consists of a pure tone audiometer. Air conduction and bone conduction is assesed, with the patient placed in a phonic isolated room. Data are recorded on a typical graph.

Figure 16. Pure tone audiometry: sensorineural hearing loss ( —< : bone conduction right ear; — : air conduction right ear;

>— : bone conduction left ear; ×— : air conduction left ear)

30

A complete audiogram, using both air- and bone-conduction, supplies important information for the diagnosis, in terms of diff erentiation between conductive and sensorineural disorders. An air- and bone-conduction loss at the same level suggests a sensorineural hearing loss, whereas a larger loss by air-conduction than by bone-conduction (the so-called air-bone gap), indicates the presence of a conductive hearing loss. Th e air-bone gap at a single frequency needs to be at least 15 dB in order to be considered statistically signifi cant10.

1.4.4.4. Speech audiometry

Speech audiometry is a considerably more complex procedure. In pure-tone audiometry, the listening task is simply detection of the stimuli. In speech audiometry the usual task is not detection but recognition, which requires both detection and identifi cation of the phonemes and recognition of sets of phonemes as words. Equipment for speech audiometry is usually integrated with a clinical pure-tone audiometer. In conventional clinical speech audiometry, the test signal is presented monaurally by means of earphones. Th e speech test material may diff er widely from nonsense combinations of consonants and vowels, so-called logatoms, to natural connected speech10. In practice, lists of two-syllable words are supplied by air-conduction through earphones or in free-fi eld to both ears. Th e patient is asked to repeat the words and the results are recorded on a special graph 1.

1.4.4.5. Objective assessment of hearing

All the methods previously described are subjective, because they require an active participation of the tested subject. A number of objective methods for hearing evaluation are also available:

Impedance audiometry: tympanometry and stapedial • refl ex (acoustic refl ex)Electro-cochleography• Auditory brainstem responses (ABR)• Oto-acoustic emissions (OAE)•

31

2. DISEASES OF THE EXTERNAL EAR

2.1. Diseases of the auricle

2.1.1. Congenital abnormalities

Congenital malformations of the external ear are, by defi nition, present at birth. Th e etiology could be determined by genetic defects (e.g. chromosomal abnormalities) or by acquired factors that disturb the normal embryonic development of the ear (drugs, nutritional defi ciencies, viral infections, hormonal, metabolic disorders, autoimmune reactions).

Microtia, macrotia, poliotia, anotia, melotia (ear located • on the cheek)Auricular appendages•

Figure 17. Ear malformations (outstanding ear, melotia, auricular appendages)

32

Atresia of the external auditory canal• Auricular fi stulas• “Bat ears” (outstanding ears) •

2.1.2. Trauma

2.1.2.1. Sharp trauma, lacerations and bites

From history, try to determine the etiology: aggression, accident, human bite, animal bite. Perform photographic documentation for legal purposes. In case of infection, a bacterial culture is required. Treatment involves:

Local toilet and disinfection (H• 2O

2, Betadine)

Removal of debris and devitelized tissues• Antibiotics• Rabies prevention in case of animal bites• Tetanos prevention for all contaminated wounds• Surgical repair if necessary •

2.1.2.2. Othematoma

Othematoma is a sero-sanguinolent fl uid accumulation between the perichondrium and the cartilage of the pinna, located at the level of the triangular fossa. Most frequently it is determined by a blunt trauma to the external ear (boxers, wrestlers, rugby players, assaults). A soft tumefaction is visible at inspection on the outer part of the auricle, at the level of the triangular fossa. Th e overlying skin is normal in appearance. Palpation of the mass reveals fl uctuation. Treatment requires the aseptic drainage of the fl uid. Simple aspiration is not recommended, because of the high risk of recurrence. It’s compulsory to place a pressure dressing on both sides of the pinna, and fi x it into the concha with through-and-through mattress sutures. To avoid secondary infection, antibiotics are prescribed. Because Pseudomonas aeruginosa has a great propensity to the ear cartilage, Ciprofl oxacin is the fi rst-choice antibiotic. Complications:

Recurrences•

33

Infection (perichondritis)• Chronic organization of the hematoma, with • chondronecrosis, perichondritis, chondroneogenesis, leading to the typical aspect of a “caulifl ower ear”

2.1.3. Perichondritis

Perichondritis is an infection and infl ammation of the ear cartilage’s perichondrium. It may follow the infection of an othematoma or other traumatic injuries (even after ear surgery). Pseudomonas aeruginosa is the most frequently involved bacterial agent (75-90%)11. Th e patient complains of severe pain. Local examination reveals a swollen, erythematous and tender pinna. Fluctuation indicates the presence of an abscess and possible chondritis. Th e lobule, which contains no cartilage, is spared. Treatment:

Local disinfection• Incisions, drainage• Broad-spectrum antipseudomonal antibiotics (oral • quinolones), non-steroidal anti-infl ammatory drugs (NSAIDs)

Figure 18. Othematoma Figure 19. Perichondritis

34

2.1.4. Skin infections

Impetigo: staphylococcal infection, most commonly • occurs in young children, frequently secondary to an otorrhoea. Treatment: crusts removal, application of an antibiotic-steroid ointmentErysipelas: streptococcal infection which produces a raised, • red edematous eruption with sharply defi ned margins, malaise, high fever. Treatment: high-doses PenicillinHerpes zoster oticus: Ramsey-Hunt syndrome (painful • neuralgia, vesicular eruption in the concha, facial nerve palsy)

2.1.5. Tumors

Benign: keloids, epidermal cysts, keratoacanthomas• Malignant: basal cell carcinomas, squamous cell • carcinomas, malignant melanomas

2.2. Diseases of the external auditory canal

2.2.1. Wax (Cerumen)

Wax represents a normal secretion of the ceruminous glands, which are located in the skin covering the outer cartilaginous portion of the canal. In case of impacted ear wax, patient complains of a hearing loss, aural fullness, earache, irritation in the ear. Th e hearing loss is conductive because it is caused by the obstruction of the external canal (air-conduction). Patients often describe a sudden onset of the hearing loss, mainly after bathing. Water causes the wax to swell and the passage is completely blocked. Wax removal:

Aural irrigation (syringing) with warm water after • preliminary softening: this maneuver is not allowed if the patient has undergone previous ear surgery, or if a perforation of the eardrum is present. Care must be taken to avoid any trauma to the tympanic membrane and the skin of the auditory canalUnder direct microscopic control using a special curette • and/or suctioningCeruminolytics: olive oil, H•

2O

2 4%, sodium bicarbonate

25%12

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2.2.2. Foreign bodies

Foreign bodies are almost invariably confi ned to children: vegetables, parts of plastic toys, beads, insects. Removal is performed under direct microscopic control. Sometimes general anesthesia is required (mainly in children). Syringing may be also useful, in case of small plastic or metallic foreign bodies. Don’t forget the main damage by a foreign body in the EAC is caused by its careless removal12!

Type of foreign body Method of removal

Living insects• Irregular/graspable objects• Organic/vegetable• Button batteries• Round, hard, smooth non-graspable•

First kill the insects with oil• Remove with crocodile forceps• Do not syringe• Do not syringe• Syringe/remove with wax hooks•

Table 3. Techniques of removal advised for diff erent types of foreign bodies13

Figure 20. Cerumen (wax)

Figure 21. Guyon’s syringe for aural irrigation (wax removal)

36

2.2.3. Trauma

Th e canal may be injured while removing wax or a foreign body with a sharp instrument or during syringing.

2.2.4. Localized external otitis (Furuncle)

2.2.4.1. Defi nition. Aetiology

Furuncle is a localized infection of a hair follicle, located at the level of the skin covering the outer cartilaginous portion of the canal, caused by Staphylococcus aureus.

2.2.4.2. Symptoms

Patient complains of extremely severe otalgia, augmented by moving or touching the pinna.

2.2.4.3. Diagnosis

Inspection may reveal swelling of the parts around the ear, corresponding to the location of the furuncle within the meatus. Palpation of the tragus is painful. Sometimes palpation may disclose a retro-auricular infl ammatory lymphadenopathy. Otoscopic examination enables an accurate diagnosis. A localized swelling of the skin is noticed, with signs of acute infl ammation and a characteristic white point (bourbillion) in the middle of the

tumefaction.

2.2.4.4. Treatment

Topical disinfectants• Topical antibiotic ointments• Analgetics, non-steroidal antiinfl ammatory drugs • (NSAIDs)Systemic antibiotics (Oxacillin, Amoxicillin with • Clavulanate)

Figure 22. Furuncle of the external ear canal

37

Surgery (incision, drainage)• In case of multiple recurrences, check if the patient doesn’t have diabetes!

2.2.5. Diff use external otitis (Swimmer’s ear)


Diff use external otitis is an acute infection and infl ammation of the skin covering the external auditory canal. It is most frequently encountered during summer season, with patients addressing the ENT doctor after bathing in public swimming pools. Th e most common bacterial agents involved are Pseudomonas aeruginosa (40% of cases), Staphylococcus epidermidis (9% of cases) and Staphylococcus aureus (8% of cases)14. Predisposing factors: absence of cerumen, high humidity, increased temperature, local trauma.

2.2.5.2. Symptoms

Otalgia, augmented by temporomandibular joint • movements or by pressure applied to the tragusItching, sensation of aural fullness• Clear or muco-purulent otorrhoea• Hearing loss•

2.2.5.3. Diagnosis

Palpation of the tragus is painful. Sometimes sensitive periauricular lymph nodes are present. Otoscopy: the entire skin of the canal has a red, swollen, edematous appearance with accumulation of debris and secretions. A careful toilette of the canal must be performed by suctioning. If the edema is pronounced, the tympanic membrane may not be visible. Th e hearing loss is conductive (Weber lateralization Figure 23. Diff use external otitis

(“swimmers ear”)

38

to the aff ected ear, negative Rinne test, air-bone gap in pure-tone audiometry). In cases resistant to adequate treatment, bacteriologic examination is required.

2.2.5.4. Diff erential diagnosis

Localized external otitis (Furuncle)• Otomycosis• Acute suppurative otitis media• Chronic otitis media• Malignant tumors of the external auditory canal (EAC)• Referred otalgia•

2.2.5.5. Treatment

Removal of the debris and secretions from the ear canal is required, under microscopic control, if available. A sterile gauze strip with a topical antibiotic/steroid otic solution is inserted within the canal. Antibiotic drops continue to be the mainstay of treatment for otitis externa. Th e main characteristics of the ideal drop are exposed here 15:

Broad-spectrum coverage for involved bacteria• An acidic vehicle• No ototoxicity (great care if the patient has a perforation • of the eardrum!)No potential for allergic reactions• Low cost• A steroid to alleviate pain and decrease edema more • rapidly

Quinolone antibiotics (no ototoxicity, no risk of contact dermatitis) are available in both otic and ophthalmic solutions (Ciplox). Other alternatives: Polymyxin, Neomycin, Gentamycin (Garasone). Th e aminoglycosides are not recommended if the patient has an eardrum perforation, because of their potential for ototoxicity! Oral antibiotics (Ciprofl oxacin) are prescribed only in severe cases with fever, peri-auricular adenopathies, and always in diabetic patients. Analgetics and non-steroidal antiinfl ammatory drugs (NSAIDs) are also prescribed.

39

It is important to tell the patient to avoid water penetration into the canal during treatment period.

2.2.5.6. Complications

Chronic external otitis with stenosis of the ear canal• Otomycosis: in case of long-term administration of • topical antibiotic/steroid otic solutionsMalignant external otitis: a necrotizing evolution with • otogenic skull base osteo-myelitis (elderly patients with diabetes mellitus or severe immunodefi ciencies)

2.2.6. Otomycosis (Fungal external otitis)


Th e most frequently involved agents are: Aspergillus fumigatus, Aspergillus fl avus, Aspergillus niger and Candida albicans. In most cases, otomycosis is not a primary infection, it follows a long-term administration of antibiotic and steroid drops.

2.2.6.2. Symptoms

Aural fullness• Itching, vague pain in the ear• Ear discharge: thick, creamy, curdlike, fl uff y •

2.2.6.3. Diagnosis

Otoscopic examination reveals the presence of masses of material like wet blotting paper, upon which the mycelia can be seen. Th e color of the mass may be whitish, yellow or black, depending on the involved agent.

2.2.6.4. Treatment

Treatment requires repeated and careful cleaning of all debris, under microscopic control, followed by topical application of

Figure 24. Otomycosis

40

an anti-fungal agent (Clotrimazole, Miconazole). Local treatment must be continued for at least 3 weeks after apparent cure. Topical disinfectants (Betadine solution) are also useful. In persistent cases of external otitis, otomycosis should always be taken into account!

2.2.7. Tumors

2.2.7.1. Benign tumors

2.2.7.1.1. Osteomas

Osteomas are benign bony tumors located at the level of the inner bony portion of the external auditory canal. Th e main symptom is a unilateral conductive hearing loss. Otoscopy reveals a solitary bony mass covered by normal skin, obstructing the canal. CT scan is useful to evaluate the extension of the mass. Treatment: surgical removal by drilling, under microscopic control.

2.2.7.1.2. Exostoses

Exostoses are areas of localized bulges of bone covered by a thin, normal skin. Th ey are located in the osseous portion of the external canal, usually multiple and bilateral. Frequently, the patients describe a history of various aquatic activities (swimming in cold water, surfi ng, diving)12. Generally asymptomatic, they require surgical treatment only when a subtotal obstruction of the canal, with subsequent wax retention and conductive hearing loss is present.

2.2.7.1.3. Ceruminomas

Ceruminomas are benign tumors developed from the ceruminous glands. Th ese glands are located in the skin covering the outer part of the external auditory canal.

2.2.7.2. Malignant tumors

2.2.7.2.1. Squamous cell carcinomas (SCC)

Squamous cell carcinoma is the most frequent malignant

41

skin tumor located at the level of the external auditory canal (60%)12. It is frequently associated with chronic muco-purulent otorrhoea. Biopsy is mandatory for an accurate diagnosis. In evolution invades the middle ear and the mastoid bone.

2.2.7.2.2. Basal cell carcinomas (BCC)

Th e external auditory canal (EAC) is rarely the primary site of development. Most frequently BCC of the EAC is the result of a local invasion originating from an auricle lesion.

2.2.7.2.3. Malignant ceruminomas

Malignant ceruminomas account for nearly 15% of all malignant tumors of the external canal12. Other rare tumors: adenocarcinomas, rhabdomyosarcomas, melanomas.

42

3. DISEASES OF THE MIDDLE EAR

3.1. Trauma

3.1.1. Traumatic perforations of the eardrum


Direct trauma: sharp instruments, sharp foreign bodies, • iatrogenic (syringing, foreign bodies removal)Indirect trauma: abrupt increase of the pressure in the • external canal caused by an explosion, diving, getting slapped on the ear

3.1.1.2. Symptoms

Otalgia• Aural fullness• Tinnitus• Conductive hearing loss with sudden onset•

3.1.1.3. Diagnosis

Otoscopy reveals the traumatic perforation, located in pars tensa. Th e perforation’s shape is usually linear, but it may be also triangular, round, ovalar. Blood may be noticed in the canal or at the level of the perforation’s margins. Th ese margins may be slightly erythematous. Photographic documentation for legal purposes is strongly recommended.

Figure 25. Traumatic perforation of the tympanic membrane

43

Evaluation of hearing: Weber lateralization to the damaged ear, negative Rinne test and a light air-bone gap (10-30 dB) in pure-tone audiometry (conductive hearing loss).

3.1.1.4. Treatment

Sterile dressings, antibiotics to prevent infection• NO water in the ear!• Observation: small recent perforations may heal • spontaneously Small perforations that do not heal spontaneously can • be treated by surgery, under microscopic control, in local or general anesthesia (placement of a steri-strip patch over the perforation, placement of a cigarette paper patch moistened with Ringer solution16, “fat graft myringoplasty” using a small piece of fat harvested from the ear lobule)Large perforations may require an underlay myringoplasty • using perichondrium, cartilage or temporal fascia

3.1.2. Hemotympanum


Hemotympanum is an accumulation of blood in the middle ear cavity, behind a non-perforated eardrum. Th e aetiology is traumatic.

3.1.2.2. Symptoms

Aural fullness• Conductive hearing loss•

3.1.2.3. Diagnosis

Otoscopic examination reveals a non-perforated eardrum with a typical dark-bluish color. Pneumatic otoscopy with the Siegle’s otoscope demonstrates a decreased mobility of the tympanic membrane, caused by the accumulation of fl uid in the middle ear. Figure 26. Hemotympanum

44

Evaluation of hearing: Weber lateralization to the aff ected ear, negative Rinne test and an air-bone gap in pure-tone audiometry (conductive hearing loss).

3.1.2.4. Treatment

Oral antibiotics (Amoxicillin, Amoxicillin with • Clavulanate): in order to prevent infectionNasal decongestants: in order to facilitate blood evacuation • from the middle ear through the Eustachian tube

3.1.3. Otitic barotrauma


“Barotrauma” is defi ned as an injury produced by mechanical forces caused by a change of pressure in a gas-fi lled space 17. Th erefore, otitic barotrauma is a term that encompasses those pathological conditions of the ear induced by pressure changes. Middle ear barotrauma is the most frequent pressure-induced ear condition. In the majority of cases, barotrauma is the outcome of air pressure changes experienced during commercial fl ights. Diving is the second important etiological factor. Transient evidence of middle ear barotrauma has been reported in 5% of adults and 25% of young children after fl ying18.

3.1.3.2. Pathophysiology

Middle ear barotrauma is the result of an acute loss of ventilation in the middle ear, caused by a sudden increase of atmospheric pressure which is not compensated by the Eustachian tube function: the external pressure increases rapidly, collapsing the eardrum inwards.

3.1.3.3. Symptoms

Aural fullness• Otalgia: typically augmented with increased compression • and the inability to equalize middle ear air pressureHearing loss• Tinnitus, vertigo in case of associated inner ear barotrauma•

45

3.1.3.4. Diagnosis

Otoscopic examination reveals various aspects: normal tympanic membrane, redness and retraction, intratympanic haemorrhage, hemotympanum, perforation. Evaluation of hearing: tuning fork tests, pure-tone audiometry, tympanometry (conductive hearing loss and decrease of compliance with negative middle ear pressure). It is also recommended to perform an evaluation of the Eustachian tube function and an accurate examination of the nose and nasopharynx.


Rupture of the round window membrane involves the labyrinth (inner ear barotrauma) with subsequent fl uctuating sensorineural hearing loss, tinnitus and vertigo. If a perforation of the eardrum occurs during diving, the penetration of water within the middle ear cleft may elicit a caloric vertigo, which can be fatal!

3.1.3.6. Treatment

Nasal decongestants: sprays/drops (0.1% Xylomethazoline) • 3-4 times dailyValsalva maneuvers, air infl ations with a Politzer bag or • a catheterIn cases of persistent negative pressure in the middle ear: • myringotomy with ventilation tube insertionIn cases of inner ear involvement: high doses of • corticosteroids, rheologic treatmentIn cases of hemotympanum: antibiotics• In cases of suspected rupture of the round window • membrane: surgical exploration of the middle ear cavity and closure of the fi stula

3.1.3.7. Suggestions

Avoid air travel during severe upper airway tract infections• In case of Eustachian tube dysfunction apply nasal • decongestants 20 minutes before take-off and landing19

Use chewing gum during fl ights•

46

3.1.4. Temporal bone trauma


Temporal bone trauma is a physical insult of the temporal bone induced by impact with a blunt surface or penetrating missile. Th is may or may not be associated with a temporal bone fracture20. Aetiology:

Road traffi c accidents (40-50%)• Falls• Assaults, aggressions• Industrial and sporting accidents•

3.1.4.2. Symptoms

History is very important!• Patient may be conscious or in coma• Headaches, otalgia• Hearing loss• Otorrhagia (blood exteriorization from the ear canal), • otoliquorea (cerebrospinal fl uid CSF exteriorization from the ear canal)Tinnitus, vertigo, nausea, vomiting (labyrinthine involvement)•

3.1.4.3. Diagnosis

Inspection must evaluate the presence or absence of lower motor neuron facial nerve palsy. Also the presence of bruising over the mastoid process must be noticed (Battle’s sign). Evidence of wounds, hematomas must be accurately documented for legal purposes. Otoscopic examination may reveal the presence of fresh blood or clots in the external canal, lacerations of the skin covering the canal, tympanic membrane perforations, hemotympanum, a step deformity in the bony wall of the canal. Radiological examinations: CT scans, MRI Hearing assessment: tuning fork tests in emergency, a standard pure-tone audiometry should be performed as soon as possible, tympanometry, electric response audiometry (can be performed in the unconscious patient) Vestibular assessment: nystagmus Evaluation of facial nerve function: observing active and passive facial movements. Evaluation of a cerebrospinal fl uid leak.

47

A complete neurological and neurosurgical examination must be performed: evaluation of the level of consciousness (Glasgow score) is mandatory.


Peripheral facial nerve palsy: which can be immediate, • determined by the trauma itself, or delayed (2-12 days) owing to neural edema or to herpes virus reactivation in the geniculate ganglion 19

Perilymphatic fi stula• CSF fi stula: clear, pink ear discharge• Meningitis• Fracture of the labyrinth: a destructive peripheral • vestibular syndrome

3.1.4.5. Treatment

3.1.4.5.1. Conservative treatment:

Analgetics, antibiotics• Collection and examination of the ear discharge: very • important in case of suspicion of otoliquoreaClose clinical survey for minimum 3 days• Toilette of the external ear canal, calibration of the meatus • if needed

3.1.4.5.2. Surgical treatment:

Emergency exploratory tympanotomy: in case of suspicion • of perilymphatic fi stulaSurgical treatment of CSF leaks: rarely indicated because • they usually heal spontaneouslyTreatment of facial nerve lesions•

3.2. Bullous myringitis

3.2.1. Defi nition. Aetiology

Bullous myringitis is an infl ammatory disease of the eardrum, characterized by the formation of sero-haemorrhagic bullae on the outer surface of the tympanic membrane. Th ese bullae may extend to the adjacent skin of the external canal.

48

An infection by infl uenza virus or by Mycoplasma pneumoniae has been suggested as the aetiological agent, but no clear evidence has been presented21.

3.2.2. Symptoms

Severe otalgia with sudden onset, usually unilateral• Blood-stained otorrhoea, which appears in the same day • with otalgiaUnilateral hearing impairment• Th e symptoms usually occur during or after an acute viral • upper respiratory tract infection

3.2.3. Diagnosis

Otoscopic examination reveals the haemorrhagic bullae, formed on the eardrum. Th ey appear as dark-bluish or red swellings. Th e bullae may spread on the wall of the external auditory canal (EAC). Th e bullae burst the same day, discharging a sero-sanguinolent fl uid. Th e tympanic membrane is intact, no perforation is seen. Sometimes this condition is associated with serous otitis media and a sensorineural hearing loss (viral infection)22.

3.2.4. Treatment

Antibiotics (Amoxicillin, Amoxicillin with Clavulanate) • to prevent bacterial infectionAnalgetics•

3.3. Acute suppurative otitis media (AOM)


A common illness in infants and young children, AOM is an acute (in most cases purulent) infl ammation of the mucosa of the middle ear cavity. Th e vast majority of AOM episodes are triggered by an acute viral upper respiratory tract infection, involving the nasopharynx. Pathogenic bacteria colonize the normally sterile middle ear space by direct extension from the nasopharynx, through the Eustachian tube.

49

Another possibility is colonization through eardrum perforations. Th e bacteria responsible for AOM are:

Streptococcus pneumoniae• (40-50%)Haemophilus infl uenzae • (30-40%)Moraxella catarrhalis • Staphylococcus aureus • (10-20%)

In children, chronically enlarged and infl amed adenoids are the most important predisposing factor for development of recurrent episodes of AOM. More than 60% of children under the age of 6 years, experience one or more episodes of AOM16.

3.3.2. Symptoms

Otalgia is severe, sharp, pulsating, with sudden onset, • causing the child to awaken from sleep and screamHigh fever (38-40°C), chills, • malaiseHearing loss• Nasal obstruction, rhinorrhoea, sore throat, cough• After 2-3 days of evolution, a purulent otorrhoea appears • bringing instant relief from the earache

3.3.3. Diagnosis

Inspection and palpation: mastoid process and retro-auricular area should be examined for tenderness or edema. Otoscopic examination reveals signs of progressive infl ammation. Th e eardrum has a typical appearance: congestion, edema, loss of normal anatomical landmarks. It bulges and fi nally ruptures after 2-3 days of evolution. Secondary, a muco-purulent discharge is noticeable in the external auditory canal. Th e eardrum perforation is not visible, because of the massive edema. Th e discharge may pulsate and refl ect light intermittently (the “Lighthouse sign”). Figure 27. Acute suppurative otitis

media

50

Hearing assessment (tuning fork tests, audiogram) reveals a conductive hearing loss. A complete ENT examination should be performed.

3.3.4. Evolution and complications

Spontaneous recovery• Complications: acute mastoiditis (infl ammation of the • mastoid cell system), facial nerve palsy, acute labyrinthitis, petrositis, meningitis, encephalitis, sigmoid sinus thrombosis

3.3.5. Treatment

3.3.5.1. Conservative treatment:

Antibiotics: AOM is a great example of the effi cacy of • an appropriate antibiotic treatment. Considering the aetiology, Amoxicillin or Amoxicillin with Clavulanate are the fi rst-choice antibiotics to be prescribed. Th e duration of treatment is at least one weekAnalgetics, non-steroidal antiinfl ammatory drugs (NSAIDs)• Nasal decongestants: nose sprays/nose drops (0.1% • Xylomethazoline for adults, 0.05% Xylomethazoline for children)Rest in a warm and well humidifi ed room• Application of local hyperthermia•

3.3.5.2. Surgical treatment:

Th e recommended European standard 16 is:Myringotomy (paracentesis): when bulging of the • eardrum persists, for immediate relief of pain!Adenoidectomy: in recurrent AOM, in most cases • accompanied by myringotomy and insertion of grommetsAntrostomy/mastoidectomy: in case of imminent • mastoiditis, labyrinthitis, sigmoid sinus thrombosis

51

3.4. Otitis media with eff usion (OME)


Otitis media with eff usion (serous otitis media, secretory otitis media, glue-ear) is characterized by an accumulation of a non-purulent eff usion in the middle ear cavity that may be either serous or mucoid. A persistent Eustachian tube dysfunction (which is the most important factor in OME’s pathophysiology) can be determined by:

Recurrent viral infections of the upper respiratory tract, • mainly of the adenoidsTumor development in the nasopharynx (especially in • adults)Allergy, chronic rhinosinusitis• Cleft palate• Radiotherapy in the area of the ear/nasopharynx•

Th e highest incidence of OME is reported within the age group between 2 and 5 years (20%). Bilateral disease is reported in 84% of cases, mainly in young children16.

3.4.2. Pathophysiology

One of the major functions of the Eustachian tube is to equilibrate the air pressure between the middle ear cleft and the external ear. Th is accounts for an appropriate physiologic vibration of the ear drum and ossicular chain. A chronic dysfunction of the tube will determine the development of a negative pressure within the middle ear cavity which elicits a transudate from the mucosa, leading to the accumulation of a serous eff usion. OME may also occur during the resolution of an AOM.

3.4.3. Symptoms

In children: reports from the parents concerned with symptoms • consistent with decreased hearing (TV volume too loud, child does not respond when called, often asks “what?”)In adults: aural fullness, conductive hearing loss• No pain•

52

3.4.4. Diagnosis

Otoscopic examination reveals a translucent, yellowish tympanic membrane, with decreased mobility, air-fl uid levels,

serous middle ear fl uid, prominence of the lateral process of the malleus, a more horizontal orientation of the malleus. If the patient is asked to perform a Valsalva maneuver, air will be infl ated within the middle ear cavity, and air bubbles can bee seen in the fl uid behind the eardrum. Evaluation of hearing: tuning fork tests, audiogram (conductive air-bone gap up to 40 dB), tympanometry (typical type B fl at curve).

An accurate and complete ENT examination must be performed. Endoscopy of the nasopharynx is mandatory, mainly in adults with unilateral disease, to rule out a nasopharyngeal malignancy!

3.4.5. Evolution. Complications

Spontaneous recovery• Chronic otitis media with eff usion: the viscosity of the • fl uid increases (“glue ear”)Recurrent episodes of AOM caused by viral/bacterial • infection of the eff usionRetraction pockets, cholesteatoma•

3.4.6. Treatment

Th e recommended European standard 16 follows:


A trial of antibiotic therapy as for AOM is benefi cial• Nasal decongestants (drops/sprays) to alleviate the • dysfunction of the Eustachian tube

Figure 28. Otitis media with eff usion (air-fl uid level)

53

Antihistamines (10 mg loratadine per day for adults and • children older than 12 years)Eustachian tube ventilation exercises: autoinfl ation • (Valsalva maneuver), infl ation with a Politzer bag (Politzer’s method), or infl ation with a catheter introduced through the nasal fossae into the nasopharynx at the level of the Eustachian tube opening


Myringotomy, followed by the insertion of a ventilating • tube (grommet, pressure equalization tube PET). Th is procedure is indicated in cases with an air-bone gap higher than 30 dB, unresponsive to conservative treatment for 3 months, and in cases of development of retraction pocketsAdenoidectomy•

3.5. Chronic suppurative otitis media

3.5.1. Simple chronic suppurative otitis media

(SCSOM)


SCSOM is a chronic infl ammatory condition of the mucosa covering the middle ear cleft, accompanied by a long-standing central perforation, with permanent or intermittent otorrhoea (active and inactive stages). It diff ers from chronic OME, which is defi ned by a middle ear eff usion, without perforation, reported to persist more than 1-3 months. Th e involved micro-organisms 16:

Pseudomonas aeruginosa• (60-80%)Staphylococcus aureus • (10-25%)Proteus • (10-20%)Streptococcus viridans• Enterobacter•

3.5.1.2. Pathophysiology

SCSOM may be the result of an AOM which has left a permanent unhealed perforation. In most cases, these long-standing central perforations

54

occur in patients with repeated episodes of infection owing to Eustachian tube dysfunction. Th is chronic dysfunction is the result of adenoid hypertrophy with repeated adenoid infections, impaired nasal breathing (septal deviations, allergy), chronic rhino-sinusal infections, cleft palate, immune defi ciencies. Th e chronic perforation of the eardrum may be accompanied by destructive lesions of the ossicular chain.

3.5.1.3. Symptoms

Chronic otorrhoea: mucoid, muco-purulent in acute stages. • Th e ear discharge may be constant, but it may dry up at times to reappear with the onset of upper respiratory tract infections or accidental entrance of water into the earHearing loss• Otalgia: only in acute exacerbations•

3.5.1.4. Diagnosis

Otoscopic examination reveals a typical central perforation, located always in pars tensa. Th e word central means that the perforation does not involve the eardrum margins! Th e shape of the perforation may be round, oval, kidney-shaped, sub-total. In active stages a muco-

purulent otorrhoea is visible. After suctioning the ear secretions, we may notice the infl amed mucosa of the middle ear cleft through the perforation, granulation tissue around the margins of the perforation. Hearing assessment: tuning fork tests, pure-tone audiometry (conductive hearing loss; a sensorineural component is not common, but is possible during evolution). A complete and careful ENT examination must

be performed, including endoscopic control of the nose and nasopharynx! Other useful diagnostic procedures: X-ray Schuller (may reveal a sclerotic mastoid or a reduction in the number of cells),

Figure 29. Simple chronic otitis media (central perforation of the

eardrum)

55

paranasal sinuses X-ray, culture swab from the ear discharge with antibiogram.

3.5.1.5. Evolution. Complications

Th is type of chronic suppurative otitis media is not characterized by development of serious life-threatening complications. Th at’s why it is called “simple CSOM”. Th e main complications are:

Acute exacerbations: after accidental entrance of water in • the ear, or after upper respiratory tract infectionsDiff use external otitis: due to chronic otorrhoea• Ossicular resorption or fi xation• Tympanosclerosis: calcifi ed white plaques confi ned to the • level of pars tensa. Th ese lesions may be limited to the eardrum or may involve the whole middle ear cavity with subsequent blockage of the ossicular chainMixed hearing loss (inner ear toxic involvement)•

3.5.1.6. Treatment:

Th e recommended European standard16 follows:


Periodic aggressive aural toilet: daily suction under • microscopic controlTopical treatment: quinolone ear drops are routinely • prescribed twice or three times daily, for 7 to 10 daysOral or parenteral antibiotics may be occasionally • benefi cial for treating particularly active and resistant drainageAdditional/useful therapeutic options: treatment of the • nose, paranasal sinuses or nasopharynx, elimination if possible of allergic factors

Patients with SCSOM respond more frequently to topical than to systemic therapy. Successful topical therapy consists of three important components: selection of an appropriate antibiotic drop, regular aggressive aural toilet and control of granulation tissue. Ciprofl oxacin remains the most eff ective of the quinolones against Pseudomonas. Fluoroquinolones are not approved for use in children because they elicit joint injury in juvenile experimental animals.

56

Th e control of granulation tissue involves the use of topical antibiotic-steroid drops, chemical cautery using silver nitrate, excision using microinstruments.


Myringoplasty and tympanoplasty without • mastoidectomy: if the perforation is dry, repair the eardrum perforation (myringoplasty) and eventually the ossicular chain lesions (tympanoplasty)Tympanoplasty with mastoidectomy: if suppurative • drainage persists and the mastoid is involved

3.5.2. Cholesteatoma of the middle ear


Cholesteatoma is recognized for decades as a destructive lesion of the skull base that can erode bone and damage important structures within the temporal bone. Its potential for causing central nervous system complications makes it a serious life-threatening disease. A cholesteatoma consists of keratinizing squamous epithelium that is trapped within the temporal bone (“skin in the wrong place”). It has a matrix which continually desquamates and sheets of exfoliated keratin debris which accumulate and form the bulk of the cholesteatoma.

3.5.2.2. Pathophysiology:

Th ree patterns of cholesteatoma formation are described 16:Congenital cholesteatoma: cholesteatoma is rarely a • primary lesion. Th is type is characterized by the presence of a cholesteatoma behind an intact eardrumPrimary acquired cholesteatoma: usually develops in • relation with a chronic Eustachian tube dysfunction. Th e result is a persistent negative pressure in the middle ear cleft and the development of retraction pockets (which may perforate). Th ese retractions are mostly located at the level of pars fl accida or in the postero-superior quadrant of pars tensa. Local infl ammation processes (Pseudomonas aeruginosa, Proteus species) stimulate the production of keratin, the exfoliation and thus the cholesteatoma

57

growth. Cholesteatoma has a great potential for bone erosion. Th erefore, local destructive processes may develop during evolution, leading to serious complicationsSecondary acquired cholesteatoma: occur as a direct • consequence of some type of injury of the tympanic membrane: perforation after AOM (unsafe marginal perforation), trauma or after ear surgery (iatrogenic)

3.5.2.2. Symptoms

Chronic foul-smelling muco-purulent otorrhoea• Hearing loss• Otalgia (acute exacerbations)• Tinnitus• Dizziness, vertigo (it may suggest the presence of a • labyrinthine fi stula)Headaches (intracranial complications?)•

3.5.2.3. Diagnosis

Otoscopy reveals a foul-smelling otorrhoea, which must be removed (suction under microscopic control) before the eardrum can be accurately examined. Th e tympanic membrane perforation is marginal, located in pars fl accida or at the level of the postero-superior quadrant of the eardrum. A white mass appears from the perforation, discharging white scales. Sometimes the visibility of the eardrum is obscured by the presence of granulation tissue or aural polyps. Audiometry: Pure-tone and speech audiometry are performed to evaluate the degree of conductive and sensorineural hearing loss. An ear swab culture may be indicated in infected cholesteatomas. Radiologic examinations: Schuller X-ray, High-resolution CT scans, MRI Vestibular testing: in case of suspicion of labyrinthine fi stula.

Figure 30. Attic cholesteatoma

58


Sometimes cholesteatoma is fi rst diagnosed based on symptoms and signs determined by a serious complication: facial palsy, headaches, fever, vertigo. Its propensity for causing central nervous system complications makes it a potentially fatal lesion.

3.5.2.4.1. Extracranial complications:

Sensorineural hearing loss: it has been suggested that • toxins in chronic suppurative otitis media can damage the cochlea23

Labyrinthine fi stula, labyrinthitis: fever, dizziness, vertigo, • nystagmus, fi stula signMastoiditis with sub-periostal abscess: fever, fl uctuance • over the mastoid area, lateral displacement of the pinna. It may present as Betzold’s abscess, which represents extension of the abscess from the mastoid tip into the digastric groove (the pus erodes the cortical bone of the mastoid tip and exteriorizes under the superior insertion of the sternocleidomastoid muscle) Petrositis: retro-orbital pain, persistent aural discharge • and diplopia caused by cranial nerve VI paralysis (the Gradenigo syndrome)Facial nerve palsy•

3.5.2.4.2. Intracranial complications:

are usually the result of direct spread of infection from the ear to the middle or posterior cranial fossae. Th is spread is favoured by bone erosion, congenital dehiscences, thrombosis of the veins between the middle ear and the meninges.

Extra-dural abscesses, sub-dural abscesses• Meningitis: headaches, fever, photophobia, neck • rigidity, Kernig’s and Brudzinski’s signs, positive lumbar punctureBrain abscess: localized in the temporal lobe or in • the cerebellum (headaches and nausea, seizures, focal neurologic signs, signs of intracranial hypertension)Sigmoid sinus thrombosis: rarely seen nowadays, this • complication does still occur and is easily overlooked because of the masking eff ects of antibiotic therapy (septic-

59

type fever, headaches, anemia, edema and tenderness over the posterior aspect of the mastoid bone)Otitic hydrocephalus: increased intracranial pressure in • the absence of a brain abscess

Modern imagistic techniques (CT scans, MRI) are mandatory for an accurate diagnosis!

3.5.2.5. Treatment


Periodic observation may be indicated in limited cases of • a dry cholesteatoma in the only functional ear, in elderely patients or in individuals with very poor health 16

Medical therapy is not a viable treatment for cholesteatoma, • it doesn’t stop further expansion and does not eliminate riskTh e mainstay of anti-microbial therapy should be topical, • but systemic therapy is occasionally a helpful adjunct


Virtually all cholesteatomas should be excised! Surgical therapy consists of cholesteatoma removal. Th e aims of surgery are eradication of disease, an epithelialized self-cleaning ear and hearing preservation or improvement 24. Two types of surgical procedures are available:

Canal wall down mastoidectomies (open techniques): the • traditional method for cholesteatoma removal was the modifi ed radical mastoidectomy, in which the mastoid is opened behind the external canal, the cholesteatoma is identifi ed and followed forwards through the aditus ad antrum into the attic, with the removal of the posterior bony wall of the external canal. Th e fi nal result is a large cavity. Smaller cavities may be obtained using the anterior to posterior endaurally approach (attico-antrostomies)Canal wall up mastoidectomies (closed techniques) have • the advantage of leaving an intact external canal and no mastoid cavities

Reconstructive procedures for hearing improvement (myringoplasties, tympanoplasties) may be performed during initial surgery or at a later stage. Canal wall down techniques have lower rates of recurrences

60

of cholesteatoma (5-15%), and recurrences are easily diagnosed in the outpatient clinic, so second look operations are not required. In canal wall up procedures the rate of recurrences is higher (20-50%). Th erefore a second look surgery after 12-18 months is recommended in all cases 24.

3.6. Otosclerosis


Otosclerosis is a localized hereditary disease aff ecting the endochondral bone of the otic capsule, that is characterized by disordered resorption and deposition of bone. An otosclerotic lesion consists of areas of bone resorption, new bone formation, vascular proliferation and a connective tissue stroma 25. Th e most common site for otosclerosis lesions is the area anterior to the oval window (80-95% of cases), followed by the round window niche (30% of cases) and the stapes footplate (12% of cases) 26. Th e fi nal result in evolution is the fi xation of the stapes footplate in the stapedio-vestibular joint, with subsequent conductive or mixed hearing impairment. Ankylosis of the stapes was fi rst described by Valsalva in 1704. Politzer described this condition as an otic capsule disorder, characterized by abnormal new bone formation. Factors involved in otosclerosis aetiology:

Genetic predisposition: otosclerosis is most common • among Caucasians, uncommon among Asian people and extremely rare in black people. Women are two times more likely to develop the clinical disease than men27. Familial aggregation of individuals aff ected by otosclerosis has been recognized for many ears. New evidences suggest some otosclerosis cases may be related to defects in expression of the COL1A1 gene 28

Measles virus infection: measles RNA has been found • in fresh footplate specimens with otosclerosis. Increased levels of anti-measles antibodies have also been reported in perilymph from patients undergoing stapedectomy, as compared to controls 29

Autoimmune disease: autoimmunity to type II collagen •

61

or a closely related antigen that is present in the regions of predilection25

Hormonal factors: pregnancy sometimes accelerates the • progression of the disease

3.6.2. Symptoms

Often a family history is revealed• Onset of symptoms: 2-3• rd decade of lifeSlow-progressive unilateral (70%) or bilateral (30%) • hearing lossTinnitus (60%)• Vestibular complaints•

3.6.3. Diagnosis

Otoscopic examination: the main clinical feature of otosclerosis is a progressive conductive/mixed hearing impairment with a normal tympanic membrane! Hearing tests:

Tuning fork tests (Weber, Rinne)• Pure-tone audiometry: pure conductive hearing loss • (30%), mixed hearing loss (70%). In 20% of patients there is an elevation of 20-30 dB of the bone conduction threshold called Carhart’s notch19

Tympanometry: normal (type A), stapedius refl ex is • absent because of stapes fi xationSpeech audiometry•

Imaging techniques: high-resolution CT scans are capable to demonstrate the otosclerotic foci within the temporal bone.

3.6.4. Diff erential diagnosis

Infl ammatory conditions of the middle ear• Post-traumatic lesions of the ossicular chain• Tympanosclerosis• Malleus head fi xation• Osteogenesis imperfecta • (van der Hoeve syndrome): classic triad of hearing loss (conductive, mixed), spontaneous bone fractures, and blue scleraPaget disease of the bone: ear symptoms are identical to • otosclerosis

62

Superior semicircular canal dehiscence: a recently • described condition resulting in a low frequency “inner ear” conductive hearing loss19

3.6.5. Treatment


If the air-bone gap is less than 20 dB, no treatment is • requiredHearing aids: if the patient refuses surgical treatment or • has strong contraindications for surgerySupplementation of fl uoride•

3.6.5.2. Surgical treatment

Surgical treatment is recommended mainly in young patients with an air-bone gap higher than 20 dB.

Stapedectomy: after total removal of the stapes footplate • and stapes suprastructure, the oval window is covered with a graft and a commercial prosthesis is placed between the incus and the graftStapedotomy: after removal of the stapes suprastructure, • a small perforation of the footplate is created using a micro-drill or the laser, followed by the insertion of the prosthesis into the calibrated hole and fi xation of the prosthesis to the long process of the incus

63

4. DISEASES OF THE INNER EAR

4.1. Sudden sensorineural hearing loss (SSHL)


SSHL is characterized by a ≥ 30 dB hearing loss in ≥ 3 contiguous frequencies, with the loss occuring ≤ 3 days30. Sometimes, the hearing loss is perceived by the patient on awakening. SSHL is an ENT emergency! Regarding the aetiology, we may refer to SSHL of a specifi c cause (clearly identifi ed from history, clinical examination and specifi c investigations), and idiopathic SSHL. In less than 5% of cases a specifi c cause is found31. Specifi c causes of SSHL:

Infections: viral (mumps, measles, herpes virus type • II, herpes zoster, HIV), bacterial (syphilis, Borellia burgdorferi, meningitis, encephalitis)Vascular: haemorrhage, vascular occlusion, thrombosis, • spasmTrauma: ear surgery, head injury, blast injury (an explosive • blast may produce injury both as a result of the high intensity of the noise and the shock wave), perilymph fi stula (abnormal communication between the perilymphatic space and the middle ear, most commonly occurs at the level of the round window membrane, the leak is usually associated with vestibular symptoms, could be determined by a minor trauma like barotraumas, physical exertions such as straining, lifting, coughing, vomiting)Tumors: acoustic neuromas• Autoimmune • Neurological: multiple sclerosis• Haematological: hyperviscosity syndromes, sickle cell • disease

64

4.1.2. Symptoms:

Abrupt unilateral hearing loss occurring in less than three • days, or apparent on awakeningTinnitus (85% of cases)• Sometimes the hearing loss is associated with vestibular • symptoms: vertigo, nausea, vomiting (30% of cases)

4.1.3. Diagnosis:

A detailed history is extremely important!• Complete ENT examination• Hearing and vestibular assessment• Imaging techniques: MRI (to rule out an acoustic • neuroma, multiple sclerosis)Blood cell count, haemoglobin, erythrocyte sedimentation • rate (ESR), lipid profi le, creatinine, urea and electrolytes, glucose, auto-antibodies, serologic tests for syphilis, Borellia, herpes viruses, HIVInternal medicine, neurological, endocrinological examinations•

4.1.4. Evolution. Prognosis

Complete spontaneous recovery is seen in about 50% of the patients31. Following treatment we may have a complete recovery, a partial recovery or no recovery. Th e presence of associated vestibular symptoms worsens the prognosis.

4.1.5. Treatment


Empirical treatment is usually initiated. Many regimens have been proposed, but there is no fi rm evidence to support the eff ectiveness of any of them. Th e most frequently used agents in the conservative management of SSHL 31:

Short-term glucocorticoid steroids (Hydrocortisone • sodium succinate i.v., Prednisolone i.v., intratympanic Dexamethasone, oral steroids) Agents to improve cochlear blood fl ow: Dextran, • hydroxyethyl starch (HES), Pentoxifylline i.v., Procaine i.v.

65

Vasoactive agents: calcium channel blockers, Histamine, • Nicergoline (Sermion)Vitamins and antioxidants: vitamin B1, B6, B12, vitamin • C, A, E, nicotinic acid, α-lipoic acidOthers: antivirals (Acyclovir, Interferon), ATP, CO•

2/O

2

inhalation (Carbogen), Ginko extracts, hyperbaric oxygen


In cases of fl uctuating unilateral sensorineural loss associated with vestibular symptoms, a tympanoscopy should be performed in order to rule out a perilymphatic fi stula. If a fi stula is discovered, it should be sealed with a connective tissue graft (e.g. fat harvested from the ear lobule).

4.2. Progressive sensorineural hearing loss

4.2.1. Presbyacusis

4.2.1.1. Defi nition. Aetiopathology

Presbyacusis or age-related hearing loss is a mid- to late-adult onset, bilateral, progressive sensorineural hearing loss 32. It is more or less symmetric and starts in the higher-frequency range, with or without tinnitus. Th e hearing impairment is determined by the normal, physiological process of aging, based on an individual genetic predisposition and, on the other hand, by exogenous degeneration of the inner ear structures (supporting cells, basilar membrane, outer hair cells). Th is degeneration is the result of various environmental infl uences, nutritional habits, toxicities etc. A hereditary component of prebyacusis has recently been demonstrated33.

4.2.1.2. Symptoms

Slow• -progressive hearing loss with insidious onset in the 5th-6th decades of life, decreased speech comprehension, mainly in ambient noise, discomfort in noisy environment, during phone calls, the TV is often louder than is comfortable for the others members of the family

66

Tinnitus (30-50% of cases) may be the most annoying • symptomTh e hearing disability, the loss of ability to communicate • and the presence of tinnitus may fi nally lead to behavioral alterations, psychosocial isolation

4.2.1.3. Diagnosis

Detailed• and accurate historyComplete ENT examination• Hearing assessment: tuning-fork tests, pure-tone • audiogram, speech audiometry, tympanogram, auditory brain-stem responses (ABR)

4.2.1.4. Treatment

Unfortunately, there’s no therapeutic modality to replace the hearing that has been lost. Medical therapy is indicated only when the patient reports a rapid deterioration of the hearing loss or annoying tinnitus. Th e best management solution for presbyacusis is the prescription of binaural hearing aids associated with psychological counselling.

4.2.2 Noise-induced hearing loss

4.2.2.1. Defi nition. Aetiopathology

Th e term noise-induced hearing loss refers to a reduction in auditory acuity associated with noise exposure. Th e term “acoustic trauma” has been used to describe the situation where a single exposure to an intense sound leads to an immediate hearing loss34. Chronic exposure to noise causes metabolic and mechanical structural damage at the level of the cochlea, leading subsequently to neuronal degeneration. Other involved factors:

Apoptosis and necrosis: there is evidence that apoptosis • (programmed cell death) play an important role in noise-induced hearing loss35

Genetic susceptibility: there is experimental evidence • from studies with mice, the Ahl gene being involved36

Smoking, diabetes, cardiovascular diseases• Th e noise-induced hearing loss is a result of working in

67

noisy environments (metal workers, mine workers, airports, disc jockeys, military personnel, etc.). Typically, the hearing loss initially occurs as a high-frequency SNHL, and a characteristic sensorineural notch (normally at 4 kHz) is visible on the pure-tone audiogram. Mid-frequencies, and low-frequencies are aff ected much later.

4.2.2.2. Symptoms

Progressi• ve bilateral hearing loss: at the debut the patient’s description involves a lack of clarity rather than a loss of volume. In time, the patient is confronted with problems of communication in noisy environments, problems during phone conversations, the TV set is louder than comfortable for the rest of the familyTinnitus (70% of cases)• 33

Th e hearing disability, the loss of ability to communicate • and the presence of tinnitus may fi nally lead to behavioral alterations, psychosocial isolation

4.2.2.3. Diagnosis

Detai• led patient history, including professional and recreational sound exposure

Figure 31. Noise-induced hearing loss

( —< : bone conduction right ear; — : air conduction right ear; >— : bone conduction left ear; ×— : air conduction left ear; red arrrow: characteristic notch at 4000 Hz)

68

Otological e• xamination is normalA complete ENT examination should be performed• Hearing assessment: tuning fork tests, pure-tone • audiometry (the characteristic sensorineural notch at 4000 Hz), speech audiometry, objective tests

4.2.2.4. Treatment

A medical therapy to recover the hearing loss is not available. Steroids and vasodilators may be indicated in the management of annoying tinnitus. As the hearing loss becomes more severe, a hearing aid should be recommended. Prevention and counselling are very important: avoid noise exposure if possible, noise surveys for the employers, periodic hearing assessment, personal protection (earplugs, earmuff s).

4.2.3. Ototoxicity


Ototoxicity is a chemical injury to the labyrinth occurring as a side eff ect of pharmacotherapy. An ototoxic insult may aff ect the hearing, the vestibular function or both 37. Th e most frequently encountered ototoxic agents are the aminoglycoside antibiotics and the chemotherapeutic agent Cisplatin (table 4).

Drugs Primarily ototoxic Primarily vestibulotoxic

StreptomycinDihydrostreptomycinNeomycinGentamicinLoop diureticsSalicylateCisplatin

+++++++

+++

++

+++++

++++--

Table 4. Ototoxic compounds in clinical practice 33

Th ere is evidence for a genetic susceptibility to aminoglycoside ototoxicity38. Aminoglycoside toxicity may occur after systemic administration or topical application to the tympanic cavity.

69

Drops containing aminoglycosides should not be administered in the presence of a tympanic membrane perforation. Th e vestibulotoxic eff ect of trans-tympanic gentamicin is used as a therapeutic method in Meniere’s disease.

4.2.3.2. Symptoms

Bilateral, symmetric, progressive hearing loss• High-pitched tinnitus• Vertigo, dizziness, nausea•

4.2.3.3. Diagnosis

Otologic examination is normal• Hearing evaluation: bilateral symmetric sensorineural • hearing lossVestibular function evaluation•

4.2.3.4. Prevention. Treatment

Th ere are strong evidences in the medical literature about the protective eff ects of antioxidants and iron-chelators. Th is effi cacy refl ects the role of oxidative stress in ototoxicity39. In patients with renal failure, doses should be accurately adjusted. Permanent hearing loss is treated with a hearing aid or a cochlear implant. Vestibular toxicity is best managed by vestibular rehabilitation.

4.2.4. Hearing loss in children


Hearing impairment in childhood refers to any unilateral or bilateral hearing loss, occurring from birth to late childhood. Th e hearing loss may be present at birth (congenital), or may be acquired after birth. Because the hearing impairment aff ects the acquisition of speech, it is classifi ed in relation to the stages of speech development as prelingual (0-2 years of age), perilingual (2-4 years of age) and postlingual (more than 4 years)33. Th e hearing loss can be conductive, sensorineural or mixed. Th e most important causes for hearing loss in childhood are:

70

Conductive hearing loss determined by otitis media • with eff usion secondary to repeated episodes of upper respiratory tract infections (see Sect. 3.4.)Other causes for conductive hearing loss: chronic • suppurative otitis media, malformations of the external canal and middle earCongenital sensorineural hearing loss: a genetic cause • accounts for more than 50% of these cases. Almost three quarters of these are non-syndromic; one quarter are part of a hereditary syndrome carrying other specifi c features besides deafness. In most of the cases the hereditary course is autosomal recessive. Th e commonest fi ndings are mutations in the gene GJB2, coding for connexin 26, a gap junction protein located in the inner ear which is essential for the maintenance of the endo-cochlear potential 40. Other causes for congenital hearing impairment are infections during pregnancy (rubella, cytomegalovirus, toxoplasmosis), inner ear malformationsAcquired sensorineural hearing loss: in the perinatal • period it may be caused by hypoxemia, severe infections, prolonged newborn icterus. Following the perinatal period the commonest aetiological factors are meningitis, infections (measles, mumps) and ototoxicityProgressive sensorineural hearing loss during childhood • may be associated with various genetic syndromes (Pendred, Usher, Alport)

4.2.4.2. Symptoms

Unresponsiveness to sound stimuli from the environment• Delay or absence of speech acquisition •

4.2.4.3. Diagnosis

Th e recommended European standard 33:General neonatal screening is widely recommended • (European consensus conference 1998). Methods available for screening are the otoacoustic emissions (OAE) and the auditory brain-stem responses (ABR)History and physical examination: note the presence of • associated cranio-facial abnormalities, as part of various hereditary syndromes (Treacher Collins, Waardenburg etc.)

71

Hearing assessment: subjective auditory tests (behavioural • response audiometry can be performed from 0-2 years, pure-tone audiometry can be performed starting from 4 years, speech audiometry) and objective measurements (tympanometry, OAE, ABR)Imaging techniques: high-resolution CT scans, MRI• Evaluation of speech development• Neurological and ophthalmological examination• Genetic testing and counselling•

4.2.4.4. Treatment


Monitoring the children’s development• Fitting of hearing aids: as soon as possible after the • hearing impairment has been confi rmed


Conductive hearing loss: surgery may be performed in • cases of OME unresponsive to conservative treatment (myringotomy with grommet’s insertion), cholesteatoma, ear malformationsIn children with severe or profound sensorineural hearing • loss, cochlear implantation must be taken into account

4.2.5. Meniere’s disease

See section 5.3.1.•

4.3. Inner ear infections

4.3.1. Bacterial labyrinthitis


Bacterial labyrinthitis may be the consequence of the dissemination of the infection from the middle ear space (AOM, cholesteatoma with perilymphatic fi stula), or from the subarachnoid spaces during the evolution of a purulent meningitis. Th e most common agents involved: β-haemolytic streptococci, pneumococci, staphylococci, Haemophilus infl uenzae, Proteus vulgaris and Pseudomonas aeruginosa41.

72

According to Schuknecht 42, bacterial labyrinthitis can be classifi ed into three stages based on the pathogenesis:

Serous labyrinthitis is an irritation of labyrinth induced • by the bacterial toxins invasion either from an acute or chronic otitis media, labyrinthine fi stula or meningitis, leading to vertigo and sensorineural hearing lossAcute suppurative otogenic labyrinthitis (a rare condition • nowadays) is the consequence of bacterial invasion into the inner ear from the middle ear clefts, the fi nal outcome being the development of a destructive peripheral cochleo-vestibular syndromeSuppurative meningogenic labyrinthitis: implies a • dissemination of bacteria from the subarachnoid spaces

4.3.1.2. Symptoms

Otogenic labyrinthitis: severe or complete hearing loss, • vertigo, nausea, vomiting, high feverMeningogenic labyrinthitis: classic symptoms of meningitis, • severe vertigo, nausea, vomiting, unilateral or bilateral often fl uctuating hearing loss or complete deafness

4.3.1.3. Diagnosis

Recommended European standard33:Otoscopic examination: purulent otitis media, • cholesteatomaHearing evaluation• Vestibular function evaluation• High-resolution “emergency” CT scan• Microbiology: cultures taken from the ear discharge or from • the nasopharynx, CSF diagnostic, serology for syphilis

4.3.1.4. Treatment


Wide-spectrum antibiotics in high-doses• Vestibular suppressant medications and antiemetics•


Myringotomy• Mastoidectomy, labyrinthectomy•

73

4.3.2. Viral labyrinthitis


Th is condition develops during the evolution of a viral upper respiratory tract infection including the middle ear (infl uenza viruses, parainfl uenza viruses, picornaviruses, respiratory syncytial viruses, adenoviruses, coronaviruses etc.).

4.3.2.2. Clinical picture

Th e clinical presentation is of an upper respiratory infection, associated with an acute disturbance of auditory and vestibular function, with vertigo and nystagmus lasting for three to fi ve days43. Varying degrees of permanent hearing loss may be noted.

4.3.2.3. Diagnosis

Recommended European standard33:Otoscopy: normal, serous otitis media• Hearing and vestibular assessment• CT scan• Cultures taken from the nasopharynx, CSF diagnostic, • serology for rubella virus, paramyxoviruses, infl uenza virus, adenoviruses, syphilis

4.3.2.4. Treatment

Glucocorticoids i.v.• Antibiotics to prevent bacterial superinfection • Vestibular suppressant medication and antiemetics• Treatment of the rhinogenic infection: nasal sprays, • mucolytics

4.3.3. Mumps

Deafness appears in as much as 4 per cent of adult cases of epidemic parotitis44. It is often severe and usually unilateral. Vertigo has also been reported in mumps45.

4.3.4. Otosyphilis

4.3.4.1. Defi nition. Aetiology. Pathology

Th e bacterial agent involved is spirocheta Treponema pallidum. Syphilis may determine a meningo/labyrinthitis or an osteitis of

74

the temporal bone with secondary changes of the membranous labyrinth, characterized by the development of an endolymphatic hydrops (abnormal increase of endolymph pressure) and degenerative changes of the sensorineural structures41.

4.3.4.2. Symptoms

Hearing loss: may be with sudden onset, or slowly-• progressive and fl uctuatingTinnitus• Vertigo, nausea, vomiting•

Symptoms are similar to Meniere’s disease, with attacks of vertigo lasting hours and fl uctuating progressive sensorineural hearing loss.

4.3.4.3. Diagnosis

Otoscopy: normal• Hearing and vestibular assessment• Serology for • Treponema pallidumCT scans•

4.3.4.4. Treatment

Penicillin G• Vestibular suppressants and antiemetics•

4.4. Management of hearing impairment

Management of patients with a hearing loss can be determined by the degree of the hearing impairment, irrespective of whether the impairment is sensorineural, conductive or mixed. For ears with a mild, moderate or severe impairment, hearing aids are the main option. For those with a profound or total impairment, cochlear implants are the more suitable management strategy. For all subjects, some accessory devices can be of benefi t46. For the child with a congenital hearing loss, the problem is more complicated because the child has not yet learned the symbols of the language system. Th e main objective is not to restore a skill that once existed (as in adults), but to help the child develop a new skill, the ability to communicate.

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4.4.1. Conventional hearing aids

Hearing aids are described according to where they are worn. Nowadays, six types of hearing aids are available: body aid, eyeglass (spectacle) aid, behind-the-ear (BTE) aid, in-the-ear (ITE) aid, in-the-canal (ITC) aid, and completely-in-the-canal (CITC) aid47. Th e hearing aid is referred to as an electroacoustic device. Initially, an ear-level microphone converts the acoustic signal (such as speech sound) into an electrical signal. Th e electrical signal is amplifi ed and manipulated in various ways by a processor, and fi nally is reconverted to an acoustic signal by a receiver, which is delivered to the ear canal of the wearer47. Hearing aids can be classifi ed by the way they process the sounds into analogue, digitally programmable analogue and fully digital types.

4.4.2. Bone-anchored hearing aids

Some patients with hearing loss are unable to use conventional air conduction hearing aids because of pinna abnormalities, atresia of the external auditory canal or chronic discharging ear diseases. In these conditions, bone conduction hearing aids represent a viable alternative. Th e traditional bone conduction hearing aids deliver amplifi ed sound to the cochlea through a bone vibrator placed on the mastoid. Th ey were used formerly with body or eyeglasses aids. In recent years, the bone-anchored hearing aid (BAHA) has been developed, and appears to avoid many of the disadvantages of conventional bone conduction hearing aids. BAHAs provide mechanical vibration that is transmitted to the skull by way of a titanium screw embedded in the mastoid. A small titanium implant in the skull, behind the ear, osseointegrates (bonds) with the living bone. An abutment is attached to the implant and a sound processor is clipped on. Th e processor can be worn or taken off at any time48.

4.4.3. Cochlear implants

In the 1980s, the cochlear implant emerged as a reliable alternative to conventional amplifi cation for individuals with profound hearing impairment.

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Th e cochlear implant is a device that is surgically implanted, with its stimulating electrode array (wire) inserted directly into the cochlea. Th e implant consists of 1 to 22 channels. Th e electrode is used to stimulate the auditory nerve directly with electric current, bypassing the damaged cochlear structures47. For older children and adults, the ideal candidates are those who acquired profound bilateral sensorineural hearing loss after acquiring language. Cochlear implants, however, appear to hold even greater promise for profoundly impaired children under 2 or 3 years of age, although research regarding the comparative benefi ts of rehabilitative devices is still in progress. Cochlear implantation can be performed as early as 8-12 months, if indicated. Th e presence of viable auditory nerve fi bers is fundamental to the success of cochlear implantation49. Cochlear implantation is not possible if the auditory nerve is absent. In these cases, brainstem implants may be an alternative approach33. Preoperative imaging is mandatory. Two modalities are available: high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI). MRI is more accurate at identifying cochlear dysplasia and the presence of the cochlear nerve. Th e most common abnormalities encountered are the ossifi ed cochlea (most frequently after meningitis) and the cochlear dysplasia (1.5% of the cases)46. A variety of surgical approaches have been developed to access the scala tympani of the inner ear, the preferred placement for the implant. Almost all surgeons use a transmastoid approach. Children with cochlear implants require regular programming and control of the speech processor, which is best ensured in multidisciplinary cochlear implant rehabilitation programmes. Additional speech and language therapy is necessary, together with general support, careful choice of educational settings and parents counselling 33.

4.4.4. Middle ear implants

Middle ear implants represent a modern alternative to conventional amplifi cation aids. Current devices are suitable for patients with mild to severe sensorineural hearing loss. Th e hearing loss should ideally be stable; however, very slowly

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progressive losses can be considered. Classically, a normal middle ear function is required. Th e indications are expanding and extended applications are being explored. Devices have been applied to stimulate the round window membrane directly, in various types of conductive and mixed hearing loss. Th e device consists of a microphone, an amplifi er and a transducer. Th e transducer uses either a coil and a magnet (electromagnetic) or a piezoelectric mode of transmission, and is connected to one of the middle ear ossicles, or cochlear windows46. A major problem of these devices has been to produce a device that is small enough to fi t within the middle ear cavity, and yet powerful enough to supply the required gain. Current devices available50 are:

Vibrant Soundbridge (Symphonix, Med-El): an active • semi-implantable device, consisting of an internal, surgically implanted vibrating ossicular prosthesis (VORP), coupled to the long process of the incus, and an external audio processorOtologics• TM middle ear transducer (MET): a fully implantable device, consisting of a subcutaneous microphone and an electronic receiver connected to a transducer, coupled to the body of the incusEsteem• R-Hearing ImplantTM: a fully implantable piezoelectric device, comprising a piezoelectric sensor on the incus body and a driver cemented to the stapes head; the implantation requires disarticulation of the ossicular chain

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5. PERIPHERAL VESTIBULAR SYNDROME

5.1. Vertigo

Th e most important clinical feature in the diagnosis of peripheral vestibular disorders is its pattern of presentation. A detailed and accurate history is of paramount importance! Vertigo is the main symptom of the peripheral vestibular syndrome. It is characterized by an acute sensation of abnormal rotatory movement of the surroundings in relation to the patient or of the patient in relation to his/her surroundings (“suddenly everything spins round”). It is accompanied by vegetative symptoms: nausea, vomiting, sweating, tachycardia, but never loss of consciousness. It is very important to diff erentiate the true vertigo from other chronic disequilibrium sensations described by patients in various ways: dizziness, giddiness, light-headedness etc. It has been pointed out that even the most enthusiastic doctor may experience a decline in spirits when faced with such patients. A minimum vertigo history should address the following:

Does the sensation of imbalance occur in attacks or is • chronic?Th e duration of the individual attack: seconds, hours or • days?Th e frequency of the crises: daily, monthly?• Th e eff ect of head movements• Is there a specifi c position that brings on the attack? (e.g. • rolling onto the right side in bed)Associated otologic symptoms: hearing loss, tinnitus• Associated neurologic symtoms• Concomitant ear disease: otorrhoea, prior ear surgery, ear • trauma

One of the most important features of the pattern of presentation is the duration of the attack. Based on this, the following classifi cation of peripheral vestibular disorders is put forward 5:

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A. Vertigo lasting minutes to hours:Idiopathic endolymphatic hydrops (Meniere’s disease)• Secondary endolymphatic hydrops (Otosyphilis)•

B. Vertigo lasting seconds: benign paroxysmal positional vertigo (BPPV) C. Vertigo lasting days: vestibular neuronitis D. Vertigo of variable duration:

Inner ear fi stula• Inner ear trauma• Superior semicircular canal dehiscence syndrome•

5.2. Examination of the vestibular function

5.2.1. Clinical examination of eye movements

(Nystagmus)

Nystagmus is characterized by involuntary, rhythmic, oscillatory movements of the eyes. Nystagmus arising from vestibular disorders has two components: a slow labyrinthine phase in one direction, and a fast correcting cerebral or voluntary phase in the other. Th e fast phase is the one visible during examination and is used to defi ne the nystagmus’ direction. Th e main nystagmus’ characteristics in the peripheral vestibular syndrome are:

It is always present during the attack• It is nearly always horizontal-rotatory or torsional (the • eyes beat like a car windscreen wiper), and it is named after the direction of the fast phaseIt is unidirectional• It is always directed to the irritated labyrinth, away from • the paretic labyrinthIt is associated with auditory symptoms and vertigo• It is visually suppressed: that’s why it is better visualized • using the Frenzel glasses (20 diopter lenses that magnify the eyes and eliminate eye fi xation)

Nystagmus of central origin beats in any direction, is often vertical or pure horizontal, is not suppressed by visual fi xation, is not accompanied by auditory symptoms, vertigo may or not be present, is associated with other neurological symptoms and signs.

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5.2.2. Romberg test

Th e Romberg test was originally described as a test for tabes dorsalis (neurosyphilis). Ask the patient to stand erect with feet together and eyes closed. It’s very important to stand nearby the patient as a precaution in order to stop the person from falling over and hurting himself or herself ! Watch the body’s deviations in relation to a perpendicular object behind the subject (corner of the room, door, window etc.). If a destructive lesion in the vestibular system is present, the patient tends to fall toward the side of the lesion. So, deviations are always to the paretic labyrinth, opposite to the nystagmus. Th e sharpened Romberg test is performed asking the patient to stand heel to toe, with one foot in front of the other. Th is test is required to detect abnormalities mainly in younger patients51.

5.2.3. Unterberger test

Th e patient is asked to step in place, keeping the eyes closed. Deviations will be always directed to the paretic labyrinth’s side.

Figure 32. Nystagmus

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Figure 33. Romberg test

5.2.3. The Dix-Hallpike manoeuvre

One of the most common causes of vertigo is the so-called benign positional paroxysmal vertigo (BPPV), accounting for 25% of all patients with dizziness and vertigo. Th e Dix-Hallpike manoeuvre is one of the most important tests for patients who experience true vertigo. Th is test involve having the patient seated (with Frenzel glasses, if available) on the examination couch in such a way that when moved into the supine position the head and neck will extend beyond the edge of the couch. Th e head is held by the examiner and is moved abruptly in the supine position, to the left and to the right side. Th is procedure will cause a vestibular attack, so the patient must be warned before. Eye movements are recorded. Th e main characteristics of this nystagmus are1:

Fixed direction• Torsional (horizontal rotatory)• Occurs after a latency period of seconds• Lasts less than one minute•

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Fatigability: weaker and shorter responses when repeating • the procedure

5.2.4. Caloric test

Caloric testing remains the most useful test in determining the responsiveness of a labyrinth. It is one of the few tests that allow one labyrinth to be studied independently of the other 52. Th e principle of the caloric testing is that changes in the temperature of the external ear canal infl uence the activity level of the vestibular labyrinth. Th e ear canal is irrigated with water (syringing) or air. In the conventional procedure two temperatures are used, one above and one below the body temperature. Water irrigation at 30 and 43º C (37 ± 7º C) is the standard original technique, described by Fitzgerald and Hallpike53. Each irrigation lasts 40 seconds. Th e patient lies down with the head raised 30º above horizontal. Th is places the horizontal semicircular canal in an approximately vertical position. Irrigation with cold water induces a nystagmus beating in the opposite direction of irrigation. Warm water causes an ipsilateral beating of the nystagmus54.

5.2.5. Other tests for vestibular function evaluation

Rotational tests: patient is seated in a rotating chair, both • labyrinths being stimulated simultaneouslyElectronystagmography, videonystagmography: the eye • movements can be recorded electrically or using a video cameraPosturography: is an objective Romberg test using the • Luzern platformClick-evoked vestibular myogenic potentials: loud sounds, • brief in duration (clicks) can stimulate the labyrinth

5.3. Vertigo: clinical syndromes

Th e vestibular syndrome can be classifi ed in two main categories:

Th e peripheral vestibular syndrome: its management • belongs to the ENT physicians

83

Th e central vestibular syndrome: its management belongs • to the neurologists

Th e peripheral vestibular syndrome can be further divided in two categories:

Irritative: serous labyrinthitis, perylimph fi stula, Meniere’s • disease (fi rst hours of the fi rst crises)Destructive: trauma, purulent bacterial labyrinthitis, • Meniere’s disease, drugs

Another classifi cation of peripheral vestibular syndromes focuses on the presence or absence of hearing impairment:

With hearing loss: Meniere’s disease, labyrinthitis, • trauma, perylimph fi stula, drugsWithout hearing loss: BPPV, vestibular neuronitis, drugs•

Central Peripheral

ImbalanceNeurologic symptomsNystagmus

Hearing lossNauseaRecovery by central compensation

SevereFrequentChanges direction in diff erent gaze positions; not infl uenced by visual fi xationRareVariable, may be absentSlow

Mild to moderateRareUnidirectional in all gaze positions; suppressed by visual fi xationFrequentSevereRapid

Table 5. Features diff erentiating central from peripheral vestibular syndromes55

5.3.1. Meniere’s disease

5.3.1.1. Defi nition

Meniere’s disease is a disorder of the inner ear characterized by recurrent spontaneous attacks of vertigo, fl uctuating progressive sensorineural hearing loss, tinnitus and often a sensation of aural fullness. It was fi rst described by Prosper Meniere in 1861.

5.3.1.2. Aetiology. Pathology

Meniere’s disease is considered to be idiopathic. Th e main pathological mechanism involved is an overproduction or malabsorption of endolymph, resulting in endolymphatic hypertension. Th is hypertension fi nally results in gross enlargement

84

of the membranous labyrinth (endolymphatic hydrops) 56. Periodic ruptures of the membranous labyrinth, which result in leakage of the potassium-rich endolymph into the perilymph, are supposed to be the trigger factor for Meniere’s attacks 57. Some inner ear diseases (e.g. otosyphilis) may develop during evolution an endolymphatic hydrops (delayed or secondary hydrops) with a clinical picture similar to Meniere’s disease (so-called Meniere’s syndrome). Th e main factors involved in the aethiopathology of Meniere’s disease are:

Obstruction of the endolymphatic duct: the basis for • development of hydrops in experimental animals 5

Autoimmune processes: studies of the human • endolymphatic sac has suggested it is the primary immunocompetent structure of the inner ear, capable of processing antigens, synthesizing antibodies, and generating a cellular immune response 58

Reactivation of a latent viral infection: Herpes simplex • virus type159,60

5.3.1.3. Symptoms

Recurring attacks of spontaneous vertigo, associated with • nausea and vomiting, lasting from several minutes to hoursFluctuating, progressive sensorineural hearing loss• Tinnitus• Aural fullness•

Between attacks patients don’t experience vertigo, but the hearing loss is present and is augmented by the next crisis. Generally, the attacks increase in frequency and severity with progression of the disease.

5.3.1.4. Diagnosis

History is extremely important• Hearing evaluation: tuning fork tests, pure-tone audiometry • (fl uctuating, progressive sensorineural hearing loss with a fl at pattern in later stages), speech audiometry, electrocochleography, auditory brainstem responses (ABR)Vestibular evaluation: between attacks the patient displays • normal fi ndings

85

Imagistic techniques: MRI with Gadolinium, to rule out • a retro-cochlear lesion (vestibular schwannoma)

Th e American Academy of Otolaryngology-Head and Neck Surgery has published diagnostic guidelines for Meniere’s disease61. A diagnosis of “defi nite” Meniere’s disease can be made if the patient has two or more spontaneous attacks of vertigo (each lasting 20 minutes or longer), hearing loss documented on pure-tone audiometry on at least one occasion, tinnitus or aural fullness on the aff ected side, and other possible causes excluded.

5.3.1.5. Treatment

Th e Recommended European Standard 33:


In acute attacks bed rest, vestibular suppressant medication (diazepam) and antiemetics are recommended. To prevent attacks:

Diet: low salt intake (less than 3 g per day) and decreased • water intakeDiuretics: acetazolamide, chlortalidone, hydrochlorothiazide, • furosemideVasoactive drugs (betahistine), to improve blood • circulation in the inner earSteroids, to suppress the infl ammatory and/or allergic • tissue reactions within the labyrinth (endolymphatic sac)Avoidance of alcohol, caff eine, smoking• Antiviral approach: oral acyclovir • 62

5.3.1.5.2. Semiconservative treatment:

Insertion of a transtympanic ventilating tube, followed by • transtympanic unilateral chemical labyrinthectomy with gentamicinIn bilateral cases, intramuscular streptomycin• Following insertion of a ventilating tube, self-administered • treatment with the Meniette device (intermittent low-pressure pulses to stimulate the fl ow of the endolymph)Intratympanic dexamethasone injections• Intratympanic application of antiviral agents (ganciclovir)• 63

86


Endolymphatic sac decompression procedures (saccotomy). • Complete resolution of vertigo is reported in about 50-75% of patients. Th e effi cacy of these procedures was seriously doubted by some authors. Foremost in this controversy is the sham study of Th omsen from 1981, in which the authors performed a simple mastoidectomy with or without an endolymphatic decompression procedure, and reported identical results in both groups5,64

Selective vestibular nerve neurectomy: 98% control rates• Labyrinthectomy: implies a complete hearing loss• Tenotomy: sections of the tendons of tensor tympani • and stapedius muscle in the middle ear cavity (a new approach)

5.3.2. Vestibular neuritis


Vestibular neuritis is an acute condition characterized by a single severe spontaneous attack of vertigo, lasting days, caused probably by a viral infl ammation. A viral infection of the vestibular nerve is considered to be the main aetiological factor. Latent infection with Herpes simplex virus type1 of the superior and inferior vestibular ganglia has been documented65, 66. Due to immunologic defi ciencies, the viruses are reactivated and subsequently destroy vestibular sensory fi bers.

5.3.2.2. Symptoms

Acute spontaneous severe vertigo attack typically lasting • days, with gradual improvement during evolutionNo hearing loss, no tinnitus!• No other neurological symptoms•

5.3.2.3. Diagnosis

History• Hearing evaluation: normal• Vestibular function evaluation: horizontal rotatory • nystagmus beating towards the intact side, Romberg deviations to the aff ected side, caloric testing proves the hypofunction of the aff ected side

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5.3.2.4. Evolution

Th e acute clinical manifestations of the disease invariably subside over the following days as a consequence of central vestibular compensation. Nearly 25 per cent of the patients will develop a BPPV on the aff ected side67.

5.3.2.5. Treatment

steroids, vasodilators, intravenous fl uid support• vestibular suppressants and antiemetics• vestibular rehabilitation•

5.3.3. Benign paroxysmal positional vertigo (BPPV)

5.3.3.1. Defi nition. Aetio-pathology

BPPV is the most common cause of vertigo seen by ENT doctors, representing 20% to 40% of patients with peripheral vestibular disease5. It is a vestibular disorder characterized by brief attacks of vertigo (lasting less than a minute) precipitated by certain changes in head position, and caused by a cupulolithiasis or a canalolithiasis. Otoconia (otoliths) are calcium carbonate crystals which are normally embedded in the gelatinous otolithic membranes of the utricle and saccule. In some conditions (trauma, old age, idiopathically), otoconia are released from these membranes and start to freely fl oat in the endolymph. In a certain head position the otoliths reach and stimulate the ampula of the semicircular canals (posterior canal is the most frequently involved 98%), causing the typical vertigo. BPPV may occur as a complication in the evolution of a head trauma or a vestibular neuritis 67

5.3.3.2. Symptoms

Brief attacks of vertigo, lasting only seconds (typically less • than a minute). Th e attacks are triggered by certain head positions (for example, rolling onto the right side in bed) and may be accompanied by nausea and occasionally vomitingNo hearing loss and tinnitus!• No other neurological symptoms•

88

5.3.3.3. Diagnosis

History• Dix-Hallpike manoeuvre (see section 5.2.3.)•

5.3.3.4. Treatment

Th ere is no medical treatment for BPPV• Th e most eff ective current therapy is organized around the • so-called repositioning manoeuvres (Epley and Semont) that use gravity to move out the otoliths from the aff ected canal into the vestibuleOnly in extremely rare cases (less than 1%) surgical • treatment can be indicated: selective neurectomy of the posterior canal nerve or posterior semicircular canal occlusion procedure33

5.3.4. Vestibular schwannoma

5.3.4.1. Defi nition. Aethio-pathology

Vestibular schwannoma is a slowly growing benign tumor which arises from the Schwann cells of the vestibular nerve, within the internal auditory canal. In evolution, it may extend into the cerebellopontine angle 19. Th e 1992 National Institutes of Health Consensus Conference made vestibular schwannoma the offi cial nomenclature for these lesions, replacing the old term acoustic neuroma 68. Th e tumors are usually solid, but may present cystic areas. Th e growth rate is slow, with an average of 0.2 cm per year. If not treated they are potentially lethal, gradual enlargement leads to indentation of the brainstem, increased intracranial pressure and death during a course of 5 to 15 years68. Vestibular schwannomas can present as sporadic tumors (95% of the cases), or as part of the familial disorder neurofi bromatosis type 2 (5%).

5.3.4.2. Symptoms

Slow-progressive unilateral sensorineural hearing loss • with poor speech discrimination (retro-cochlear lesion)In 5-20% of the cases patients may present with a sudden • sensorineural hearing lossUnilateral tinnitus•

89

Mild balance disturbances: not a true vertigo • Facial hypoesthesia due to compression of the Vth cranial • nerveFacial palsy due to compression of the VIIth cranial nerve• Cerebellar ataxia, symptoms of raised intracranial pressure • (headaches, visual disturbances, nausea, vomiting, mental status changed) in late stages of evolution

5.3.4.3. Diagnosis

Otoscopy is normal• Hearing evaluation: tuning fork tests, pure-tone • audiometry (unilateral or asymmetric bilateral sensorineural hearing loss), speech audiometry (poor speech discrimination), auditory brainstem responses (ABR)Vestibular evaluation• Imaging techniques: CT scan may detect large tumors, • MRI with gadolinium enhancement is the gold-standard method for diagnosing vestibular schwannomasNeurologic and ophtalmologic examinations•

Figure 34. MRI - Right vestibular schwannoma

90

5.3.4.4. Treatment

Th e Recommended European Standard19:


Wait and see • strategy, mainly in elderly patients and individuals with poor health and serious contraindications for surgeryRadiotherapy: stereotactic radiosurgery (gamma knife) or • fractionated stereotactic radiotherapy (55-60 Gy)Annual imaging is recommended for all patients being • managed conservatively


Translabyrinthine approach• Middle fossa approach• Retrolabyrinthine or retrosigmoid approach•

A multi-disciplinary team (otologic surgeon, neurosurgeon) is the key for success.

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40. Kelsell DP, Dunlop J, Stevens HP et al. Connexin 26 mutations in hereditary non-syndromic sensorineural deafness. Nature 1997; 387: 80-83.

41. Bagger-Sjoback D, Rask-Andersen H. Pathology of the vestibular system. In: Browning GG, Luxon LM, eds. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. 2008; 3675-3701.

42. Schuknecht HF, M.D. Pathology of the ear. 1993; 43. Bordley JE, Brookhouser PE, Worthington EL. Viral infections and

hearing: a critical review of the literature, 1969-1970. Annals of Otology, Rhinology and Laryngology 1972; 82: 557-577.

44. Lindsay JR, Davey PR, Ward PH. Inner ear pathology in deafness due to mumps. Annals of Otology, Rhinology and Laryngology 1960; 69: 918-935.

45. Hyden D, Odkvist LM, Kylen P. Vestibular symptoms in mumps deafness. Acta Oto-Laryngologica 1979; 360: 182-183.

46. Browning GG. Management of hearing impairment. In: Browning GG, Luxon LM, eds. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Edward Arnold (Publishers) Ltd 2008; 3629-3672.

47. Bess FH, Humes LE. Amplifi cation and rehabilitation. eds. Audiology: the fundamentals. Philadelphia: Lippincot Williams & Wilkins 2009; 239-292.

48. Tjellstrom A, Lindstrom J, Hallen O, Albrektsson T, Branemark PI. Oseointegrated titanium implants in the temporal bone. A clinical study on bone-anchored hearing aids. American Journal of Otology 1981; 2: 304-310.

49. Otte J, Schuknecht HF, Kerr AG. Ganglion cell populations in normal and pathological human cochleae: implications for cochlear implantation. Th e Laryngoscope 1978; 88: 1231-1246.

50. Backous DD, Duke W. Implantable middle ear hearing devices: current state of technology and market challenges. Current Opinion in Otolaryngology and Head and Neck Surgery 2006; 14: 314-318.

51. Shaia WT, Sargent EW. Inner Ear, Evaluation of Dizziness: Treatment & Medication. 2010; http://emedicine.medscape.com/article/1831429-treatment.

52. Hullar TE, Minor LB, Zee DS. Evaluation of the patient with dizziness. In: Harker LA, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 3160-3198.

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53. Cawthorne TE, Fitzgerald G, Hallpike CS. Observation on the directional preponderance of caloric nystagmus resulting from unilateral labyrinthectomy. Brain 1942; 65: 138-160.

54. Bronstein AM. Evaluation of balance. In: Browning GG, Luxon LM, eds. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Edward Arnold (Publishers) Ltd 2008; 3706-3747.

55. Eggers SDZ, Zee DS. Central vestibular disorders. In: Harker LA, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 3254-3289.

56. Anatoli-Candela F. Th e histopathology of Meniere’s disease. Acta Oto-Laryngologica 1976; 340: 5-42.

57. Schuknecht HF, M.D. Meniere’s disease: A correlation of symptomatology and pathology. Th e Laryngoscope 1963; 73: 651-665.

58. Harris JP. Autoimmunity of the inner ear. American Journal of Otolaryngology 1989; 10: 193-195.

59. Bergström T, Edström S, Tjellström A, Vahlne A. Meniere’s disease and antibody reactivity to herpes simplex virus type 1 polypeptides. American Journal of Otolaryngology 1992; 13: 295-300.

60. Vrabec JT. Herpes simplex virus and Meniere’s disease. Th e Laryngoscope 2003; 113: 1431-1438.

61. Monsell EM, Balkany TA, Gates GA, Goldenberg RA, Meyerhoff WL, House JW. Committee on Hearing and Equilibrium. Guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. Otolaryngology - Head and Neck Surgery 1995; 113: 181-185.

62. Gacek RR. Evidence for a viral neuropathy in recurrent vertigo. ORL: Journal for Oto-rhino-laryngology and its Related Specialties 2008; 70: 6-14; discussion 14-5.

63. Guyot JP, Maire R, Delaspre O. Intratympanic application of an antiviral agent for the treatment of Meniere’s disease. ORL: Journal for Oto-rhino-laryngology and its Related Specialties 2008; 70: 21-6; discussion 26-7.

64. Th omsen J, Bretlau P, Tos M, Johnsen NJ. Placebo eff ect in surgery for Meniere’s disease: a double-blind, placebo-controlled study on endolymphatic sac shunt surgery. Archives of Otolaryngology 1981; 107: 271-277.

65. Arbusow V, Schulz P, Strupp M, Dietrich M, von Reinhardstoettner A, Rauch E. Distribution of herpes simplex virus type 1 in human geniculate and vestibular ganglia: implications for vestibular neuritis. Annals of Neurology 1999; 46: 416-419.

66. Th eil D, Arbusow V, Derfuss T, Strupp M, Pfeiff er M, Mascolo A. Prevalence of HSV-1 LAT in human trigeminal, geniculate, and vestibular ganglia and its implications for cranial nerve syndromes. Brain Pathology 2001; 11: 408-413.

67. Halmagyi MG, Th urtell MJ, Curthoys IS. Vertigo: Clinical syndromes. In: Browning GG, Luxon LM, eds. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Edward Arnold (Publishers) Ltd 2008; 3748-3790.

68. Brackmann DE, Arriaga MA. Extra-axial neoplasm of the posterior fossa. In: Harker LA, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 3803-3844.

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II. NOSE

1. NOSE AND PARANASAL SINUSES BASICS


1.1.1. External nose and nasal vestibule

External nose (nasal pyramid) is a bony and cartilaginous prominence covered by skin, located at the level of the mid-face. Its shape is very important for facial aesthetics. Th e bony framework consists of the paired nasal bones and the frontal (ascending) processes of the maxillary bones. Th e cartilaginous supporting framework consists of the upper lateral cartilages (triangular cartilages), the lower lateral cartilages (alar cartilages with the lateral and medial crurae) and the midline septum. Th e medial crurae of the alars are loosely attached to each other in the midline and contribute to the columella. Attached to the external surface of the cartilages are the muscles for dilating the nares. Th e pyriform bony aperture is bounded inferiorly and laterally by the maxilla, and superiorly by the nasal bones. Th e nasal vestibule is a dilated passageway leading from the external nares (nostrils) into the nasal cavities (fossae). Th e boundary between nasal vestibule and nasal fossae is called limen nasi and is located at the level of the superior margin of the alar cartilages. Th e nasal vestibule is covered by skin bearing hair follicles (vibrissae), sebaceous and sweat glands.

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1.1.2. Nasal cavities

Th e nasal cavity extends from the limen nasi to the posterior nasal orifi ces (choanae). Th ese choanae allow the posterior communication between the nasal fossae and the nasopharynx. Each nasal cavity has a fl oor, a roof, a medial wall and a lateral wall. Th e fl oor separates the nasal cavity from the oral cavity, is nearly horizontal and consists of the palatine process of the maxilla (3/4 anteriorly) and the horizontal process of the palatine bone (1/4 posteriorly). Th e roof is narrow and may be divided from anteriorly to posteriorly into frontonasal, ethmoidal and sphenoidal parts. Th e ethmoidal part consists of the cribriform horizontal plate. It is covered by sensory olfactory epithelium. Th e nasal septum has a posterior bony framework (the perpendicular plate of the ethmoid bone and the vomer), and an anterior cartilaginous part (the quadrilateral cartilage).

Figure 1. Nasal anatomy

1 - Glabella; 2 - Root of the nose; 3 - Nasion; 4 - Nasal bone; 5 - Ascending process of the maxilla; 6 - Upper lateral cartilage (triangular cartilage); 7 - Anterior septal cartilage; 8 - Lateral crus of the alar cartilage; 9 - Medial crus of the alar cartilage; 10 - Columella; 11 - Nasal vestibule

Figure 2. Nasal anatomy

1 - Septal cartilage; 2 - Medial crus of the alar cartilage; 3 - Lateral crus of the alar cartilage; 4 - Nasal vestibule; 5 - Anterior nasal spine

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Deviations may develop at any of the septal level, and spurs may be noticed. Th ey are most likely to be acquired due to trauma than congenital1. Th e septum is covered by mucoperichondrium, mucoperiosteum and mucosa. Th e lateral nasal wall displays three pairs of small, thin, shell-like bones: the superior, middle and inferior conchae, which form the bony framework of the turbinates. Each of these curved turbinates overhangs a groove known as a meatus (superior, middle and inferior). Th e inferior turbinate is an independent bony structure attached to the lateral wall of the nose, while the middle and the superior turbinates belong to the ethmoid bone. Th e inferior meatus 2 is that part of the lateral wall, lateral to the inferior turbinate. Th e nasolacrimal duct opens at this level. Th e middle meatus is located lateral to the middle turbinate and receives drainage from the frontal sinus, maxillary sinus and anterior ethmoidal cells. Th is meatus is the surgical approach in functional endoscopic sinus surgery (FESS). Th e middle meatus contains several well-defi ned anatomical structures that represent signifi cant surgical landmarks: the uncinate process, the ethmoidal bulla, the inferior and superior semilunar hiatuses, the ethmoidal infundibulum, the frontal recess. Th e so-called ostiomeatal complex cannot be considered an anatomical structure. It is a physiological entity of the anterior ethmoid, where frontal, ethmoidal and maxillary sinuses drain3. Th e superior meatus is the drainage area for the posterior ethmoidal cells. Th e opening of the sphenoid sinus lies in the sphenoethmoidal recess, medial to the superior turbinate. Th e turbinates are covered by respiratory ciliated epithelium. Underlying the mucosa, there is an erectile tissue, mainly at the anterior end of the inferior and middle turbinate.

1.1.3. Paranasal sinuses

1.1.3.1. The frontal sinus

Th e frontal bone forms the forehead and orbital roof and is pneumatized to a variable degree. Th e size and the shape of the frontal sinuses present a lot of variations. In a small percentage of cases it may be absent. Th e frontal sinus drains in the middle meatus (frontal recess).

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Figure 3. Nasal septum

Figure 4. Frontal section through the nose and paranasal sinuses

1 - Maxillary sinus; 2 - Inferior turbinate; 3 - Middle turbinate; 4 - Middle meatus; 5 - Nasal septum; 6 - Orbit

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1.1.3.2. The maxillary sinus

Th e maxillary bone contributes to the formation of the greatest part of the roof of the oral cavity, the lateral wall and the fl oor of the nasal cavity and the fl oor of the orbit. Within the maxillary bone’s body lies the maxillary sinus (antrum). Th e maxillary sinuses are relatively symmetrical and rarely absent. Th e maxillary sinuses drain into the middle meatus (at the base of the ethmoidal infundibulum). Th e maxillary sinus is covered by ciliated respiratory epithelium which contains the highest density of secretory goblet cells, compared to the other paranasal sinuses (median: 9700/mm2)4. Anatomical relations:

Superior: the fl oor of the orbit• Inferior: hard palate, upper dentition• Anterior: skin of the cheek, fat, muscles• Posterior: pterygo• palatine and infratemporal fossae

1.1.3.3. The ethmoid sinus

Th e ethmoid bone contributes to the formation of the medial orbital wall (a thin bony structure called lamina papyracea), the superior and lateral wall of the nasal cavity (see Sect. 1.1.3.) and the bony nasal septum (the perpendicular plate). Th e complex ethmoidal cells system is divided by the insertion of the middle turbinate into an anterior and a posterior group. Th e anterior group drains within the middle meatus, while the posterior group drains within the superior meatus. Anatomical relations:

Lateral: the orbit• Medial and inferior: the nasal cavity, middle and superior • meatusSuperior: the skull base•

1.1.3.4. The sphenoid sinus

Th e sphenoid sinus lies within the body of the sphenoid bone, at the midportion of the skull base. Th e natural drainage ostium is located in the sphenoethmoidal recess, superior and medial to the superior turbinate. Th e sinuses are divided by a paramedian septum. It is completely absent in nearly 1% of the population4.

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1.1.4. Vascular and nerve supply

Th e vascular supply of the superior part of the nose comes from the anterior and posterior ethmoidal arteries, derived from the ophthalmic artery (which is a terminal branch of the internal carotid artery ICA). Th e lower part is supplied by branches derived from the maxillary artery (branch of the external carotid artery ECA). Th ese internal and external carotid sources anastomose freely at the level of the nose. Th e Kiesselbach’s area (or Little’s area) is located on the anterior part of the nasal septum. It is an aggregation of poorly supported blood vessels, being the most frequent site for the anterior epistaxis. Th e sensory nerve supply of the nose is derived from the fi rst two branches of the trigeminal nerve: ophthalmic and maxillary nerves. Th e sphenopalatine ganglion is not a sensory ganglion, it belongs to the parasympathetic system. Th e motor innervation of the nose’s muscles is supplied by the facial nerve.

Figure 5. Vascular supply of the nasal septum

1 - Anterior ethmoidal artery; 2 - Branches of posterior ethmoidal artery; 3 - Sphenopalatine artery; 4 - Greater palatine artery; 5 - Kiesselbach’s plexus

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1.2. Physiology of the nose

1.2.1. Nasal breathing

Th e nose acts as an air conditioner and performs three major functions: heat transfer, humidifi cation and fi ltration. Because of its ability to transfer heat, the nose may be more important in temperature regulation than in respiration5. Th e anterior nasal valve is the narrowest part of the airway, and therefore the greatest resistor, producing the most turbulent airfl ow6. It is delineated by the inferior margin of the upper lateral cartilages, the head of the inferior turbinate and the adjacent nasal septum. Th e nasal cycle is a physiological alternate nasal blockage between the two nasal passages. It was known by the Yogis since antiquity, but Kayser gave its fi rst description in 18957. It is produced by vascular activity, particularly the blood volume on the venous sinusoids of the turbinates (capacitance vessels). It can be demonstrated in 80% of the people.

1.2.2. Protection of the lower airways

Th e nose protects the lower airways by fi xing and removing particles up to 30 μm from the inspired air. Th e mucus fi lm covering the nasal mucosa is responsible for trapping the particles. Th e transport of the particles to the nasopharynx and oropharynx depends on the mucociliary clearance, which is determined by the motion of the mucus blanket. Th is motion is supplied by the coordinated waves of cilia8. In addition to this mechanical fi ltration, the nose actively participates in the immunological defense of the airways.

1.2.3. Olfaction

Olfaction is important in regulation of food intake, in the perception of fl avors, it has also a protective function by helping to identify toxic and irritating agents from the inspired air. Humans can detect more than 10,000 diff erent odors and discriminate between 5,000 of them8.

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1.3. Symptoms of nasal diseases

1.3.1. Nasal obstruction

Nasal obstruction (“stuff y nose”, nasal congestion, nasal blockage) is the unpleasant sensation of having diffi culty breathing through the nose. It can be unilateral or bilateral, partial or complete, transient or permanent. Nasal obstruction can be the result of two main causes9:

Mechanical or structural abnormalities: foreign bodies, • deviated nasal septum, nasal polyposis, benign tumors, malignant tumors, turbinate hypertrophy, malformations, choanal atresiaFunctional: allergic rhinosinusitis, acute and chronic • rhinosinusitis, rhinitis medicamentosa, rhinitis of pregnancy, atrophic rhinitis, chemical injury of nasal mucosa (inhaled drugs, occupational)

1.3.2. Nasal discharge (Rhinorrhoea)

Th e main semiologic features of nasal discharge are:Th in and watery: onset of common cold, allergic rhinitis• Th ick mucus: chronic rhinitis• Mucopurulent: if bacterial infection is superimposed • (persistent unilateral discharge of yellow pus is usually indicative of sinus disease)A foul unilateral mucopurulent discharge in a child: • usually a foreign bodyMucopurulent, sanguinolent, blood-stained: tumors• Crusts: atrophic rhinitis• Cerebrospinal fl uid rhinorrhoea: the leakage of CSF • from the subarachnoid space into the nasal cavity due to a defect in the dura, bone and mucosaPost-nasal discharge (post-nasal drip): disease located at • the posterior group of paranasal sinuses

1.3.3. Headache and facial pain

Headaches and facial pain can be related to sinus diseases (infl ammatory, neoplasms, barotrauma). Many patients are referred to the ENT doctor with chronic headaches, claiming

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they have sinusitis. But there are a lot of other non-sinus causes which must be ruled out: dental pain, vascular (migraine, cluster headaches, temporal arteritis), neuralgias, cervical spine disorders, temporomandibular joint dysfunction. A good collaboration with a neurologist and a maxillo-facial surgeon is mandatory for a proper management of these patients. Cluster headache typically aff ects men between 30 and 50 years old. Th e aetiology is vascular. Pain is located frontal, temporal, extends over the cheek or even into the teeth, and is accompanied by lacrimation, rhinorrhoea and nasal obstruction. Th ese symptoms may lead to misdiagnosis of sinusitis. Th e pain may last for 15 minutes up to two hours. Clusters of attacks may continue for several weeks10. Trigeminal neuralgia is characterized by severe paroxysms of unilateral lancinating pain in one or more branches of the trigeminal nerve, induced by a specifi c trigger point (lips, nasolabial folds, gingivae).

1.3.4. Smell disorders

Smell disorders may be quantitative (anosmia/hyposmia and hyperosmia) or qualitative (parosmia and phantosmia). Decreased or loss of smell perception can be provoked by rhinologic or neurologic causes. Any nasal obstruction which doesn’t allow the odor particles to reach the olfactory area in the upper nose will cause hypo- or anosmia. Neurological causes include viral upper respiratory tract infections, head trauma, neurodegenerative disorders (Alzheimer disease, Parkinson disease, multiple sclerosis), epilepsy and migraine. Hyperosmia should be considered rather a quality feature (cooks, wine tasters, perfumes and fragrances makers) than a pathological condition. Parosmia is a wrong, altered perception of a smell which does exist in the environment (“nothing smells normal”). It is described mainly by women during pregnancy. Olfactory hallucinations or phantosmias (perception of a smell without an odor present) can occur in psychiatric disorders.

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Prior to instrumental examination, the nasal pyramid and adjacent facial structures should be carefully inspected and palpated. We should evaluate the overall shape of the nose, the presence of nasal deformities of the dorsum, tip or columellae, scars, abnormal swellings, skin lesions. Crepitations or pain on palpation suggest a fracture of the nasal bones. Palpation of the sinusal “trigger” points:

Frontal sinus: the supraorbital point (at the junction of • the lateral 2/3rds of the superior orbital rim with the medial 1/3rd) and supero-internal angle of the orbit (corresponds to the fl oor of the frontal sinus)Anterior ethmoid cells: the internal angle of the orbit • (the Ewing’s point)Maxillary sinus: the infraorbital point (located 1/2 cm • below the middle of the inferior orbital rim) and the canine fossa (located laterally to the bony prominence of the canine’s root)

Th ese points are sensitive in acute rhinosinusitis, acute exacerbations of chronic rhinosinusitis and dental problems (the canine fossa). Palpation of these points should be performed alternatively, not simultaneously, on both sides of the face.

1.4.2. Examination of the nasal vestibule

Instruments:Light source• Head mirror•

Th e examiner is in front of the seated patient, light beam is focused on the tip of the nose, the examiner’s left hand is placed on the patient’s forehead, fi xing the head. Using the left thumb, the examiner elevates the tip of the nose, allowing the visualization of the nasal vestibule.

1.4.3. Anterior rhinoscopy

Instruments:Light source•

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Figure 6. Examination of the nasal vestibule

Figure 7. Hartmann nasal speculum

Head mirror• Nasal speculum (Hartmann, Killian) • Otoscope: recommended for small children• Straight and curved suction tips•

With the patient seated, light beam is focused on the tip of the nose. Th e left hand of the examiner is placed on the forehead, fi xing the patient’s head, while the other gently opens the nostril with the nasal speculum. Anterior rhinoscopy allows visualization of the septum, inferior turbinate and meatus and

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Figure 8. Anterior rhinoscopy

the fl oor of the nasal cavity. Tilting the patient’s head backwards gives a consecutive view of the middle turbinate and meatus. Th e superior turbinate can’t be usually visualized because of the extremely narrow space between the middle turbinate and the septum. Vasoconstrictors may be used in selected patients in order to improve the visibility of the nasal cavities. Nasal secretions should be removed before examination by asking the patient to blow the nose, or by suctioning.

1.4.4. Nasal endoscopy

Nasal endoscopy has become the gold standard in nasal examination, for it provides accurate and reliable information on the entire nasal cavity, including traditionally inaccessible areas like the sphenoethmoidal recess or the ostiomeatal complex 11. Instruments:

Light source• Fiber optic cable• Endoscopes: rigid telescopes of 25 cm length, 4 mm diameter • and angulations of 0º and 30º are most commonly used. Smaller endoscopes are also available (2.7 mm diameter).

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Figure 9. Nasal endoscopes

More recently, 45º and 70º endoscopes have been developed. Fiber optic fl exible endoscopes can also be usedStraight and curved suction tips, fl exible metal cotton-• tipped applicators, straight and upbiting forceps, anti fog solutionsTopical nasal decongestants and anaesthetics• Video and digital recording equipment, if available•

Figure 10. Nasal endoscopy

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Before starting the examination, explain the procedure to the patient. Apply topical vasoconstrictors and anaesthetics. Remove nasal secretions by suctioning. Nasal endoscopy can be performed with the patient either sitting, or in the supine position. Th e endoscope should be held with one hand, while the other is placed on the patient’s forehead in order to fi x the head. Th e endoscope is gently introduced into the nasal vestibule and nasal cavity, allowing an accurate examination of the whole nasal cavity and the nasopharynx12.

1.4.5. Examination of the paranasal sinuses

1.4.5.1. Maxillary sinusoscopy

Direct visualization of the maxillary sinus cavity can be obtained by performing an antrostomy or maxillary sinusoscopy, via inferior meatus or canine fossa puncture. It is an invasive procedure. It must be performed in the operating room, under local or general anaesthesia. Rigid endoscopes are used to examine the cavity.

1.4.5.2. Transmeatal puncture

Local anaesthesia must be applied at the level of the inferior meatus. Th e tip of the trocar is placed 1 cm behind the head of the inferior turbinate, in the inferior meatus, being pointed to the external commisure of the orbit. Once the sinus cavity is reached, it can be either aspirated or irrigated. Possible complications of the procedure are penetration of the orbit, and perforation of the posterior wall of the sinus.

1.5. Clinical investigations

1.5.1. Imaging techniques

1.5.1.1. Conventional radiography

Plain sinus radiographs: diagnosis of rhinosinusitis (sinus • opacifi cation, air-fl uid levels, mucosal thickening), tumors of the nose and paranasal sinusesNasal pyramid radiographs: diagnosis and legal • documentation in traumatic lesions of the nasal bones

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Figure 11. Conventional radiography of the paranasal sinuses

1 - Maxillary sinus; 2 - Ethmoid sinus; 3 - Frontal sinus; 4 - Orbit

1.5.1.2. Computed tomography CT

Th e gold standard technique for imagistic evaluation of • sino-nasal pathologyFor screening the sinuses, an axial view is available• When endoscopic sinus surgery is anticipated, direct • coronal views are mandatory for pre-operative evaluation of the extent of the disease, to detect anatomic variations and for planning the surgical approach13

For evaluation of tumors, both axial and coronal views are • indicated, with contrast enhancement

1.5.1.3. Magnetic resonance imaging MRI

Is primarily indicated for evaluation of sinus tumors, and • occasionally infl ammatory diseases, such as mucocelesTh e main advantage of MRI, comparing to CT scans, • is the ability to distinguish between tumoral tissues and obstructed sinus secretions and to predict the true extent of the tumor 13

Sagittal, axial and coronal views are available, with or • without contrast enhancement

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1.5.2. Rhinomanometry

Rhinomanometry allows the simultaneous assessment of the fl ow and pressure variations to which an air current is subjected, as it passes through the nasal airways. Th e obtained data are used to calculate the nasal resistance. Two methods are available: active anterior rhinomanometry (most frequently used) and active posterior rhinomanometry. Both are based on the same principle, in which the airfl ow and nostril pressure are measured inside a mask covering the nose and the mouth 14. Acoustic rhinometry is a recently developed modern technique, based on acoustic scanning, which provides an objective geometric study of the nasal cavity 15.

1.5.3. Allergy testing

For the diagnosis of allergic aetiology in rhinosinusal diseases, a number of tests are available:

Skin tests: scratch, prick or intradermal injections •

Figure 12. MRI of the nose and paranasal sinuses

1 - Maxillary sinus; 2 - Left ethmoid sinus; 3 - Right ethmoiditis; 4 - Orbit; 5 - Right inferior turbinate; 6 - Left middle turbinate

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demonstrate immediate hypersensitivity mediated by IgE antibodiesRAST (Radio Allergo Sorbent Test) detects specifi c • circulating IgE antibodies

1.5.4. Nasal cytology

Nasal cytology can be assessed by taking a smear or a nasal brushing. A swab is wiped over the outer surface of the middle turbinate. Afterwards it is smeared onto glass slides and fi xed cytologically. Th e diff erent epithelial cells and the percentage of each leukocyte type is measured by May-Grunwald-Giemsa staining. Th e normal nasal mucosa does not contain polynuclear neutrophils or eosinophils 14.

1.5.5. Exploration of mucocilliary function

Exploration of mucocilliary function is not a routine clinical examination. Th e most frequently used test is the saccharine transit time test 16. It is performed by placing some saccharine powder at the level of the head of the inferior turbinate. Th e time needed by the patient to feel the saccharine taste is normally less than 20 minutes 14. Modern techniques used to evaluate the ciliary beat rate and electron microscope analysis of ciliary structure have been developed for scientifi c purposes.

1.5.6. Olfactory tests

Th e most widely used olfactory test is the 40-item University of Pennsylvania Smell Identifi cation Test (UPSIT). Th is test can be self-administered in 10-15 minutes by most patients in the waiting room, and scored in less than a minute 17. Other modern techniques, available only in specialized centers, include the recording of olfactory event-related potentials (OERP) and evaluation of olfactory mucosa biopsies.

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2. DISEASES OF THE EXTERNAL NOSE AND

NASAL VESTIBULE

2.1. Acute nasal infections

2.1.1. Nasal vestibulitis


Nasal vestibulitis is characterized by a diff use infl ammation of the skin of the vestibule. Th e infective process is secondary to local trauma (mainly of the fi nger variety) or excoriations from rhinitis. Th e bacterial agents involved are commensal bacteria residing in the upper respiratory tract: Staphylococcus aureus (30%) and Streptococcus pyogenes (10%) 18.

2.1.1.2. Symptoms and signs

Mild to moderate local discomfort is frequently reported• Local erythema, edema and tenderness• Crusting•

2.1.1.3. Treatment

Topical agents (antibiotics plus corticoids)• Oral antibiotics are rarely necessary•

2.1.2. Nasal furuncle


A furuncle (common boil) is a localized infection of the nasal vestibule’s skin, centered on a hair follicle, caused by Staphylococcus aureus.


Local severe pain, accompanied sometimes by fever, • malaise

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It usually starts like a tender, fi rm nodule with surrounding • infl ammation which subsequently develops an abscess, with a characteristic white point (bourbillion) in the middle of the tumefaction


Persistent or recurrent disease may suggest an underlying • diabetes mellitus or an immunocompromised stateRetrograde dissemination of the infection through the • valveless facial veins can determine an extremely serious complication: cavernous sinus thrombosis (periorbital swelling, chemosis, ophtalmoplegia, pupillary changes)

2.1.2.4. Treatment

If no systemic symptoms or extensive cellulitis are present, • simple topical care is usually curative: topical antibiotics and steroids, topical disinfectantsIn severe cases: oral antibiotics (Oxacillin, Doxycycline, • Clindamycin, Vancomycin)Analgetics, non-steroidal antiinfl ammatory drugs • (NSAIDs)Larger, collected furuncles should be drained by surgical • incision

2.1.3. Erysipelas


Erysipelas is a skin infection caused by Streptococcus pyogenes group A


Sudden onset with fever, chills, • malaiseRed, tender, indurated skin plaque with well-defi ned • elevated borders

2.1.3.3.

Penicillin in high doses• Analgetics and non-steroidal antiinfl ammatory drugs • (NSAIDs)

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2.2. Trauma

2.2.1. Nasal fractures


A nasal fracture is a disruption of the nasal bone structure, due to trauma. It is the most frequently fractured facial bone, because of its location, prominence and delicate structure. Sports, falls and aggressions are the major involved aetiological factors. Men are aff ected twice as often as are females. Children usually develop cartilaginous lesions and “greenstick” fractures 19.

2.2.1.2. Symptoms

History is extremely important and must be documented • for legal purposesPain, nasal obstruction, epistaxis•

2.2.1.3. Diagnosis

Inspection may reveal local edema, deviations and asymmetry of the nasal pyramid, epistaxis. Periorbital ecchymosis suggest associated lesions of the lamina papyracea (medial wall of the orbit). Th e post traumatic appearance of the nose should be compared with older photos (ID card). Palpation of the nose reveals crepitations, abnormal mobility of the nasal pyramid structures and local pain. A careful palpation of the adjacent facial bony structures must be performed. Anterior rhinoscopy allows removal of blood clots by suction, assessment of the associated septal lesions, identifi cation of the bleeding site. Nasal pyramid radiographs (lateral views) help the diagnosis and provide legal documentation. Th ree-dimensional CT is recommended in case of extensive injuries involving adjacent structures.

2.2.1.4. Treatment

Treatment of bleeding (see Epistaxis)• Nondisplaced fractures should be treated with observation • alone

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Closed nasal reduction is the method of choice in the • majority of the casesAn open approach is rarely indicated (severe trauma, the • need for wide surgical exposure)

2.3. Dermatological conditions

2.3.1. Rhinophyma (Rosacea)


Rosacea is a dermatological condition involving the central face, characterized by the presence of papules and pustules on a background of erythema, edema and telangectasia 18. Th e fi nal stage of rosacea, aff ecting almost exclusively men’s nose, is called rhinophyma. Th e exact cause is unknown. Genetic factors, infection and alcohol consumption have been involved in aetio-pathology. Th ere is hyperplasia and hypertrophy of the sebaceous glands and chronic deep infl ammation.

Figure 13. Rhinophyma

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2.3.1.2. Diagnosis

Patients address to the doctor mainly for cosmetic • reasonsSometimes, due to gross enlargement, nasal obstruction • is reportedPseudo-tumoral disfi guring appearances of the tip of the • nose and alar region may develop during later stages

2.3.1.3. Treatment


Long-term antibiotics for treatment of secondary • infections (topical Metronidazole, oral Tetracycline)Synthetic retinoids (Isotretinoin)• Skin care•


Full-thickness excision followed by application of free • skin graftsPartial-thickness excision or decortication: razor blades, • surgical scalpel, electrocoagulation, dermabrasion, carbon dioxide, argon and Erbium-Yag lasers 20

2.4. Tumors

2.4.1. Benign tumors

2.4.1.1. Keratoacanthoma

2.4.1.1.1. Defi nition. Aetiology

Keratoacanthoma, or molluscum sebaceum, is a benign cutaneous tumor that follows a specifi c growth pattern, terminating in complete resolution18. Aetiological factors: ultraviolet radiation exposure, occupational exposure to tars, mineral oils, viral infection.

2.4.1.1.2. Diagnosis

Firm, round lesion which grows rapidly (up to 6 weeks), • becomes globular and develops a central horn-fi lled or keratotic plug

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Complete resolution occurs in a variable interval of 3 • months - one yearDiff erential diagnosis: squamous cell carcinoma• Histopathology: proliferating keratinizing cells centered • on hair follicles

2.4.1.1.3. Treatment:

Simple curettage• Excision biopsy•

2.4.2. Malignant tumors

2.4.2.1. Squamous cell carcinoma (SCC)

2.4.2.1.1. Defi nition

SCC is a malignant cutaneous tumor developed from epidermal keratinocytes. Th e most frequently involved aetiological factors are exposure to hydrocarbons (tar and mineral oils), thermal factors and solar radiation. Metastases are found in 10 per cent of the patients18.

Figure 14. SCC of the nose

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Vario• us clinical aspects: erythematous plaque, nodular, verrucousFirm lesion with induration of the surrounding tissue and • fi xation to underlying structures in later stagesIn evolution, crusting, ulceration and bleeding occurs• Histhopatology• is mandatory for diagnosis

2.4.2.1.3. Treatment

Surgic• al excision in oncological safety limits, followed by reconstruction of the defect with various fl apsIn case of neck metastases, a neck dissection must be • performedRadiotherapy and cryotherapy are satisfactory treatment • alternatives

2.4.2.2. Basal cell carcinoma (BCC)


BCC is the most frequently encountered skin cancer in white races, with the highest incidence reported in Australia (726 cases per 100,000)21. It is a neoplastic proliferation of cells that resembles the epidermis basal cells, although the precise origin is still unclear. Th e tumor is locally invasive. Metastases are uncommon, being reported in less than 0.1 per cent of the cases22. It is more common in men at older ages. Th e single most important aetiological factor is ultraviolet (UV) solar exposure. Other predisposing factors: light skin color, poor tanning ability, blonde hair, immunosuppressive therapy, arsenic exposure.


Various clinical aspects: nodular (erythematous papules • or plaques, pedunculated lesions), superfi cial BCC (scaly plaque with poorly defi ned margins, making excision diffi cult), morpheaform BCC (skin plaque which develops telangiectasia)Th e lesion appears initially innocent and is neglected, • but cure failures to various topical treatments refers the patient to the doctor

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Slow-growing evolution, locally invasive• Histopathology is mandatory for diagnosis•


Shave biopsy, curettage, cautery are indicated for small • lesionsSurgical excision: a 4 mm safety margin is recommended • and excision should involve the dermal layer23, followed by reconstruction of the defectMoh’s surgical technique: sequential accurate excision of • the tumor under frozen section controlIntralesional interferon injection, photodynamic therapy, • radiotherapy18

2.4.2.3. Malignant melanoma (MM)


MM is the leading fatal skin malignancy. It develops from melanocytes, in evolution invades the dermis, the depth of the lesion being the most important prognostic factor 24. Th e most important aetiological factor involved is intense, intermittent sun exposure in fair-skinned individuals.


Clinical features suggesting a melanocitic lesion’s • malignancy: rapid growth, irregularity, asymmetry, changes in color, pruritus, bleeding and infl ammationSeveral forms exist: superfi cial spreading type (brown • macular lesion which exhibits a long horizontal growth phase and close inspection reveals a variety of hues within the lesion), nodular type (rare on the nose), lentigo melanoma (fl at brown-black patch which extends horizontally for months to years)Lymph nodes metastases may be present• Histopathology is mandatory for diagnosis•


Surgical removal within safety oncological margins (1-3 • cm), associated with neck dissectionsAdjuvant therapeutic procedures in advanced stages: • chemotherapy protocols, Interferon alpha-2b (Intron), Interleukin-2 (Proleukin)25

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3. RHINITIS

3.1. Acute rhinitis

3.1.1. Acute viral rhinitis


Acute rhinitis is an infl ammatory condition of the nasal mucosa, caused by a viral infection. It is known also as “common cold” or “coryza”. Th e disease is highly contagious and is transmitted by means of airborne droplets. It rarely appears as an isolated form, in most of the cases it is part of an upper respiratory tract infection (rhino-pharyngitis, rhino-pharyngo-laryngitis). It may be followed by secondary bacterial infection. Viruses that cause rhinitis include rhinoviruses, adenoviruses, infl uenza and parainfl uenza viruses, coxsackie virus and respiratory syncytial virus 26. Most important predisposing factors are lack of immunity and fatigue.

3.1.1.2. Symptoms

Th e prodromal • stage: local irritation, sneezing, dryness in the noseTh e acute phase: serous • rhinorrhoea, nasal obstruction, fever, headaches, malaise, watery eyesTh e nasal discharge • may become thicker and even purulent if secondary bacterial infection occurs

Figure 15. Acute rhinitis

1 - Middle turbinate; 2 - Nasal septum; 3 - Middle meatus

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After 6-7 days, the nasal passages reopen and the • secretions gradually return to normal

3.1.1.3. Diagnosis

Anterior rhinoscopy or endoscopic examination: • generalized hyperemia of the nasal mucosa, which is covered with serous secretionsA complete ENT examination should be performed: • rhinitis is frequently associated with acute pharyngitis, laryngitisSerologic assessment of antiviral antibodies titers is not • a routine laboratory procedure; it is used mainly for scientifi c purposes

3.1.1.4. Treatment

As the disease is highly contagious, the patient should be • isolated from collectivities, if possibleBed rest, hydration• Topical nasal decongestants drops/sprays (ephedrine, • oxymethazoline, xylomethazoline)Analgetics and antipyretics • Antihistamines are frequently prescribed to help decongest • the nose and diminish watery serous secretionsAntibiotics are not necessary (viral infection), but may • be prescribed in order to prevent secondary bacterial infection in predisposed patients

3.1.2. Acute purulent rhinitis


Purulent rhinitis commonly complicates the exanthemas, or may appear in the evolution of a common cold by secondary bacterial infection. Membranous forms may occur in pneumococcal, staphylococcal or streptococcal infection, mainly in young children and debilitated patients.

3.1.2.2. Diagnosis

Nasal obstruction, purulent nasal discharge, fever, • headaches, malaiseComplete ENT examination should be performed•

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A nasal swab culture may be indicated for accurate • antibiotic prescriptions

3.1.2.3. Treatment

Th e same as for the common cold, but requires antibiotics • or chemotherapy with bacteriological control

3.1.3. Nasal diphtheria


Diphtheria is considered nowadays an eradicated disease. It is caused by Corynebacterium diphteriae (Klebs-Loeffl er bacillus). Th e nose can occasionally be aff ected, mainly in children.

3.1.3.2. Diagnosis

Sero-purulent nasal discharge, which may be blood-• stained and is often associated with excoriation of the upper lip, nasal obstructionAnterior rhinoscopy or endoscopic examination reveals • grayish-white membranes covering the inferior turbinates and adjacent structures, which are extremely adherent and bleed when removedA culture swab should always be taken whenever • membranes are seen in the nasal cavities

3.1.3.3. Treatment

Patients must be isolated and declared• Early administration of antitoxin is mandatory• Antibiotics•

3.2. Chronic specifi c rhinitis

3.2.1. Nasal tuberculosis


Nasal tuberculosis is a chronic specifi c granulomatous infl ammatory condition, caused by the infection with Mycobacterium tuberculosis. Nasal infection may be the consequence of direct

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inoculation (nose picking and fi nger nail trauma), dissemination from an open pulmonary tuberculosis or hematogenous spread.

3.2.1.2. Diagnosis

Th ree clinical patterns are described27:Lupus vulgaris (nodular form) usually develops in the • nasal vestibule and then extends to the adjacent skin and mucosa. It is the consequence of direct inoculation. Th e clinical aspect is characterized by the presence of papules or nodules, which in evolution may coalesce and break down to form typical ulcers with a pale granular base. Th e disease is very slowly progressive, if not treated scarring and deformities occur. Ulcerative form involves the nasal cavity (nasal septum, • inferior turbinate) and presents with nasal obstruction, nasal discharge, crusting, epistaxis. Septal perforation may occur, but septal bone is usually sparedSinus granuloma: isolated sinus involvement have been • reported without any signs and symptoms in the nose. Th e CT and MRI characteristics are not specifi c and reveal a soft tissue polypoid mass with or without bone destruction28

Diagnosis is usually established by a tissue biopsy (typical epitheloid cell granulomas). Microbiologic diagnosis is ideal and is the only confi rmatory test for infection with Mycobacterium tuberculosis. Skin testing for delayed hypersensitivity and pulmonary radiographs should also be performed. Diff erential diagnosis must rule out cancer and other granulomatous diseases aff ecting the nose.

3.2.1.3. Treatment

Chemotherapy protocols•

3.2.2. Syphilis


Syphilis is an infectious disease caused by Treponema pallidum. Th e infection may present in various forms that have been classically described as primary, secondary, tertiary and

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congenital syphilis. Nose involvement can occur at any age, from the neonate to the elderly.

3.2.2.2. Diagnosis

Primary syphilis rarely involves the nose. Th e typical • lesion is an ulceration (chancre) at the inoculation site, accompanied by regional lymphadenopathySecondary stage is the most infectious and manifests 6-10 • weeks after inoculation. It is a systemic disease. Nasal manifestations include a simple catarrhal rhinitis with no special features, except in its persistence. Crusting and fi ssuring of the nasal vestibule may occur. Mucous patches are visible at anterior rhinoscopy.Tertiary syphilis is the stage most commonly encountered • in the nose. Th e lesions are chronic and destructive. Th e typical lesion is a gumma. It begins as a nodule which fi nally progresses to involve the overlying skin, and then breaks down to form a destructive ulcer. Th e bony portion of the nasal septum is the predilection site of involvement. In neglected cases, perforation of the aff ected nasal walls and collapse of the bony support, accompanied by severe scarring may occur27

Congenital syphilis: any of the lesions of secondary or • tertiary may be present. “Snuffl es” is the most frequently encountered lesion in children. It begins as a simple catarrhal rhinitis, which becomes purulent in short time. Examination reveals accompanying fi ssuring and excoriation of the nasal vestibule and upper lip. Gummatous lesions occur mainly at puberty. Other stigmata may be present: Hutchinson’s incisors, interstitial keratitis, corneal opacities and sensorineural hearing loss27

Diagnosis is clinical, serological (Venereal disease reference laboratory VDRL, Treponema pallidum haemagglutination test TPHA, Fluorescent treponemal antibody test FTA-ABS), microbiological and histopathological (biopsy).

3.2.2.3. Treatment

Antibiotics: Penicillin• Local treatment: cleansing of the crusts, nasal irrigations • with saline solutions

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3.2.3. Rhinoscleroma


Rhinoscleroma is a progressive granulomatous infl ammatory disease, commencing in the nose, with later involvement of the pharynx, larynx and sometimes trachea and bronchi29. Rhinoscleroma is a misnomer, the condition is an infectious disease, not a tumor. Th e causative agent is the gram-negative bacillus Klebsiella rhinoscleromatis (Frisch bacillus). It occurs sporadically throughout the world and is endemic in Eastern Europe and South America30.

3.2.3.2. Diagnosis

Th ree stages are described in the evolution of rhinoscleroma27:Th e atrophic stage: nasal obstruction, crust formation and • foul-smelling rhinorrhoeaTh e granulomatous, nodular or proliferative stage: bluish-• red nonulcerative nodules developTh e cicatrizing stage: fi brosis, adhesions, stenosis, • distortions of normal anatomy

Diagnosis is diffi cult in the fi rst atrophic stage. It is usually done in the proliferative and cicatrizing stages. It requires a biopsy. Th e histopathological fi ndings typical for scleroma are the presence of granulomatous infl ammatory lesions (plasma cells, lymphocytes, eosinophils, foamy Mikulicz cells with cytoplasm containing bacilli and Russell bodies).

3.2.3.3. Treatment

Long-term and high-dose antibiotic treatment (minimum • 4-6 weeks) until two consecutives cultures from the biopsy material are proven negative 31. Th e traditional used antibiotics are streptomycin and tetracycline, but recent reports have emphasized the good results obtained with a combination of oral ciprofl oxacin, rifampicin and sulphametoxazole-trimethoprim32

In later stages, plastic and reconstructive surgery may be • required

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3.2.4. Leprosy


Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae (Hansen’s bacillus), an acid-fast bacillus morphologically similar to Mycobacterium tuberculosis. Five countries (Brazil, India, Myanmar, Indonesia and Nigeria) account for 82 per cent of all leprosy patients worldwide27.

3.2.4.2. Diagnosis

Th e clinical features in the nose depend on the type of leprosy33:

In tuberculoid leprosy (strong host resistance, non • infective, localized) solitary skin lesions are described causing anaesthetic cutaneous patches with involvement of sensory or motor nerves (V, VII cranial nerves). Nasal mucosa is not involvedIn lepromatous leprosy (poor host resistance, infective and • systemic), diff use infi ltration of skin, nerves and mucosa occurs. Clinical features include nasal obstruction, crusts formation, blood-stained nasal discharge containing infecting bacilli. Late disease is characterized by destructive lesions leading to the classical leonine faciesBorderline leprosy is of intermediate severity. Skin lesions • resemble the tuberculoid form but are more numerous and irregular

Diagnosis is made on clinical, bacteriological and histopathological evidences.

3.2.4.3. Treatment

Long term (6-12 months) triple medication regimens: • Rifampicine, Dapsone and ClofazimineDirect nasal administration of rifampicin has been • reported to be more eff ective than the oral route34

Local treatment: crust removal, nasal irrigations• Surgery for cosmesis improvement•

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3.2.5. Rhinosporidiosis


Rhinosporidiosis is a chronic granulomatous fungal infection. It has a worldwide distribution, mainly in tropical countries, but is endemic only in India and Sri Lanka 30. Th e disease is caused by Rhinosporidium seeberi, a fungus-like organism, not yet successfully grown in culture medium or transferred from a human to an animal host35.

3.2.5.2. Diagnosis

Symptoms: nasal obstruction, epistaxis, blood-stained • mucopurulent rhinorrhoeaNasal examination: fl eshy nasal polyps, often with grey • specks upon them , which gives a “ripe strawberry” appearance. Th e polyps are frequently pedunculated and arise mainly from the septum, nasal fl oor and inferior turbinateHistological examination is mandatory•

3.2.5.3. Treatment

Complete surgical excision• No medical management have been found to be • eff ective

3.3. Atrophic rhinitis


Atrophic rhinitis is a chronic nonspecifi c disease of the nasal mucosa and subjacent bones, leading to abnormally wide nasal cavities, dryness, crusting, atrophy and a paradoxical subjective sensation of nasal blockage 19. Changes may be slight (rhinitis sicca) or severe, with massive crusting and an extremely unpleasant fetid odor (ozena). Th e aetiology is still unclear. Originally it was attributed to colonization by Klebsiella ozenae, and now this form is considered primarily. It is seen mostly in young females, in developing countries with warm climates. Genetic, occupational, endocrine, vascular, autoimmune and nutritional factors have also been involved.

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Nowadays, secondary atrophic rhinitis may follow aggressive surgery for nasal obstruction (nasal turbinectomies), trauma, radiation therapy.

3.3.2. Diagnosis

Th e earliest complaint • is a feeling of nasal dryness, accompanied by headachesDryness or rawness in • the rhinopharynx is not unusualParadoxical nasal blockage • is often described, when in fact there is no obstruction present and the airways are wide. Th is feeling is probably caused by the lack of normal airfl ow sensation in the presence of a dry, insensitive mucosa. However, the characteristic accumulation of crusts in the nasal fossae may fi nally determine a true obstructionNasal examination: atrophy, crusting, septal perforations •

3.3.3. Treatment

Local antibiotics (Bioparox spray) and nasal irrigations • with crust removalSea-water sprays or drops (Sterimar, Nazomer)• Vitamin A• Topical decongestants must be avoided• Surgical treatment (Ozena): various techniques were • described for narrowing the nasal airways

3.4. Nasal manifestations in systemic diseases

3.4.1. Wegener’s granulomatosis (WG)


WG is a systemic chronic vasculitis of small- and medium-sized blood vessels, with an autoimmune component 19. In 1939,

Figure 16. Atrophic rhinitis

1 - Inferior turbinate; 2 - Nasal septum; 3 - Crust

129

Friedrich Wegener fi rst described necrotizing granulomas and vasculitis of the upper and lower respiratory tract, occurring either together or as separate components.

3.4.1.2. Symptoms

Th e disease may present with upper respiratory tract, pulmonary or renal involvement in a localized or disseminated form.

Th e localized form (25% of the patients) is confi ned to the • upper respiratory tract. Nasal symptoms are non-specifi c and include nasal obstruction, severe crusting, purulent, blood-stained rhinorrhoea, pain over the dorsum of the noseIn the disseminated form, pulmonary involvement is • manifested by cough, dyspnoea, haemoptysis, pleuritic pain. Systemic symptoms are also present (malaise, fever, loss of appetite, migratory arthralgias). 90% of the patients have nasal symptoms, which are usually the fi rst manifestations of the disease

3.4.1.3. Diagnosis

Nasal examination: painful mucosal ulcers, crusting, • septal perforationsComplete ENT examination: pharyngeal, laryngeal, • otological and orbital involvement may occurBlood tests: elevated ESR, anemia, evaluation of renal • function, cytoplasmic-staining antineutrophil cytoplasmic antibodies (c-ANCA) test, confi rmed by proteinase 3 (PR3) ANCA test or myeloperoxidase (MPO) ANCA test19

Pulmonary radiographs• Nasal mucosa biopsy: mandatory for the diagnosis•

3.4.1.4. Treatment

Th e current standard regimen is oral cyclophosphamide • and prednisone, associated with Trimethoprim-sulphametoxazole36

In localized nasal forms, Trimethoprim-sulphametoxazole • is recommended as single therapy37

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3.4.2. Sarcoidosis


Sarcoidosis is a systemic disorder of unknown aetiology, characterized by the presence of non-caseating epitheloid granulomas. It may aff ect any part of the body, but most frequently involves the lymph nodes, the skin, the lungs, the eyes and the liver. Skin lesions were fi rst described by Besnier in 1889 as lupus pernio, but the generalized nature of the disease was pointed by Boeck, ten years later38.


Sarcoidosis is a multisystem disease, primarily aff ecting the lower respiratory tract, but which may involve the upper respiratory tract, often more frequently than previously realized. Nasal involvement is reported to occur in 3% of the cases 39.

Nasal symptoms: nasal obstruction, crusting, blood-• stained rhinorrhoea, facial painYellowish lesions occur on the septum and lateral nasal • wall, associated with crustingSeptal perforations and saddle-nose deformity may be • presentLupus pernio• can occur on the nasal skin (thickening and discoloration of the skin with granular appearance, “strawberry skin”)

3.4.2.3. Diagnosis

Complete ENT examination• Chest X-rays, CT scans• Biopsy and histopathology• Th e Kveim intradermoreaction, one of the most reliable • diagnostic test was unfortunately withdrawn from many countries over fear of virus and prione transfer though without any clear evidence for this 40

3.4.2.4. Treatment

Systemic therapy: combination of oral steroids, • methotrexate and hydroxychloroquine 38

Local treatment: saline irrigations, crust removal, • intranasal steroids

131

3.5. Allergic rhinitis

3.5.1. Defi nition. Classifi cation

Allergic rhinitis is an infl ammatory condition of the nasal mucosa as a result of an IgE-mediated hypersensitivity reaction. Allergic rhinitis is a global health problem, an increase in its prevalence was noticed during the last 40 years. In European countries, the prevalence of this disease has been rated from 17 to 29%41. Allergic rhinitis has a great impact on the patient’s social life, aff ecting school performance and work productivity, the costs incurred by rhinitis being substantial. Th e Allergic Rhinitis and its Impact on Asthma (ARIA) Initiative has recently developed a state-of-art document including modern guidelines for the diagnosis and treatment of allergic rhinitis42. Allergic rhinitis has been classically described as seasonal if the allergy is to pollen, and perennial if the allergen is present all year round, like the house dust. Th e modern classifi cation, according to ARIA guidelines, is based on symptoms and quality-of-life parameters. It divides the disease into intermittent and persistent, while severity of symptoms is sub-divided into mild and moderate-severe.

DurationIntermittent symptoms:

< 4 days per week• Or < 4 weeks•

Persistent symptoms:> 4 days per week and > 4 weeks•

Severity

Mild:Normal sleep• Normal daily activities• Normal work and school• No troublesome symptoms•

Moderate-severe:Abnormal sleep• Impairment of daily activities• Problems at school or work• Troublesome symptoms•

Table 1. Classifi cation of allergic rhinitis according to the Aria guidelines

3.5.2. Aetiology. Pathophysiology

3.5.2.1. Aetiology:

Inhalants, seasonal or perennial, are the most common • and important group of allergens in nasal atopy (pollens, house dust)Various types of food (wheat, dairy products)• Drugs (aspirine, iodine, antibiotics)•

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3.5.2.2. Risk factors:

Genetics and family history: the best established risk • factor for allergic rhinitis is a family history of allergy 43

Environment: living in developed countries, pollution • and “good hygiene”

3.5.2.3. Co-morbidities:

Allergic rhinitis is a risk factor for the development • of subsequent asthma, is a frequent cause of asthma exacerbations and there is evidence that an eff ective treatment for rhinitis reduces asthma 44

Others: sinusitis, otitis media, sleep disorders, lower • respiratory tract infections

3.5.2.4. Pathophysiology:

Allergens such as grass pollens, house dust mite, cat dander are harmless molecules which do not elicit symptoms in nonatopic individuals. In atopics, these molecules are inhaled and reach antigen-presenting cells in the nose, the most important of which are the dendritic Langerhans cells. Antigen presentation is a critical fi rst step in activating local T lymphocytes. Once activated, these Th 2 cells secrete cytokines (IL-4, IL-13, IL-5) and contribute to the further activation of B lymphocytes. Th is leads to antibody production of the IgE class. Th ese specifi c IgEs coat the surface of the mast cells, which are present in the nasal mucosa. A subsequent exposure to the allergen determines a cross-linkage between the antigen and the IgE attached to the mast cells, leading to immediate and delayed release of a number of pre-formed or newly synthesised mediators (mast cells degranulation)45.

Preformed mediators Newly synthesized mediators

Histamine• Serotonin• Eosinophil chemotactic factor ECFA• Neutrophil chemotactic factor NCF•

Leucotriens LTC4, LTB4• Prostaglandins D2• Kinins• Platelet activating factor PAF• Lipid chemotactic factor HETE•

Table 2. Preformed and newly synthesized mediators released after mast cells degranulation

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Th e immediate mast cells degranulation is responsible for the acute response, but a more prolonged “late-phase” reaction, which develops within a few hours, is common. Th is late response is believed to be associated with the eosinophils.

3.5.3. Symptoms

Sneezing• Profuse watery rhinorrhoea• Nasal obstruction• Itching• Lacrimation•

Perennial allergic rhinitis often presents as chronic infl ammatory rhinosinusitis without acute allergic symptoms.

3.5.4. Diagnosis

A careful and detailed history is mandatory• Inspection: allergic salute, • allergic crease, allergic shinersAnterior rhinoscopy: • pale, bluish, edematous nasal turbinates, coated with a thin, clear, watery secretionNasal endoscopy• A full ENT examination• Plain sinus radiographs, • CT, MRIRhinomanometry, • OlphactometryNasal smears: presence • of eosinophils (their detection in nasal secretions is no longer considered pathognomonic of atopic disease)Allergy tests: skin prick test SPT, radioallergosorbent test • RAST ( detects specifi c circulating IgE antibodies), nasal allergen challenge (allergen is introduced into the nose and any reaction is measured and compared to placebo)Evaluation of the pulmonary function (important co-• morbidity with asthma)

Figure 17. Allergic rhinitis

1 - Inferior turbinate; 2 - Nasal septum

134

3.5.5. Treatment

Environmental control: allergen avoidance whenever and • wherever possiblePharmacotherapy• Immunotherapy (specifi c hyposensitisation): the only one • that off ers the hope of “cure”Surgery is rarely needed•

Medical treatment:Oral antihistamines: the fi rst generation of H1-blockers • (e.g. Chlorpheniramine) had a marked sedative eff ect; the newer, second-generation antihistamines (e.g. Loratadine, Desloratadine, Cetirizine, Ebastine) are non-sedatingTopical antihistamines: Azelastine, Levocabastine• Topical steroids: most eff ective treatment for rhinitis • (Beclomethasone dipropionate, Fluticasone propionate, Fluticasone furoate, Mometasone furoate, Budesonide, Flunisolide)Systemic corticosteroids• Topical anticholinergic agents: Ipratropium bromide• Topical chromones: sodium cromoglycate, Nedocromil• Oral decongestants (pseudo-ephedrine, • phenylpropanolamine) and topical decongestants (oxymethazoline, xylomethazoline)Antileukotriens: Montelukast, Zafi rlukast, Pranlukast•

3.6. Non-allergic perennial rhinitis

Th e term “non-allergic rhinitis” commonly refers to a diagnosis of any nasal condition in which the symptoms are quite similar to those seen in allergic rhinitis, but an allergic aetiology has been excluded. Th ese conditions can broadly be classifi ed as46:

Idiopathic rhinitis: also referred to as vasomotor • rhinitis or non-allergic non-infectious perennial rhinitis (NANIPER)Drug-induced rhinitis• Hormonal rhinitis• Non-allergic occupational rhinitis• Other forms: non-allergic rhinitis with eosinophilia • syndrome (NARES), food-induced rhinitis, rhinitis

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due to physical and chemical factors, emotion-induced rhinitis

3.6.1. Idiopathic rhinitis

Idiopathic rhinitis is characterized by symptoms of nasal obstruction, rhinorrhoea and sneezing. Th e aetiology is unknown in most cases and the disease is thought to be triggered mainly by irritants and changes in atmospheric conditions47.

3.6.2. Drug-induced rhinitis

3.6.2.1. Topical vasoconstrictors

When used topically, the vasoconstriction induced by the nose drops/sprays is so intense that a semi-ischemic state occurs, during which time products of metabolism accumulates that are strong vasodilators. Subsequently, a rebound vasodilation occurs. Th e more frequent and prolonged the use of topical vasoconstrictors is, the more profound the rebound, until a loss of vascular tone develops, accompanied by hypertrophy of the nasal mucosa and nasal hyperreactivity (Rhinitis medicamentosa).

3.6.2.2. Other drugs

Other drugs involved in drug-induced rhinitis: Cocaine abuse, Aspirin, other non-steroidal antiinfl ammatory drugs (NSAIDs), beta-blockers, angiotensin-converting enzyme (ACE) inhibitors, oral contraceptives, psychotropic agents48.

3.6.3. Hormonal rhinitis

Th is form is associated mainly with pregnancy and “pre-menstrual colds”. Estrogens cause vascular engorgement not only in the female genital tract, but also in the nose, leading to nasal obstruction and/or nasal hypersecretion46.

3.6.4. Nonallergic occupational rhinitis

Occupational rhinitis may be defi ned as rhinitis caused by exposure to airborne agents present in the work place. Th ese agents may act via both immunologic (IgE) and non-immunologic mechanisms. Th e non-immunologic triggers are often irritant or toxic small molecular weight compounds (aldehydes, isocyanates, solvents), or may be physical (long-term exposure to cold air)46.

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3.6.5. NARES

Th e term NARES (Non-allergic Rhinitis with Eosinophilia Syndrome) refers to a nasal condition characterized by symptoms similar to allergic rhinitis and the presence of more than 20% eosinophils in the nasal smears. A marked feature of this disease is the lack of evidence of allergy (negative skin tests, absence of serum IgE antibodies) 49.

3.6.6. Other forms of rhinitis

Rhinitis due to physical and chemical factors: cold, dry • air (“skiers nose”), air conditioning, air pollutants Emotionally induced rhinitis: stress, sexual arousal (“post-• coital rhinitis”)50

End-stage vascular atony of chronic allergic or • infl ammatory rhinitis: prolonged parasympathetic stimulation of the nasal vascular system may lead fi nally to permanent loss of vascular tone. Richardson described this condition in 1948 as “nasal turbinate priapism”48

Food-induced rhinitis: spicy foods cause watery • rhinorrhoea, a phenomenon called “gustatory rhinitis”51

3.6.7. Treatment of nonallergic perennial rhinitis

3.6.7.1. Non-surgical management:

Avoid knowing irritants: tobacco smoke• Sleep and work in a cool-air (not cold) environment, • keeping the body warmOral decongestants: pseudo-ephedrine, • phenylpropanolamineOral antihistamines• Topical steroids: the most eff ective treatment•

3.6.7.2. Surgical management:

In cases when nasal obstruction is non-responsive to medical treatment and if the inferior turbinate is hypertrophic, surgery to reduce the size of the turbinate may be an option. Various procedures48 has been shown to be useful:

Inferior turbinate injection with corticosteroids: no more •

137

recommended Electrical or chemical cauterization of the inferior turbinates• Partial turbinectomy (lasers can be used)• Total turbinectomy: no more recommended, because • atrophic rhinitis may develop afterwardsSubmucous resection of the turbinate bone•

Modern approaches include radiofrequency turbinate tissue reduction (Coblation-assisted turbinoplasty) and microdebrider-assisted turbinoplasty52.

3.7. Nasal polyposis

3.7.1. Defi nition

Nasal polyposis is a part of an infl ammatory condition of unknown aetiology, involving the mucous membranes of the nose, the paranasal sinuses and often the lower airways. A nasal polyp presents in the nasal cavity with a grape-like appearance, having a “body” and a “stalk”, a smooth surface and a yellowish color. Nasal polyps originate in the upper part of the nose, around the openings of the ethmoidal cells (middle and superior meatus)53. Th e prevalence rate of nasal polyposis is about 2%. It increases with age. Th e male/female ratio is 2/154.

3.7.2. Aetiology and associated diseases

Th e aspirin triad (Samter’s triad, Widal’s triad): a • medical nonallergic condition consisting of asthma, aspirin sensitivity and nasal polyposis . It represents the most aggressive form of the disease. Th e intolerance is not confi ned to aspirin, as the patients react to other non-steroidal antiinfl ammatory drugs (NSAIDs). Th e aetiology is unknown, but it is widely believed that the disorder is caused by an anomaly in the arachidonic acid cascade, which causes undue production of leukotrienes. When prostaglandin production is blocked by NSAIDs, the cascade shunts entirely to leukotrienes, determining the severe allergy-like eff ects55

Allergy (?): as tissue eosinophilia is a characteristic feature • of most nasal polyps, it has been the belief for decades that allergy is a signifi cant cause of nasal polyposis. However,

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recent studies suggested that IgE-mediated allergy does not play an aetiological roleAllergic fungal sinusitis: nasal polyps occur in almost all • patients with allergic fungal rhinosinusitis. In this disease, the tissue infl ammation is typically eosinophil-dominated56

Cystic fi brosis: nasal endoscopy demonstrated polyps • in 45% of adults with cystic fi brosis 57. In children with cystic fi brosis the prevalence of polyposis is even higher. Any child with nasal polyps should be regarded as having cystic fi brosis until proven otherwisePrimary ciliary dyskinesia (Kartagener’s syndrome)•

3.7.3. Pathogenesis

Th e site of polyp formation is mainly the middle meatus, and they originate from the mucous membrane of the outlets (ostia, clefts, recesses) from the paranasal sinuses. Th is region, so critical for sinus pathology, is also referred as the ostiomeatal complex 53. Contact areas may contribute to the formation of the polyps. Polyps are edematous sacks covered by normal airway epithelium and containing very few nerves, blood vessels and glands with cystic degeneration. Polyps contain degranulated mast cells, very high concentrations of histamine and are characteristically infi ltrated with eosinophils, which are upregulated by cytokines (most importantly IL-5). As eosinophils generate IL-5, attracting more eosinophils, nasal polyps can be considered as a self-perpetuating infl ammatory process53. Various theories involving fungi and “superantigenes” were suggested to explain the presence of eosinophils. According to the fungi theory, eosinophils are attracted to a stimulus (fungus) in the mucus in patients who are immunologically sensitive to fungus58.

3.7.4. Symptoms

Nasal obstruction is the major symptom, constantly present, • but of varying degrees depending on the polyps’ sizeWatery rhinorrhoea, post-nasal drip• Hyposmia/anosmia• Headaches and facial pain may be present• Patients may have symptoms of acute, recurrent, or chronic • rhinosinusitis if the polyps obstruct the sinus ostia

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3.7.5. Diagnosis

Anterior rhinoscopy: fl eshy translucent mass or masses in • the nasal cavity, usually originating in the middle meatusNasal endoscopy• Imaging: plain X-ray, CT scans, MRI• Allergy testing in general is not indicated but it is often • expected by the patientAny child with nasal polyposis must be evaluated for • cystic fi brosis!Unilateral polyposis requires a careful examination in • order to rule out a malignancy!Antro-choanal polyp: it usually originates from the • postero-lateral wall of the maxillary sinus and extends through the ostium towards the posterior choana. It is more common in adolescents and young adults. Endoscopic removal is the primary treatment

3.7.6. Treatment

Medical treatment: topical and systemic steroids• Surgery is not curative and patients must be counselled • that polyps may recur and that repeated procedures may be requiredEndoscopic intranasal procedures (FESS) can range from a • simple polypectomy to a complete sphenoethmoidectomyFollowing surgery, patients usually require a long-term • intranasal steroid administration to prevent recurrences

Figure 18. Nasal polyposis

1 - Nasal polyp; 2 - Middle turbinate; 3 - Nasal septum

Figure 19. Nasal polyposis

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4. RHINOSINUSITIS

4.1. Acute rhinosinusitis


Acute sinusitis (acute bacterial sinusitis ABS, acute bacterial rhinosinusitis ABRS) is an infectious condition characterized by an acute infl ammation of the paranasal sinuses’ mucosa. Th e ostia of the paranasal sinuses are the key to the development of acute sinusitis. Any factor which narrows the ostia will predispose the patient to acute sinusitis. Viral upper respiratory tract infections, allergic infl ammation, nasal polyposis are the most frequently encountered causes. Th e main bacterial agents involved are: Streptococcus pneumoniae and Haemophilus infl uenzae, while Moraxella catarrhalis, Staphylococcus pyogenes, Staphylococcus aureus and various anaerobes are less frequent59. ABRS is frequently encountered in clinical practice, aff ecting between 10 and 15% of the population of central Europe annually60.

4.1.2. Symptoms

History: biphasic evolution of a common cold• Nasal obstruction• Purulent nasal discharge• Facial pain/pressure• Fever, • malaise

4.1.3. Diagnosis

Palpation of the sinusal trigger points (see section 1.4.1.)• Anterior rhinoscopy: congestion, edema of the nasal • mucosa, mucopurulent discharge coming out from the middle meatus

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Nasal endoscopy• Imaging: plain X-ray, CT scans, MRI, ultrasound have • limited value in the routine diagnosis of uncomplicated acute sinusitis Th e gold standard for diagnosis of bacterial sinusitis is • sinus puncture with aspiration of purulent secretions, but this invasive procedure is seldom performed in primary care61

Figure 20. Acute rhinosinusitis

1 - Middle turbinate; 2 - Inferior turbinate; 3 - Nasal septum 4 - Pus in the middle meatus

Figure 21. Left maxillary sinusitis

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4.1.4. Complications

Orbital complications: periorbital cellulitis (most • commonly seen in children with acute ethmoiditis, the upper eyelid becomes swollen without evidence of orbital infection), subperiostal abscess (accumulation of pus at the medial aspect of the orbit), orbital abscess (severe proptosis and ophtalmoplegia, visual acuity is impaired), cavernous sinus thrombosis (usually bilateral, sepsis, orbital pain, chemosis, proptosis and ophtalmoplegia)Osteomyelitis: frontal sinus osteomyelitis with erosion of • the anterior wall of the sinus produces the classic forehead swelling known as Pott’s puff y tumor62

Intracranial complications: meningitis, epidural abscess, • subdural abscess, brain abscess

Figure 22. Left orbital celullitis

4.1.5. Treatment

4.1.5.1. Medical treatment:

Antibiotics: fi rst-line antibiotics (Amoxicillin, • Amoxicillin-clavulanate), second-line antibiotics (second generation cephalosporins, macrolides, fl uoroquinolones with anti-pneumococcal activity, Doxycycline)63

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Oral and topical nasal decongestants• Nasal irrigations with saline, sea-water drops/sprays• Analgetics and antipyretics•


Antral puncture is rarely performed nowadays, since the • appropriate use of antibiotics cures the disease

4.2. Chronic rhinosinusitis

4.2.1. Defi nition. Aetio-pathology

Th e European Position Paper on Rhinosinusitis and Nasal Polyps64 proposed the criteria for diagnosis of chronic rhinosinusitis in adults as 12 or more weeks of persistent symptoms and signs with no complete resolution. Rhinosinusitis (including nasal polyps) is defi ned as infl ammation of the nose and paranasal sinuses, characterized by two or more of the following symptoms: nasal blockage/congestion, nasal discharge (anterior/posterior discoloured), facial pain/pressure, hypo/anosmia plus either endoscopic signs of polyps, mucopurulent discharge from middle meatus, edema/mucosal obstruction primarily in the middle meatus and/or CT scan changes (mucosal changes within the ostiomeatal complex and/or sinuses. Predisposing factors: allergic rhinitis, nonallergic rhinitis and immune defi ciency. Structural abnormalities (concha bulosa, reversed middle turbinate) are no more common in chronic rhinosinusitis than they are in a control group, with the possible exception of septal deviation65. Systemic diseases as Wegener’s granulomatosis and sarcoidosis may present with chronic rhinosinusitis.

4.2.2. Diagnosis

A careful clinical history remains the cornerstone of • diagnosis!Symptoms: see defi nition of rhinosinusitis above• Complete ENT examination• Nasal endoscopy•

144

Radiology: plain sinus X-rays, CT scan (mainly coronal • sections), MRI

4.2.3. Treatment

4.2.3.1. Medical treatment:

Allergen/irritant avoidance• Nasal douching: saline, sea-water drops/sprays• Nasal decongestants (brief use)• Topical corticosteroids• Antibiotics: are needed for acute exacerbations, their • place in the chronic form is controversial

A recent randomized prospective study 66 has revealed that medical treatment in chronic rhinosinusitis is as eff ective as endoscopic sinus surgery combined with topical steroids, both in polypoid and nonpolypoid chronic rhinosinusitis.


In the last decade, many ENT doctors accepted endoscopic • sinus surgery as the standard procedure for the surgical management of chronic rhinosinusitisTh e technique, as proposed by Messerklinger and • championed by Stammberger in Europe and Kennedy in USA, is minimally invasive endoscopic sinus surgery

Figure 23. FESS basic concept

1 - Frontal sinus; 2 - Orbit; 3 - Maxillary sinus; 4 - Inferior meatus; 5 - Inferior turbinate; 6 - Middle turbinate; 7 - Nasal septum; 8 - Middle meatus; 9 - Ostiomeatal complex; 10 - Ethmoidal bulla

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(MISS), or functional endoscopic sinus surgery (FESS)Th e basic principle of FESS is to remove only the diseased • areas (at the level of the ostiomeatal unit), in order to relieve obstruction of the ostia and so restore natural sinus drainage, ventilation and physiology67 Other surgical procedures: antral puncture with lavage, • the Caldwell-Luc radical operation

4.3. Fungal rhinosinusitis


Th e role of the fungi in the nose and paranasal cavities is still unclear. Th ere is poor understanding of when fungi are present as pathogens or simply as part of normal fl ora 68. A range of acute and chronic manifestations are described. Several fungal organisms may be involved: Aspergillus, Scedosporium, Alternaria and Mucor 69. Classifi cation 70:

Noninvasive: sinus fungus ball (mycetoma), allergic fungal • rhinosinusitisInvasive: acute fulminant invasive fungal rhinosinusitis, • chronic invasive fungal rhinosinusitis

4.3.2. Fungus ball

Fungus ball occurs mainly in adults. It is the main fungal • rhinosinusitis in immunocompetent patientsAspergillus is the most common agent involved (• Aspergillus fumigatus, Aspergillus fl avus)

Figure 24. Right maxillary sinus ostium (endoscopic view during

FESS)

1 - Right maxillary sinus ostium; 2 - Middle turbinate; 3 - Nasal septum

146

Unilateral post-nasal discharge is the most frequent • symptom, but some patients are asymptomaticCT, MRI are helpful in diagnosis (MRI is the most • reliable modality to identify fungal sinus disease) 71

Histopathology: no mucosal invasion• Surgery (FESS) is the most eff ective treatment. No • antifungal therapy is required.

4.3.3. Allergic fungal rhinosinusitis (AFRS)

AFRS is a noninvasive form of fungal rhinosinusitis in • immunocompetent patients with allergy to fungusClinical and biological criteria for diagnosis: nasal • polyps, thick mucin, hypersensitivity type 1 for fungus, eosinophilic mucin 69

Treatment: surgical removal of eosinophilic mucin, topical • and oral steroids

4.3.4. Chronic invasive fungal rhinosinusitis

It is probably one of the less frequent forms, occuring in • immunocompetent patients.History of chronic rhinosinusitis• In evolution: bone destruction, invasion into the orbit or • brainTreatment includes a combination of surgery and anti-• fungal therapy

4.3.5. Acute fulminant fungal rhinosinusitis

It occurs in immunocompromised patients (AIDS, • hematologic diseases, type 1 diabetes, chemotherapy, neutropenia). It is characterized by a gangrenous necrosis of the soft tissues and bony structures of the midface with invasion of the orbit and the skull baseEarly diagnosis and control of the primary immunological • disorder are mandatory for the prognosisMucor• and Aspergillus are most frequently encounteredNasal endoscopy, CT, MRI are essential for diagnosis• Treatment: surgery, anti-fungal therapy, re-establishment • of immunity

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5. EPISTAXIS

5.1. Defi nition. Classifi cation

Epistaxis is defi ned as bleeding from the nasal fossae. Classifi cation72:

Adult (>16 years) or childhood (<16 years) epistaxis• Primary (no proven causal factor) or secondary (proven • causal factor) epistaxisAnterior (more common in the young adult) and • posterior epistaxis (more often seen in the older adult with hypertension and arteriosclerosis)

About 90% of recurrent epistaxis occur from the antero-inferior septal region called Little’s or Kiessellbach’s area, where there is a rich vascular anastomotic supply formed by end arteries (see section 1.1.5.).

5.2. Aetiology

About 70-80% of all cases are idiopathic, without any proven precipitant or causal factor73.

5.2.1. Local causes:

Trauma: direct trauma to the nose determining fracture • of the nasal bones, habitual nose picking, nasal surgery, barotraumasInfl ammation• Tumors: benign (juvenile nasopharyngeal angiofi broma • JNA), malignantSeptal deformities: septal spurs and sharp deviations, • septal perforationsForeign bodies• Granulomatous diseases: tuberculosis, syphilis, Wegener’s • granulomatosis, sarcoidosis

148

5.2.2. Systemic causes:

Blood dyscrasias: leukaemias, haemophilias, idiopathic • thrombocytopenic purpuraDrugs: aspirin, non-steroidal antiinfl ammatory drugs • (NSAIDs)Hereditary hemorrhagic teleangiectasia (the Osler-• Weber-Rendu disease)Arteriosclerosis: elderly patients with associated • hypertension

5.2.3. Idiopathic

5.3. Management

Th e patient should be assessed in a semi-recumbent position. Everyone involved should wear protective glasses and clothing as blood aerosol contamination is common, especially when inserting nasal packing74. Four major objectives:

Resuscitation, assessment of the blood loss, evaluation • of associated cardio-vascular co-morbidities, previous medicationsDetermination of the cause: if possible• Determination of the site: if possible• Control of bleeding•

Control of bleeding may be divided into direct (bleeding point-specifi c therapies under endoscopic control in general anesthesia) or indirect treatments, which do not require the precise identifi cation of the bleeding point72. Treatment modalities:

In minimal hemorrhages, pinching the nose with the head • leaned forward to avoid blood swallowing is eff ectiveCauterization of an identifi ed small bleeding area, mainly • in anterior epistaxisAnterior packing (vaseline gauze or expandable packing • with Merocel) and posterior packing. Nasal packing has to be removed after 24-48h. Insertion of nasal balloon catheters•

149

In recurrent life-threatening epistaxis, surgical therapy may be required:

Ligation techniques: anterior/posterior ethmoidal artery • ligation, endonasal sphenopalatine artery ligation, internal maxillary artery ligation, external carotid artery ligationEmbolization under angiographic guidance• 75

Figure 25. Anterior nasal packing

Figure 26. Baloon catheter

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6. TUMORS OF THE NOSE AND PARANASAL

SINUSES

6.1. Benign tumors

Squamous papillomas: in the nasal vestibule• Osteomas: arise in the frontal, ethmoid and maxillary sinuses• Angiofi bromas: see Pharynx, section 3.1.1.1.• Inverted papillomas: commonly arise at the level of the • lateral nasal wall, present as unilateral polyps, are locally aggressive causing bony erosion of the lateral wall, have a high propensity for recurrence after removal and are associated with malignancy in 15% of cases30

6.2. Malignant tumors

6.2.1. Epidemiology. Aetiology

Sinonasal malignancies are uncommon. Th ey account for 0.2-0.8 percent of all malignancies and 3 percent of upper aerodigestive tract neoplasms76. Most develop in the 5th and 6th decades of life. Th e male/female ratio is 2/177. A large number of sinonasal malignancies are caused by inhaled carcinogens78. Risk factors include:

Wood dust • Nickel• Chrome• Polycyclic hidrocarbons• Afl atoxin•

6.2.2. Pathology

Sinonasal malignancies tend to spread by local invasion: orbit, anterior skull base, pterygopalatine fossa, infratemporal fossa, nasopharynx, palate, cheek. Ohngren described a line

151

running from the eye’s medial canthus to the mandible’s angle. Th is line divided tumors into two groups depending on the localization above or below the line. Superiorly based cancers have the tendency to be more aggressive and poorly diff erentiated. Regional spread: only 10 percent of the patients have nodal involvement at the time of presentation78. Depending on the site of origin, maxillary sinus tumors are the most frequent (55 percent), followed by the nasal cavity (35 percent), ethmoid sinuses (9 percent) and rarely frontal and sphenoid sinuses (less than 1 percent)78. Histopathology:

Squamous cell carcinoma is the most common malignancy • (80%)Adenocarcinoma, adenoid cystic carcinoma, olfactory • neuroblastoma (aesthesioneuroblastoma), undiff erentiated carcinoma, mucosal melanoma, hemangiopericytoma

6.2.3. Symptoms

Unilateral nasal obstruction• Muco-purulent/sanguinolent rhinorrhoea• Epistaxis• Facial pain, facial numbness (infi ltration of the infraorbital • nerve)Trismus (invasion of the pterygopalatine and infratemporal • fossae)Diplopia (invasion of the orbit)• Oral symptoms: loose of teeth, inability to wear the • denture

6.2.4. Diagnosis

Inspection: facial asymmetry, proptosis, epiphora, cranial • neuropathies Palpation: neck lymph node metastasis is less frequent• Anterior rhinoscopy and nasal endoscopy reveals a nasal • mass. Nasal endoscopy allows an accurate biopsy.Buco-pharyngoscopy: swelling/ulcerations of the hard • and soft palateHistopathology is mandatory for diagnosis!• Imaging: CT, MRI to assess the disease’s extension•

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Figure 27. Malignant tumor of the left paranasal sinuses

Figure 28. Tumor in the left maxillary sinus

153

6.2.5. Treatment

Surgery combined with radiotherapy/chemotherapy• Orbit exenteration may be required if the tumor invades • the orbitCraniofacial resection: for tumors of the ethmoid sinuses • with invasion of the anterior skull base

Th e prognosis of these sinonasal malignancies has improved over the past three decades, but remains poor overall and is directly related to the degree of local control. Absolute local control rates for all malignancies are 50 percent at 5 years and 31 percent at 10 years78.

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68. Klossek JM, Kauff mann-Lacroix XD. Sinusites fongiques: classifi cation, methodes diagnostique et prise on charge. Journal de Micologie Medicale 2001; 11: 216-221.

69. Klossek JM. Fungal rhinosinusitis. In: Lund VJ, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1449-1457.

70. deShazo RD, Chapin K, Swain RE. Fungal sinusitis. New England Journal of Medicine 1997; 337: 254-259.

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71. Lund VJ, Lloyd G, Savy L, Howard D. Fungal rhinosinusitis. Journal of Laryngology and Otology 2000; 114: 76-80.

72. McGarry GW. Epistaxis. In: Lund VJ, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1596-1608.

73. Stell PM. Epistaxis. Clinical Otolaryngology 1977; 2: 263-273.74. Carney AS, Weir J, Baldwin DL. Contamination with blood during

management of epistaxis. British Medical Journal 1995; 311: 1064.75. Scaramuzzi N, Walsh RM, Brennan P, Walsh M. Treatment of intractable

epistaxis using arterial embolization. Clinical Otolaryngology 2001; 26: 307-309.

76. Rice DH, Stanley Jr RB. Surgical therapy of tumors of the nasal cavity, ethmoid sinus and maxillary sinus. In: Panje W, ed. Comprehensive management of head and neck tumors. 1999; 558-581.

77. Goldenberg D, Golz A, Fradis M, Martu D, Netzer A, Joachims HZ. Malignant tumors of the nose and paranasal sinuses: A retrospective review of 291 cases. Ear, Nose and Th roat Journal 2001; 80: 272-277.

78. McMonagle BA, Gleeson M. Nasal cavity and paranasal sinus malignancy. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2417-2436.

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III. PHARYNX

1. PHARYNX BASICS


Th e pharynx is a “barrel-shaped” anatomical structure, lying at the crossroads between the respiratory and digestive tracts. Th e pharynx is divided into three areas: the rhinopharynx (nasopharynx, cavum), the oropharynx and the hypopharynx (laryngopharynx). Th e boundaries of the three areas are: the cavum is limited superiorly by the skull base (basisphenoid), and inferiorly by a line at the level of the free border of the soft palate; the oropharynx commences at the level of the soft palate and extends to the level of the tip of the epiglottis; the hypopharynx extends from the level of the epiglottis’ tip to the level of the inferior margin of the cricoid cartilage, where it is continuous with the oesophagus1.

1.1.1. Rhinopharynx

Th e superior wall corresponds to the skull base (basisphenoid)• Th e posterior wall lies onto the prevertebral space. At • the junction of the roof and the posterior wall, a mass of lymphoid tissue is noticed (Luschka’s pharyngeal tonsil)Th e anterior wall presents two orifi ces (the posterior nasal • choanae): the communication between the nasal cavities and the nasopharynxTh e inferior wall is formed by the superior surface • of the soft palate and opens into the oropharynx. Th is

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communication is closed by the elevation of the soft palate when swallowingAt the level of the lateral walls, the openings of the • Eustachian tubes (triangular-shaped with a prominent posterior ridge called torus tubarius) are located. Behind and above the torus is the pharyngeal recess (fossa of Rosenmuller), in close proximity to the lower cranial nervesTh is fossa is an important anatomical landmark because • it’s the site where nasoparyngeal carcinoma arises most commonly

1.1.2. Oropharynx

Th e oropharynx is separated from the rhinopharynx by • the mobile, membranous-muscular soft palate. In the midline, the uvula hangs from the soft palate and is occasionally bifi dTh e anterior wall presents the communication between • the oropharynx and the oral cavity (the bucco-pharyngeal isthmus or fauces)At the level of the lateral walls, two folds of muscles • covered by mucosa stretch down on each side from the palate to the lateral border of the tongue and to the lateral wall of the pharynx. Th ese are the anterior (palatoglossus muscle) and posterior (palatopharyngeus muscle) tonsillar pillars, and between them lie the palatine tonsilsTh e posterior wall consists of the constrictor muscles • and overlying mucosa. It corresponds to the prevertebral spaceAt the level of the tongue base lie the lingual tonsil• Between the tongue base and the lingual face of the epiglottis • are two spaces known as the valleculae. Th e valleculae are divided in the midline by the glosso-epiglottic fold and laterally by the pharyngo-epiglottic foldsTh e Waldeyer’s ring consists of a circle of lymphoid tissue • surrounding the opening of the aero-digestive tract (the pharyngeal, palatine and lingual tonsils). Aggregations of lymphoid tissue are also located on the posterior wall, which can bee seen mainly in chronic infl ammation (the “cobble-stoned” pharynx)

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Figure 1. Nasopharynx (posterior view)

1 - Inferior turbinate; 2 - Middle turbinate; 3 - Superior turbinate; 4 - Nasal septum; 5 - Torus tubarius; 6 - Rosenmuller fossa; 7 - Uvula; 8 - Eustachian tube opening ; 9 - Soft palate

Figure 2. Oropharynx (anterior view)

1 - Hard palate; 2 - Anterior tonsillar pillar; 3 - Tongue; 4 - Palatine tonsil; 5 - Posterior tonsillar pillar; 6 - Soft palate (uvula)

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1.1.3. Hypopharynx

It is the inferior part of the pharynx, lying behind and to • the sides of the inner part of the larynxTh e oesophagus commences at the level of the inferior • margin of the cricoid cartilage, corresponding to the 6th cervical vertebraTh e anterior wall: laryngeal inlet (superiorly) and the • post-cricoid area (inferiorly). Th e post-cricoid area is the site of the only head and neck squamous cell carcinoma (SCC) that is more common in women!To each side of the larynx lie the piriform fossae or sinuses, • triangular-shaped spaces with their apices inferiorly (“lateral food channels”). Th ese are bounded laterally by the thyroid cartilage and medially by the lateral surface of the aryepiglottic folds, the arytenoid and cricoid cartilages

1.1.4. The palatine tonsil

Th e palatine tonsil lies within the tonsillar fossa located at the level of the lateral wall of the oropharynx. It’s a triangular-

Figure 3. Oropharynx and hypopharynx (posterior view)

1- Bucco-pharyngeal isthmus (fauces); 2 - Tongue base; 3 - Epiglottis; 4 - Aryepiglottic fold; 5 - Piriform sinus; 6 - Prominence over cricoid cartilage (retrocricoid area)

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shaped space delineated by the anterior tonsillar pillar, the posterior tonsillar pillar and the tongue base, inferiorly. Th e medial surface of the tonsil is characterized by the presence of numerous tonsillar crypts. Th e deep surface of the tonsil is covered by a fi brous capsule. Th e space between this capsule and the lateral wall of the pharynx is fi lled with loose areolar tissue, which enables a fi ne tonsillar dissection during tonsillectomy. Th is is also the site where peri-tonsillar abscesses develop.

1.1.5. The pharyngeal muscles

Th e pharyngeal wall consists of four layers. From the inside out, are the mucous membrane lining, the pharyngobasilar fascia (pharyngeal aponeurosis), the muscular layer and the buccopharyngeal fascia2. Th e muscular layer consists of three constrictor muscles (superior, middle and inferior constrictors). When contracting in succession, these muscles squeeze food downwards to the oesophagus. Th e lower fi bers of the inferior constrictor muscle are called the cricopharyngeus muscle. Th is muscle relaxes during swallowing, thus acting as the upper oesophageal sphincter. Because the pharynx must allow air to fl ow between nose and larynx, and food from mouth to oesophagus, a complex system of muscle coordination is required to close the communication between the rhinopharynx and oropharynx, and the laryngeal inlet at appropriate times, and so keep these two functions separately. Th e communication between the rhinopharynx and the oropharynx is closed by the elevation of the soft palate during swallowing. Th e muscles which control the soft palate are the levator veli palati, tensor veli palati, palatoglossus and palatopharyngeus. Th e tensor veli palati and levator veli palati muscles also control the patency of the eustachian tube.

1.1.6. Nerve supply of the pharynx

Th e motor innervation to the constrictors and the soft palate is supplied by branches from the vagus nerve (X). Th e sensory nerve supply of the rhinopharynx is from branches of the maxillary nerve (V). Branches from the glossopharyngeal nerve (IX) supply sensory innervation of the oropharynx. Branches from the internal laryngeal nerve (X) supply the hypopharynx.

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1.1.7. Pharyngeal vasculature

Arterial blood supply comes from branches of the external carotid artery (ascending pharyngeal artery, ascending palatine artery, facial artery, maxillary artery). Venous drainage is directed to the internal jugular vein. Lymphatics drain to the retropharyngeal and laterocervical lymph nodes.

1.2. Phisiology

1.2.1. Deglutition (Swallowing)

Th e pharyngeal stage of the swallowing is the most physiologically important, because airway protection occurs during this stage in healthy people 3. Th is second stage of deglutition is a refl ex one, following the oral voluntary stage (mastication and food bolus formation) and preceding the 3rd refl ex oesophageal stage. Th e tongue forces the bolus through the oropharyngeal isthmus into the oropharynx. Th e palatoglossus muscle (anterior tonsillar pillar) contracts in order to prevent refl ux in the oral cavity. Th e larynx is elevated under the tongue base, the soft palate elevates, closing the communication between the rhinopharynx and the oropharynx. Th rough a combination of tongue thrusts and pharyngeal muscular contractions, the food is propelled through the lateral aspects of the hypopharynx (the piriform fossae) into the oesophagus 4. A concomitant relaxation of the cricopharyngeus muscle (upper oesophageal sphincter) occurs.

1.2.2. Phonation

Th e pharyngeal cavities participate to the subsequent modulation of the fundamental sound generated by the vocal folds vibration.

1.2.3. Upper airways protection

Protection against food aspiration during swallowing (see • section 1.2.1)Protection against viral and bacterial infections: the •

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Waldeyer’s lymphatic ring (the pharyngeal, palatine and lingual tonsils) is the fi rst-line defense mechanism of the human body

1.2.4. Taste perception

Th e bitter taste is perceived at the level of the tongue base, sensorial innervation being supplied through branches from the glossopharyngeal nerve (IX).

1.3. Symptoms of pharyngeal diseases

1.3.1. Pharyngeal pain

Pharyngeal pain (sore throat, pain in the throat, odynophagia) is typically augmented by food swallowing and may irradiate in the ear.

1.3.2. Dysphagia

Dysphagia is defi ned as having diffi culty in swallowing, which may aff ect any part of the swallowing pathway from the mouth to the stomach. Patients complain that food or liquids are no longer being swallowed easily and that there is a sensation of food sticking. Additional symptoms may include early (pharyngeal blockage) or late (oesophageal blockage) regurgitation, odynophagia, referred otalgia, hoarseness, coughing after eating. Causes of dysphagia: congenital, trauma, foreign bodies, infections, infl ammatory, oesophageal motility disorders, tumors, neurological 5.

1.3.3. Upper airway obstruction

Obstruction at the level of the rhinopharynx (adenoids, tumors) causes nasal blockage, oral breathing, nasonated speech. Obstruction at the level of the oro- and hypopharynx (edema, tumors, foreign bodies) may cause signifi cant upper airway obstruction (dyspnea, stridor) requiring emergengy procedures: intubation, tracheostomy.

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1.3.4. Lump in the throat

Lump in the throat (globus pharyngeus, globus hystericus, pharyngeal paresthesias) is an abnormal feeling of something stuck or a sensation of a lump or tightness in the throat 6. Hippocrates fi rst mentioned the term globus, and regarded it as a disease of women being inextricably involved in the uterine axis from which all hysteria was believed to be derived at that time 7. Globus accounts for almost 4 percent of otolaryngological referrals 8. Up to 45 percent of the general population have mild, intermittent symptoms resembling globus at some time in their lives 9. Globus is considered functional when no organic explanation is detected. It must be always a diagnosis of exclusion, only after a complete and accurate ENT examination is performed! Th e most popular organic aetiological theory of recent years is that globus is an atypical manifestation of silent gastro-oesophageal refl ux disease (GERD) or oesophageal dysmotility10.


1.4.1. Examination of the rhinopharynx

1.4.1.1. Inspection and palpation

Inspection may reveal the characteristic adenoid face, • in children with long-term untreated adenoidsPalpation of the rhinopharynx: not recommended• Palpation of the cervical lymph nodes (infl ammatory • or metastatic)

1.4.1.2. Posterior rhinoscopy

Before endoscopy was available, visualization of the nasopharynx was one of the most diffi cult clinical examinations in ENT. Instruments:

Light source• Head mirror• Small rhinopharyngeal or dental mirrors• Tongue depressors• A spirit lamp•

Th e examiner is in front of the seated patient, light beam is

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focused on the oral cavity. Previously, the small mirror is warmed with the spirit lamp, to avoid blurring. Th e temperature of the mirror is checked on the examiner’s back of the hand. Ask the patient to open the mouth and keep the tongue within the oral cavity. Press the anterior two thirds of the tongue with the tongue depressor and ask the patient to breath simultaneously through the mouth and nose. Th is will relax the soft palate. Th e small mirror is gently introduced through the oral cavity, behind the uvula, and the rhinopharyngeal walls are examined. Touching the posterior wall of the pharynx with the mirror causes gagging refl exes, which may compromise the examination.

1.4.1.3. Endoscopy of the rhinopharynx

Endoscopy became in the last years the gold-standard method for an accurate examination of the rhinopharynx. Instruments:

Light source• Fiber optic cable• Endoscopes: nasal rigid telescopes of 25 cm length, 4 • mm diameter and angulations of 0º and 30º are most commonly used. Smaller endoscopes are also available (2.7 mm diameter). More recently, 45º and 70º endoscopes have been developed. Fiber optic fl exible endoscopes can also be used. Laryngeal rigid telescopes (90º) can be used for trans-oral endoscopyStraight and curved suction tips, fl exible metal cotton-• tipped applicators, straight and upbiting forceps, anti fog solutionsTopical nasal decongestants • and anaestheticsVideo and digital recording • equipment, if available

After anesthetizing the nares with topical lidocaine spray, many otolaryngologists will spray the nares with a decongestant. Th e endoscope (fl exible or rigid) is then gently passed along the fl oor of the nasal cavity, beneath the inferior turbinate. Th e eustachian

Figure 4. Nasopharynx (endoscopic view)

1 - Eustachian tube opening: 2 - Torus tubarius

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tube orifi ce, torus tubarius and Rosenmuller fossa are carefully inspected on each side. Arguably, the best view of all may be obtained using a 90º rigid scope in the oropharynx. By advancing the rigid scope through the mouth and by placing the beveled edge posterior to the soft palate, the nasopharynx may bee seen in its entirety 11.

1.4.2. Examination of the oropharynx


Palpation of the tongue base (using a glove) may reveal • indurated areas, which may require a further biopsyPalpation of the cervical lymph nodes (infl ammatory or • metastatic)

1.4.2.2. Oropharyngoscopy

Instruments:Light source• Head mirror• Tongue depressors•

Th e examiner is in front of the seated patient, light beam is focused on the oral cavity. Ask the patient to open the mouth and keep the tongue within the oral cavity. Gently apply the tongue depressor over the anterior two thirds of the tongue, in order to avoid gagging refl exes. Ask the patient to say “a”, to check the mobility of the soft palate, which normally elevates in the midline. Th e motor innervation to the soft palate is supplied by branches of the vagus nerve (X). A unilateral paralysis will determine a deviated elevation of the palate towards the healthy side. Examine the palatine tonsil, the tonsillar pillars, the posterior wall of the oropharynx. Th e tongue base should be examined using a laryngeal mirror or endoscopy.

1.4.3. Examination of the hypopharynx


Palpation of the cervical lymph nodes (infl ammatory or • metastatic)

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1.4.3.2. Indirect laryngoscopy

Th e hypopharynx is visible only during indirect • laryngoscopy (see examination of the larynx)

1.4.3.3. Endoscopy

Endoscopy (fl exible or rigid) provides the most accurate • examination of the hypopharynx (see examination of the larynx)

1.4.4. Other investigations

Bacteriological examinations• Biopsy• Imaging: CT scans, MRI• Functional investigations of the upper gastrointestinal tract • (dysphagia): radiology (barium swallow, videofl uoroscopy, CT and MRI), pharyngeal and oesophageal manometry, oesophageal and pharyngeal pH monitoring

Figure 5. Oropharyngoscopy

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2. ACUTE AND CHRONIC PHARYNGEAL

INFECTION

2.1. Acute viral pharyngitis


Acute pharyngitis is an infl ammatory infectious condition aff ecting the pharyngeal mucosa. Often viral in origin (bacterial superinfection may occur during evolution), it usually involves the entire pharyngeal mucosa, is very contagious and is characterized by a seasonal pattern. Viral causes of acute pharyngitis, in order of frequency, include rhinovirus, adenovirus (5%), parainfl uenza virus, Coxsackie virus, corona virus, echovirus, herpes simplex virus (<5%) 12.

2.1.2. Symptoms

Sudden onset: fever, • malaise, headaches, painful dryness of the throat (hyperesthesia of the pharyngeal mucosa), odynophagia (sore throat)Symptoms of nasal disease may be associated: nasal • obstruction, nasal dischargeTh e disease follows a short course, usually resolving • spontaneously within 5-6 days

2.1.3. Diagnosis

Palpation: there is seldom any cervical adenopathy• Oropharyngoscopy: generalized hyperemia of the entire • pharyngeal mucosaA complete ENT examination should be performed: pharyngitis • is frequently associated with acute rhinitis, laryngitisSerologic assessment of antiviral antibodies titers is not • a routine laboratory procedure; it is used mainly for scientifi c purposes

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2.1.4. Treatment

Treatment is symptomatic: analgetics, antipyretics, bed • rest, hot drinksAntibiotics are of no value (it’s a viral infection); may • be recommended in immunocompromised patients to prevent bacterial superinfectionLocal treatment: topical disinfectants (sprays, gargles, tablets)•

2.2. Acute bacterial pharyngitis (Follicular tonsillitis)


Th e disease remains localized to the tonsillar tissue and is only moderately contagious. Group A beta-haemolytic streptococci (GABHS) are the commonest cause of acute bacterial pharyngitis and are spread via respiratory secretions through close contact. More than 120 M-protein types of GABHS have been isolated, with serotypes 1, 3, 5, 6, 18, 19 and 24 associated with rheumatic fever and others, such as serotypes 49, 55 and 57, associated with acute poststreptococcal glomerulonephritis 12.

Figure 6. Acute viral pharyngitis

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Pneumococcus may also be responsible, Gram-negative microorganisms are rarely involved.

2.2.2. Symptoms

Th e i• nitial symptoms, both local and general, do not diff er from those of erythematous pharyngitisSevere odynophagia (extremely painful swallowing); it • may be associated with referred otalgiaHigh fever, • chills

2.2.3. Diagnosis

Palpation of th• e neck: tender anterior jugulo-digastric cervical lymphadenopathyOropharyngoscopy: the tonsils are enlarged, very red, • covered with a grayish-white exudate which can be easily detached with a cotton probeWhite blood count is usually raised, around 10 - 12,000/• mm3 (leukocytosis) associated with elevated erythrocyte sedimentation rate (ESR) valuesTh roat culture is described as the gold-standard • diagnostic test in the North American literature, but recent evaluations recommend that throat swabs should not routinely be carried out in sore throat 13

Figure 7. Follicular tonsillitis

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Complications are rare nowadays, peri-tonsillar abscess being commonest, arthritis, endocarditis, nephritis being much less frequent.

Suppurative: peri-tonsillar abscess, parapharyngeal • and retropharyngeal abscesses, descending necrotizing mediastinitis, septicemiaToxin-mediated: toxic shock-like syndrome• Non-suppurative, immune-mediated: rheumatic fever, • poststreptococcal glomerulonephritis, pediatric autoimmune neuropsychiatric disorder and acute disseminated encephalomyelitis (similar to Sydenham’s chorea) 12

2.2.5. Treatment

Antibiotics are mandatory: best antibiotic to start • treatment is still Penicillin!If the patient is allergic to Penicillin, it is recommended • to use ErythromycinIf the responsible agent is Gram-negative, broad-spectrum • antibiotics are indicatedBed rest, hydration, analgetics, antipyretics, topical • disinfectantsIn recurrent cases (> 3-4 episodes per year): tonsillectomy•

A recent assessment of Cochrane reviews suggests that for streptococcal tonsillitis the use of antibiotics seems to be discretionary rather than prohibited or mandatory. Th is is because the benefi t in terms of symptoms is only about 16 hours compared with placebo, and that serious complications such as rheumatic fever and glomerulonephritis are now rare in developed countries14.

2.3. Acute adenoiditis


Acute adenoiditis is an infl ammatory condition of the Luschka’s pharyngeal tonsil, determined by a bacterial superinfection during the evolution of a viral upper respiratory tract infection. It is one of the most frequent diseases in childhood.

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Th e bacterial agents involved are Streptococcus pneumoniae, Haemophilus infl uenzae, Moraxella catarrhalis and staphylococci.

2.3.2. Symptoms

nasal obstruction, mucopurulent rhinorrhoea, nasonated • speechfever, chills, • malaise

2.3.3. Diagnosis

Palpation: upper cervical infl ammatory lymphadenopathies• Anterior rhinoscopy: congestion of the nasal mucosa • which is covered by mucopurulent secretionsEndoscopy of the rhinopharynx: the pharyngeal tonsil • is congested, hypertrophied and covered by a yellowish exudateOropharyngoscopy: congestion of the pharyngeal mucosa, • mucopurulent secretions on the posterior pharyngeal wall


Acute suppurative otitis media, otitis media with eff usion• Retropharyngeal abscesses•

2.3.5. Treatment

Antibiotics: Amoxicillin, Amoxicillin-clavulanate• Nasal decongestants, analgetics, antipyretics• Hydration•

2.4. Suppurative complications of acute bacterial pharyngitis

2.4.1 Peritonsillar abscess (Quinsy)


Peritonsillar abscess is a collection of pus developed between the fi brous capsule of the tonsil, usually at the upper pole, and the lateral pharyngeal wall. It generally arises as a complication of acute tonsillitis. It may happen at any age, but the majority is in young adults between 20 and 39 years of age.

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Th e aetiology of peritonsillar abscesses involves the usual beta-haemolytic streptococcal infection, associated with the presence of anaerobic organisms. A recent study 15 noted an association between periodontal disease and quinsy, which may represent a source of anaerobic organisms. Infection extends beyond the capsule into the loose peri-tonsillar space, where produces a cellulitis and then an abscess. Th e abscess is most commonly sited anteriorly, pushes the tonsil medially, inferiorly and posteriorly.

2.4.1.2. Symptoms

Debut: unilateral progressive pharyngeal pain, referred • otalgia, moderate feverAfter 2-3 days of evolution: extremely severe odynophagia • with referred otalgia, drooling of saliva, trismus (pathognomonic), rhinolalia, fever, deterioration of the general condition

2.4.1.3. Diagnosis

Palpation of the neck: ipsilateral jugulodigastric lymph • nodes are enlarged and tenderOropharyngoscopy: unilateral oropharyngeal swelling at • the level of anterior tonsillar pillar and the soft palate, the tonsil is displaced medially and covered by soft exudate , uvular edema

Figure 8. Peritonsillar abscess (Quinsy)

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Th • e abscess takes 5-8 days to accumulate and may rupture spontaneously if it is not incisedDeep neck space infections and mediastinitis have been • described in 1.8% of cases16. Mediastinitis is a condition with signifi cant mortality (23%) even with aggressive management (combined neck and mediastinal drainage, as well as appropriate antibiotics)17

2.4.1.5. Treatment

Admission to hospital and treatment with intravenous • antibiotics should be the baseline treatment off ered to all patientsSurgical management: incision through the anterior pillar • with pus’ evacuation, preceded by needle aspiration to confi rm the presence of pus and to guide the site of incisionIntravenous antibiotics, steroids (extensive edema), analgetics• Tonsillectomy, in case of recurrences•

2.4.2. Parapharyngeal abscess


Th e parapharyngeal space lies on either side of the superior pharynx (rhino- and oropharynx), lateral to the superior constrictor muscles. It contains the internal carotid artery, the internal jugular vein, the last four cranial nerves and some lymph nodes. Parapharyngeal abscess is a collection of pus developed at the level of the parapharyngeal space. Th e most common causes are acute bacterial tonsillitis, peritonsillar abscesses and dental infections. Th e bacteriology of deep space neck infections appears to change with increasing numbers of cases being due to gram-negative aerobic infections, which will not respond to fi rst-line penicillin treatment and may be contributing to the signifi cant mortality still associated with these conditions18.

2.4.2.2. Diagnosis

Clinical features are similar to the peritonsillar abscess, except • that maximum swelling in the pharynx is more inferiorly placed and behind the tonsil, with less uvular edemaPalpation of the neck: tender, fi rm but fl uctuent swelling• Neck ultrasound, CT scans•

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Complications: carotid sheath vessel thrombosis, • necrotizing fasciitis, mediastinitis

2.4.2.3. Treatment

Admission to hospital for intravenous antibiotics is the • baseline treatmentFailure to respond to conservative therapy within 48 • hours should prompt surgical abscess drainage

2.4.3. Retropharyngeal abscess


Th e retropharyngeal space lies behind the pharynx, just anterior to the prevertebral fascia. Th is space contains two layers of lymph nodes (Gillette’s nodes). Th e retropharyngeal abscess occurs mainly as a complication of acute adenoiditis in children. Adults develop rarely retropharyngeal abscesses, the most frequent causes being tuberculosis and foreign body penetration 4.

2.4.3.2. Diagnosis

Neck stiff ness, odynophagia, drooling, dyspnoea and • dysphagiaFever, chills, • malaise Examination reveals a fl uctuant mass at the level of the • posterior pharyngeal wallX-ray, ultrasound, CT scans•

2.4.3.3. Treatment

Surgical drainage• High-dose intravenous antibiotics, analgetics•

2.5. Specifi c acute bacterial pharyngitis

2.5.1 Vincent’s angina


Vincent’s angina is an unilateral ulcerative pharyngitis, which evolves in two stages: the fi rst being pseudo-membranous and the second ulcerative. It is caused by an association of fusiform bacilli

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and spirochaete (Treponema vincentii, Spirochaeta denticulata, Fusobacterium necroforum). Infection arises in times of overcrowding and is usually found in young adults with poor oral hygiene, chronic gingivo-dental infections and lowered resistance4.

2.5.1.2. Diagnosis

History: slow, insidious onset• Sore throat, unilateral odynophagia, mild fever• Oropharyngoscopy: examination in early stages reveals • a friable, necrotizing, exudative false membrane on one tonsil. Later, in evolution, ulceration appears (irregular, fairly deep, soft margins, covered by a yellowish exudate)Laboratory tests, biopsy (to rule out a malignancy and • other ulcerative anginas)

2.5.1.3. Treatment

aggressive oral hygiene• antibiotics (penicillin and metronidazole), topical • disinfectants

2.5.2. Diphtheria


Diphtheria is a pseudo-membranous pharyngitis, caused by Corynebacterium diphtheriae (the Klebs-Loeffl er bacillus), a facultative aerobic gram-positive agent. Th is condition has now become exceedingly rare. Th e bacillus, spread by air-borne droplets, remains at the level of the pharynx, causing the local typical manifestations of the disease. In the meantime, it produces a strong neurotropic exotoxin which is spread into the organism, causing serious systemic manifestations.

2.5.2.2. Symptoms

Th e common form is usually seen in children, the early • symptoms being insidiousMalaise• , mild fever, headachesSevere sore throat•

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2.5.2.3. Diagnosis

Palpation of the neck: extensive cercical lymphadenopathy• Oropharyngoscopy: pearly-white, velvety, fi rm, extremely • adherent pseudomembranes covering the tonsils, and then extending progressively onto the tonsillar pillars and the soft palate. When these pseudomembranes are detached, the underlying surface bleeds easilyTh e “malignant form” of the disease: extension of the • pseudomembranes to the entire pharynx, nose and larynx, cardiac disorders, peripheral and cranial nerve palsies. Th is form is generally fatal within 10-12 daysDirect cultures should be obtained for smear and culture • (Klebs-Loeffl er medium) tests

2.5.2.4. Treatment

Early administration of antitoxin is mandatory, based • only on clinical suspicion, without confi rmation of the result of the throat culture!Antibiotics should be also administered: Penicillin G is • requiredTh e patients need to be isolated and reported•

2.5.3. Scarlet fever


Scarlet fever is caused by group A beta-haemolytic streptococci (GABHS) which produce a pathognomonic erythrogenic exotoxin. Some authors consider scarlet fever as a nonsuppurative complication of follicular tonsillitis 4. Th e pharyngitis constitutes both the portal entry for this exanthematous fever and the fi rst sign of the disease.

2.5.3.2. Diagnosis

History: sudden onset with high fever, chills, headaches, • nausea, vomiting, tachycardiaIntense dysphagia and odynophagia• Oropharyngoscopy: a diff use erythematous pharyngitis • contrasting with the white appearance of the dorsum of the tongue. Th e descuamation of the tongue causes the characteristic “strawberry tongue” within 5-6 days

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Th e exanthema appears the following day, making the • diagnosis easy. It begins on the thorax, spreading all over the body and excluding the perioral region

2.5.3.3. Treatment

Antibiotics: Penicillin for 10 days• Topical disinfectants, analgetics•

2.6. Specifi c viral pharyngitis

2.6.1. Infectious mononucleosis (IMN)


Infectious mononucleosis is an acute, systemic viral infection presenting typically with sore throat and generalized lymphadenopathy (glandular fever). It aff ects primarily young adults. Transmission is via saliva (the “kissing disease”). Th e causative infectious agent is the Epstein-Barr virus (EBV), one of six human herpes viruses isolated from blood, lymph nodes and saliva 12.

2.6.1.2. Symptoms

History: there is a prodrome of 4-5 days with • malaise, fatigue and headachesOdynophagia, dysphagia•

2.6.1.3. Diagnosis

Palpation: generalized tender lymphadenopathies (100% • of the patients) Oropharyngoscopy: pharyngeal signs range from acute • follicular tonsillitis, indistinguishable from classic streptococcal tonsillar infection, to pseudo-membranous forms, even ulcero-necroticOccasionally, all the lymphoid tissue of the Waldeyer’s • ring may enlarge and become covered with pseudo-membranesSplenomegaly (50% of the patients), hepatomegaly (10%)• A typical rubelliform skin rash develops almost invariably • if ampicillin is prescribed

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Leukocytosis (10-20,000/mm• 3, even 40,000/mm3) with an inversion of the ratio of the white cells and presence of abnormal monocytesPaul Bunnell test: evidence of heterophile antibodies • (agglutinins to sheep and horse red cells)Th e gold-standard serologic testing: evidence of EBV-• specifi c antibodies (IgM to the EBV viral capsid antigen)12

Diff erential diagnosis: Cytomegalovirus (CMV) • infection, toxoplasmosis, HIV, acute streptococcal tonsillitis, leukaemiaTh e course of the disease is benign and prolonged for 2-3 • weeks, followed by a period of malaise and chronic fatigue

2.6.1.4. Treatment

Symptomatic for mild-to-moderate cases• If there is super-added infection, antibiotics and steroids can • be prescribed. Ampicillin-based antibiotics should be avoided

2.6.2. Cytomegalovirus infection (CMV)

CMV infection may occur in non-immunocompromised or immunocompromised patients (HIV). Th e clinical aspects are similar to IMN. Ragnaud reported pharyngitis is much less common in adult CMV than with adult EBV mononucleosis. In children, CMV pharyngitis is more common than in adults 19. Serological diagnosis is confi rmed with ELISA test (anti CMV IgM) or complement fi xation test.

2.6.3. Herpangina

Herpangina is a self-limiting vesicular eruption that occurs on the oropharyngeal mucosa. A number of enteroviruses (30 and 71) and Coxsackie virus group A are involved in aetiology. Its regular spread to the oropharynx diff erentiate it from herpes simplex type 1 infection, which aff ects mainly the oral cavity 12.

2.6.4. Hand, foot and mouth disease

Th is condition is caused by enterovirus 71 or Coxsackie viruses, and is characterized by vesicular eruptions in the oral cavity and oropharynx, accompanied by vesicles on the hands and feet, fever, malaise, vomiting.

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A fulminant form may lead to severe neurological complications, acute pulmonary embolus and cardiopulmonary decompensation 12.

2.6.5. Herpes viruses

Type 1 herpes simplex infection usually aff ects young children. It causes vesicular and ulcerative lesions of the lips, tongue, gums, buccal mucosa and occasionally oropharyngeal mucosa. Herpes zoster pharyngitis is characterized by the presence of unilateral painful vesicular lesions.

2.7. Pharyngeal manifestations in blood disorders

2.7.1. Agranulocytosis


Agranulocytosis is characterized by a considerable decrease in the number of polymorphonuclear leucocytes in the peripheral blood. Pharyngeal manifestations are frequent when their absolute number falls below 1000/mm3 . Th is condition may present as pure agranulocytosis or as part of a pancytopenia syndrome. Th e history may reveal the cause: sensitivity to a drug (amidopyrine, phenylbutazone, chloramphenicol, sulphonamides, antithyroid drugs, oncological chemotherapy), exposure to various agents (benzene) 1.

2.7.1.2. Diagnosis

Severe dysphagia and odynophagia, foetid breath• Th e general health is profoundly aff ected• Oropharyngoscopy: pharynx and later the mouth are • covered fi rst by false membranes and then by necrotic ulcers which bleed (in pancytopenia), and extend rapidlyHematologic tests•

2.7.1.3. Treatment

Massive doses of antibiotics, steroids, blood transfusions•

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2.7.2. Acute leukemia


Leukemia is a generalized neoplastic proliferation of leukocytes and their precursors. Patients with leukemia are more susceptible to severe infections because of the ineff ectiveness of the leukocytic and antibody response. Chemotherapy may further depress the leukocytic response. Hemorrhages are frequent because of the decreased number of platelets, and are quite diffi cult to control 20.

2.7.2.2. Diagnosis

Inspection and palpation: severe paleness (anemia), • generalized lymphadenopathy, hepato/splenomegalyOropharyngoscopy: ulcero-necrotic lesions of the • pharyngeal mucosa, which may bleed; leukemic infi ltrations of the gingiva and oral cavity mucosa (hypertrophic gingivo-stomatitis), of the tonsillar tissue (tonsillar enlargement)Hematologic tests•

2.7.2.3. Treatment

Is conducted by the hematologist• Pharyngeal infections are treated with massive doses of • antibiotics, steroids

2.8. Chronic non-specifi c conditions

2.8.1. Chronic non-specifi c pharyngitis


Chronic infl ammation of the pharynx occurs when the mucosa is irritated over a long period of time. Chronic infl ammation of the pharyngeal mucosa may present in two diff erent forms:

Chronic catarrhal hyperplastic pharyngitis• Chronic atrophic pharyngitis•

Extrinsic and intrinsic factors are involved in the aetiology4:

184

Extrinsic factors: nicotine, alcohol, chemical inhalational • irritants, allergens, radiotherapyIntrinsic factors: acute and chronic infections (tonsillitis, • rhinosinusitis, bronchitis), gastroesophageal refl ux disease (GERD)

2.8.1.2. Diagnosis

Persistent sore throat, foreign body sensations, coughing • and hawkingIn the catarrhal form pharyngeal mucosa is red, dotted • with hypertrophic lymphoid follicles, mainly posterior to the posterior pillar, even granulations (the “cobble-stoned” pharynx)Atrophic pharyngitis is often the fi nal stage of the • catarrhal form and manifests itself by dryness of the mucosa and sometimes crusting; atrophic pharyngitis can also develop secondary to radiotherapy for head and neck tumorsInvestigations for gastroesophageal refl ux disease • (GERD)

Figure 9. Chronic nonspecifi c pharyngitis

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2.8.1.3. Treatment

Elimination of predisposing factors and mucosal care• Vitamin A, topical disinfectants, non-steroidal • antiinfl ammatory drugs (NSAID’s)

2.8.2. Chronic tonsillitis


Chronic infection can often be found in large cryptic tonsils, in small sclerosed tonsils and in tonsillar remnants left behind by incomplete tonsillectomy. Th ese recurrent infections lead to permanent infl ammation in the tonsillar crypts and scarring of the tonsillar tissue. Th e organisms responsible for chronic tonsillitis are similar to those encountered in acute infections. Chronic infected tonsils are considered to be a “focus” which may activate other chronic infl ammatory diseases in the body by spreading bacteria and toxins4.

2.8.2.2. Diagnosis

History: recurrent or persistent sore throat, dysphagia, • malaise, fetid breathPalpation: infl ammatory upper laterocervical adenopathies•

Figure 10. Chronic hypertrophic tonsillitis

186

Oropharyngoscopy: tonsils may appear as hypertrophic, • normal or scarred/atrophic, often with surrounding peritonsillar erythematous tissuePresence of pus when the tonsils are squeezed• Blood tests: elevated ESR, white blood cells as well as the • concentrations of antistreptolysin may increaseTh e most frequent complications: peri-tonsillar abscesses• Chronic suppurative tonsillitis must be diff erentiated • by the chronic caseous tonsillitis which is characterized by the presence of crypts full of white concretions, the product of maceration of food debris (caseum), it is not accompanied by suppuration, no complications occur, no need for tonsillectomy

2.8.2.3. Treatment

Conservative: analgetics, topical disinfectants, antibiotics • when indicatedSurgery: tonsillectomy is generally suggested when • patient complains of more than fi ve to seven episodes of recurrent infections per year or several infections in two or more subsequent years4

2.8.3. Adenoids


Hyperplasia of the pharyngeal tonsillar tissue (Luschka’s pharyngeal tonsil), owing to chronic infl ammation or allergy of the upper airways. Hyperplastic adenoids are found almost exclusively in children. Enlarged adenoids after the age of puberty and in adults is a very rare condition, various alternative diagnoses must be taken into account (hysthopathology).

2.8.3.2. Symptoms

Nasal obstruction, mucopurulent rhinorrhoea, • nasonated speechChronic oral breathing, sleep disorders, snoring, • obstructive sleep apnoea syndromeConductive hearing loss• Delay in development•

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2.8.3.3. Diagnosis

Inspection: severe long-standing obstruction can produce • the dull expression known as the “adenoid face” (narrow, pale, dumb facial expression with open mouth and sunken eyes)Palpation: cervical lymphadenopathies• Anterior rhinoscopy: congestion of the nasal mucosa, • mucous secretions on the walls of the nasal cavitiesPosterior rhinoscopy/endoscopy of the nose and • rhinopharynx reveals the hypertrophied lymphoid tissue, fi lling the cavumOrophar yngoscopy : • dental malocclusion, gothic palate, tonsillar hyperplasia, secretions on the posterior oropharyngeal wallOtoscopy/microscopy • of the ear: retracted eardrum with or without middle ear eff usionHearing tests: • tympanometry, pure tone audiometry (conductive hearing loss)Biopsy: if there is a suspicion of neoplasm•


Recurring nasal, sinus and tonsillar infections• Persistent dysfunction of the Eustachian tube, followed • by otitis media with eff usionRecurrent acute suppurative otitis media•

2.8.3.5. Treatment

2.8.3.5.1. Conservative treatment: is only appropriate preoperatively (e.g. acute rhinosinusitis) or when surgery is contraindicated (e.g. cleft palate): antibiotics, nasal decongestants, immunostimulants (Broncho-Vaxom)

Figure 11. Adenoids (endoscopic view)

188


Th e recommended European standard21 is:Surgical removal of the adenoids is the fi rst-line • treatment!Adenoidectomy: any adenoid hyperplasia with chronic • nasal obstruction, recurrent or persistent rhinosinusal infections, chronic purulent rhinorhoea, persistent or recurrent dysfunction of the Eustachian tube, otitis media, chronic bronchitis or in sleep apnoea syndromeSleep apnoea syndrome: as a rule with simultaneous • tonsillectomyAdditional useful procedures: myringotomy with • grommets’ insertion

Figure 12. Th e Beckmann curette

Adenoids are removed with a special curette (Beckmann’s adenotome) in general or local anesthesia. Th e child leaves hospital the day after operation.

2.9. Chronic specifi c pharyngitis

2.9.1. Tuberculosis of the pharynx


Th e pharynx is not a common site for clinically manifest tuberculosis. It is the site of primary infection almost always in children, resulting in an asymptomatic primary focus usually located in tonsils and adenoids, with accompanying cervical adenopathies. Secondary tuberculosis involves the pharynx, but only in patients with massive, positive and usually cavitating pulmonary lesions. Lupus vulgaris is a low-grade fi bro-sclerotic cutaneous form and has been described in the nasal cavities and in the pharynx.

189

Th e aetiology of tuberculosis is represented by the Mycobacterium tuberculosis agent. Th e resurgence of tuberculosis has paralleled the HIV epidemic and it will inevitably increase in the next years 12.

2.9.1.2. Diagnosis

Th e primary form in children: usually asymptomatic, • adenoid and tonsillar hyperplasia, cervical adenopathiesTh e secondary form: multiple, painful shallow ulcers, • pseudo-tumor formation (tuberculoma)Chest X-rays, microscopic examination of stained smears • for acid-fast bacilli, new ELISA tests to detect specifi c antigens and PCR testing for genetic elements 12

Biopsy, to rule out a malignancy!•

2.9.1.3. Treatment

Treatment is with triple therapy, but drug resistance is an • increasing problem worldwide

2.9.2. Syphilis


Syphilis is an infectious disease caused by Treponema pallidum. Th e infection may present in various forms that have been classically described as primary, secondary, tertiary and congenital syphilis. In primary syphilis the typical lesion is the chancre, which appears after an incubation period of 2-3 weeks. Th e most frequent extragenital sites for the chancre are lips, buccal mucosa, tongue and tonsils. Th e lesion is characterized by a painless ulcer with indurated margins, accompanied by enlarged upper cervical adenopathies22. Secondary form usually occurs several weeks (four to six) after the primary lesion, and is characterized by malaise, headaches, fever, generalized lymphadenopathy, mucocutaneous rash and sore throat. Th e pharyngeal mucosa displays congestion, infl ammation and is covered by typical mucous patches and superfi cial ulcerations. Tertiary syphilis develops 3-10 years after the initial infection. Th e typical lesion is the gumma, located on the hard palate, tonsil or

190

the posterior pharyngeal wall. It is a granulomatous, necrotic painless lesion. In evolution there may be perforation of the soft or hard palate, and considerable destructions of the surrounding structures.

2.9.2.2. Diagnosis

In the primary and secondary forms, spirochetes can be • identifi ed by darkfi eld illumination microscopy in smears taken directly from the lesionSpirochetes can also be identifi ed in biopsy specimens using • silver stains or fl uorescent-labelled antibodies. Th e biopsy of a tertiary lesion is mandatory for the diff erential diagnosis with malignancies and other granulomatous diseases!Serological tests: those used to identify nonspecifi c • antibodies to cardiolipin Venereal Disease Research Laboratory (VDRL), and those to detect specifi c treponemal antibodies (TPI, FTA tests)Two rapid enzyme immune assay techniques, the Enzywell • TP and the Syphilis ICE enzyme immunoassay (EIA), have been recently developed 12

2.9.2.3. Treatment

High-doses of Penicillin• Ceftriaxone and Azithromicin are studied as alternative • antibiotics 23

2.9.3. Other chronic specifi c granulomatous diseases

2.9.3.1. Leprosy

Chronic granulomatous infection caused by • Mycobacterium lepraeIsolated leprosy of the pharynx does not occur; it spreads • to the pharynx from the nasal cavities Diagnosis: biopsy is mandatory• Treatment: multi-drug therapy regimens (see Nose, • section 3.2.4.)

2.9.3.2. Rhinoscleroma

Progressive granulomatous infl ammatory disease, • commencing in the nose, with later involvement of the pharynx, larynx and sometimes trachea and bronchi

191

Th e disease begins in the nose, but may secondarily aff ect • the pharynx: granulomatous lesions and scarringTh e causative agent is the gram-negative bacillus • Klebsiella rhinoscleromatis (Frisch bacillus) (see Nose, section 3.2.3.)

2.9.4. Candidiasis


Candida albicans is found in 15-25% of oral cavity and pharyngeal mucosal surfaces, being considered a normally saprophytic agent22. In order to become pathogenic and symptomatic, some predisposing factors must coexist (Table 1 ).

Local Systemic

Local disease: lichen planus, leukoplakia• Systemic antibiotics may change local oral fl ora to allow • overgrowth of candidaRadiotherapy•

Diabetes mellitus• Lymphoma• Immunosuppressive drugs• AIDS•

Table 1. Predisposing factors for candidiasis

2.9.4.2. Diagnosis

It may be asymptomatic or accompanied by dysphagia, • sore throat, painful swallowing

Figure 13. Oral candidiasis

192

Th e mucosa shows red, irregular and fl at lesions, may be • covered by small white patches, which after removal leave an erythematous lesionDiagnosis is confi rmed by a Gram stain or a culture•

2.9.4.3. Treatment

Candidiasis with local predisposing factors responds well • at topical anti-fungal therapy (nystatin, clotrimazole)When systemic factors are present, systemic anti-fungal • therapy (fl uconazole) may be required

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3. TUMOURS OF THE PHARYNX

3.1. Tumors of the nasopharynx


3.1.1.1. Juvenile nasopharyngeal angiofi broma (JNA)


JNA is an uncommon, benign and highly vascular tumor that arises at the level of the tissues within the sphenopalatine foramen. Rarely, it is found at other sites in the nasal cavity or paranasal sinuses24. It develops almost exclusively in male adolescents. As it grows, the tumor invades the nasal cavities, the rhinopharynx, the paranasal sinuses, pterygopalatine and infratemporal fossae, the orbit and even the skull base25.

3.1.1.1.2. Pathology

JNAs are well-defi ned, circumscribed, lobulated tumors, covered by normal nasopharyngeal mucosa. Th ey are highly vascular and locally destructive, but non-infi ltrating. Th e tumor consists of a network of proliferating and irregular vascular channels, embedded within a fi brous stroma. Because this tumor occurs exclusively in adolescent boys, there has always been speculations that sex-hormone receptors could play a signifi cant role in its development. Recent studies demonstrated that androgen receptors are present in at least 75% of tumors. In contrast, estrogen receptors have not been demonstrated26. Other factors involved: the angiogenic vascular endothelial growth factor (VEGF)27, overexpression of insulin-like growth factor II (IGFII)28.

194

3.1.1.1.3. Symptoms

Recurrent episodes of severe epistaxis in young male • adolescents (the highest incidence is between the age of 13 and 17)Nasal obstruction, mucopurulent rhinorrhea• Hypo/anosmia• Nasal speech (• rhinolalia clausa)


Inspection: oral breathing, protrusion of the eye-ball, • facial deformities, cranial nerve defi citsPosterior rhinoscopy, • endoscopy of the nose and nasopharynxBiopsy should • not be performed, risk of important haemmorhage (!)Ear microscopy: • retraction of the eardrum, middle ear eff usionCT scans, MRI: • evaluation of tumor extentAngio-MRI/digital • substraction angiography: identifi cation of the blood vessels to the tumor and mostly embolization of the aff erent arteries at the same timeOphthalmological and neurological examination•

3.1.1.1.5. Diff erential diagnosis

Adenoids• Craniopharyngioma: benign epithelial tumor, originating • from remnants of epithelium of the hypophyseal duct and/or Rathke’s pouchTh ornwaldt’s cysts (pharyngeal bursitis): benign cystic • formation from remnants of Rathke’s pouchChoanal polyp• Malignant tumors of the nasopharynx•

Figure 14. Juvenile nasopharyngeal angiofi broma (endoscopic view)

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3.1.1.1.6.1. Surgical treatment:

Th e recommended European standard 21 is:Complete resection of the tumor via a transnasal or • transmaxillary approach, or endoscopic access. If necessary, a midfacial degloving is performedPreoperative embolization is appropriate in most cases, • if availablePreoperative external carotid ligation is also • recommended

3.1.1.1.6.2. Conservative treatment:

Radiotherapy• Long-term estrogen therapy•


3.1.2.1. Nasopharyngeal carcinoma (NPC)

3.1.2.1.1. Epidemiology

Incidence (new cases/inhabitants/year): in central • Europe 0.5-1:100,000, in southern Europe 1:100,000, in southern China, South Asia up to 20:100,000 21

Th e highest incidence rate occurs in southern China, • among the Cantonese population of the Guangdong province. In this province, the NPC is referred as the “Guangdong tumor” 29. In Hong Kong, where 98% of the population is Cantonese, the incidence rate for men was once reported as 30:100,000, the highest in the world 30

Emigrants from southern China retain a signifi cantly • higher risk of developing a NPC, although it decreases in subsequent generations 31

NPC shows a predilection for middle-aged patients; • however, it was also observed in children

3.1.2.1.2. Aetiology

Genetic factors: HLA class 1 genotypes (HLA-A, B • and DR)Epstein-Barr virus (EBV) involvement: mainly •

196

in undiff erentiated or nonkeratinizing forms. Th e discovery of EBV receptors at the level of human pharyngeal epithelia fi rmly revealed the close association between EBV and NPC 32. To date, circumstantial evidences has proved that the virus plays a critical role in the pathogenesis of NPC 33

Environmental carcinogens: nitrosamines from salted • fi sh (southern China)

3.1.2.1.3. Pathology

Th e WHO (1978) classifi cation of NPC was revised in 1991. It divides the NPC into two grades: grade 1 (keratinizing squamous cell carcinomas) and grade 2 (nonkeratinizing squamous cell carcinomas and undiff erentiated carcinomas)34. Th e older classifi cation (1978) divided the NPC into three types:

Type I: Squamous cell carcinoma (keratinizing): well-• diff erentiated, moderately diff erentiated and poorly diff erentiatedType II: Nonkeratinizing carcinoma• Type III: Undiff erentiated carcinoma•

3.1.2.1.4. Clinical features

Several diff erent clinical presenting patterns have been described 35:

An upper neck mass (enlarged metastatic laterocervical • lymph nodes) is the commonest complaint at fi rst presentation (50% of the patients)Otological symptoms: unilateral conductive hearing loss • due to the impairment of eustachian tube’s function, secondary to the presence of the nasopharyngeal mass (20% of the patients)Rhinological symptoms: nasal obstruction, blood-stained • nasal discharge, post-nasal drip, epistaxis (mainly in large tumors)Neurological complaints: headaches, cranial nerve • symptoms secondary to invasion of the skull base (foramen lacerum: III, IV, ophthalmic division of V and VI, foramen jugulare: IX, X, XI)

197


Palpation of the neck: to evaluate the metastatic • lymphadenopathies Posterior rhinoscopy• Endoscopy: the site of origin is the Rosenmuller fossa, • behind the eustachian tube’s opening, on the lateral nasopharyngeal wall; this site must be extremely carefully examinedBiopsy is considered the fi rst necessary investigation for • NPC if a suspicious lesion is found!A complete ENT examination should be performed; • care should be taken to look for middle ear eff usions and cranial nerves defi citsImaging: CT, MRI to evaluate the extent of the disease• Serologic tests for EBV: the detection of IgA antibodies • to EBV (anti-VCA, anti-EA titres)36


Curative radiotherapy: NPC is an extremely radiosensitive • tumor and the mainstay of treatment for primary local and regional disease is radiotherapy, almost irrespective of the disease’s stage37

For patients with advanced disease, the addition of • chemotherapy appears to enhance the overall treatment outcomesSurgery only plays a limited role in the management of • NPC: neck dissections for metastatic disease in the neck, if the nasopharynx is free of tumor; salvage surgery in local recurrencesPrognosis: In undiff erentiated carcinoma, the 5-year • survival is about 60% for stages 1 and 2, and about 20% for advanced stages; in keratinizing and non-keratinizing forms, the 5-year survival is 20%21

3.1.2.2. Other nasopharyngeal malignancies

Epithelial tumors: adenocarcinomas, mucoepidermoid • carcinomas, adenoid cystic carcinomasSoft tissue tumors: rhabdomyosarcomas, fi brosarcomas• Malignant lymphomas•

198

3.2. Tumors of the oropharynx


Benign tumors occur mainly in the oral cavity than in the • oropharynxSquamous papillomas are most frequently reported, they • may arise on the tonsil, tonsillar pillars or uvula; treatment consists of surgical removal in local anesthesia Lingual thyroid: mass of ectopic thyroid tissue located at • the level of the base of tongueMinor salivary gland adenomas: soft palate•

Figure 15. Papilloma of the uvula


Th e great majority of oropharyngeal malignant tumors are squamous cell carcinomas (70%)38. Other malignancies include lymphomas, minor salivary gland tumors, sarcomas and metastatic lesions. Lymphomas occur most frequently in the tonsils, where they represent 16% of the tonsilar malignancies. Th ey are almost

199

always non-Hodgkin lymphomas and can occur anywhere at the level of the Waldeyer’s ring.

3.2.2.1 Tonsillar and lateral pharyngeal wall cancers

In 85-90% of the cases we have squamous cell carcinomas. Non-Hodgkin lymphomas are on the second place (formerly known as lymphosarcomas and reticulosarcomas).

3.2.2.1.1. Squamous cell carcinomas

Males over 50 years of age, smokers, heavy alcohol drinkers• Enhanced expression of the human papilloma virus type • 2, 11 and 16 has been reported39

Initial symptoms are often vague and nonspecifi c, leading • to a delay in diagnosis: sore throat, globus sensationsTh e fi rst presenting symptom may be the presence of a • lump in the neckIn advanced disease: dysphagia, odynophagia with • referred ear-pain, fetid breath, haemorrhagesNeck palpation for evaluation of cervical metastatic • adenopathiesCareful palpation of the tonsillar region, the fl oor of the • mouth and the tongueOropharyngoscopy: the tonsillar tumor is usually • ulcerative and infi ltratingBiopsy is mandatory for the diagnosis!• Pan-endoscopy: to eliminate the presence of a possible • synchronous second primary malignancy40

Imaging: CT scans, MRI, to evaluate the extent of the • diseaseTreatment: radiotherapy (in early stages), surgery followed • by radiotherapy (in advanced disease)Prognosis: in early tonsillar cancer, the expected 5-year • survival is 50-90%; in advanced cancer is 20-55%38

3.2.2.1.2. Non-Hodgkin lymphomas

Early symptoms are similar• Th e appearance of the tonsillar tumor is diff erent: the • entire tonsil is hypertrophied, bulky, grossly enlargedFor histologic diagnosis, an adequate amount of tissue is • taken for biopsy

200

Figure 16. Squamous cell carcinoma of the left palatine tonsil

Figure 17. Non-Hodgkin lymphoma of the left palatine tonsil

201

Treatment is non-surgical: chemo/radiotherapy protocols• 5-year survival rates have been reported at 58-77% • 41

3.2.2.2. Base of tongue cancers

Squamous cell carcinoma is the most frequently • encountered histopathological type. Other malignancies involved are non-Hodgkin lymphomas and malignant glandular tumorsConsiderable delay in diagnosis, because of the lack of • specifi city of initial symptoms: sore throat, foreign body sensations, lump in the neckIn advanced disease: lump in the neck, dysphagia, • odynophagia with referred ear-pain, fetid breath, haemorrhagesPalpation of the neck: to evaluate the cervical metastatic • adenopathiesCareful palpation of the tongue base!• Indirect mirror examination, pan-endoscopy, biopsy!• Imaging: CT scans, MRI• Treatment: surgical resection with or without • reconstructive procedures, followed by postoperative radiotherapy; chemoradiotherapy protocols in advanced disease Prognosis: because most of the patients present with • advanced disease, the overall 5-year survival for stages 3 and 4 is around 21% 42; in early stages the expected 5-year survival rate is 45-70% 38

3.2.2.3. Soft palate

Squamous cell carcinomas (75-95%); other malignancies: • minor salivary gland tumorsTreatment: radiotherapy, chemotherapy, surgery• Th e functional outcomes may be critically diff erent, • with signifi cant hyponasality of speech and nasal food regurgitation which may be intolerable to patients

3.2.2.4. Posterior pharyngeal wall

Very rare• Disastrous prognosis• Treatment: radiotherapy, chemotherapy•

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3.3. Tumors of the hypopharynx

3.3.1. Aetiology. Pathology

Nearly all tumors of the hypopharynx are malignant and • virtually all are squamous cell carcinomas Lymphomas, undiff erentiated carcinomas, adenocarcinomas, • benign tumors were reported in less than 3.5% of the cases43

Piriform sinus cancer: more common in males, heavy • smokers, heavy alcohol drinkersPostcricoid cancer: the only head and neck cancer site which • is characterized by a higher incidence in women, classically associated with a history of the Patterson-Kelly-Brown syndrome (glossitis, sideropenic anaemia, dysphagia)

3.3.2. Symptoms

Unilateral sore throat, odynophagia (pain on swallowing), • referred ipsilateral otalgia, dysphagia, weight lossTh ese tumors invade the adjacent larynx, so hoarseness • appears during evolutionA lump in the neck is the presenting symptom in about • one in fi ve patients with hypopharyngeal cancer 43

3.3.3. Diagnosis:

Inspection and palpation: neck palpation will demonstrate • metastatic lymph nodes in 75% patients with piriform sinus cancers and 20% patients with postcricoid cancerIndirect mirror examination (indirect hypopharyngo-• laryngoscopy): allows the evaluation of the larynx invasion, vocal cord mobilityPan-endoscopy (fl exible or rigid): provides the most • accurate examination of the hypopharynx, allows an appropriate evaluation of the tumor’s relationship with the larynx, cervical oesophagus and superiorly with the oropharynxBiopsy!• Imaging: CT scans, MRI•

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3.3.4. Treatment. Prognosis

Surgery (partial hypopharyngectomies in small tumors, • partial hypopharyngectomy associated with total laryngectomy and neck dissection, total pharyngo-laryngectomies associated with reconstructive procedures in advanced tumors)Post-operative radiotherapy, chemotherapy• Prognosis: Patients with small lesions and no lymph node • metastases have a median 5-year survival rate of 70%. Patients with advanced disease have a median survival of 20-30%44

Figure 18. Squamous cell carcinoma of the left piriform sinus (surgical specimen)

204

4. NEUROLOGICAL DISORDERS

OF THE PHARYNX

4.1. Sensory disorders

4.1.1. Anaesthesia/Hyperestesia

Anaesthesia of the pharyngeal mucosa• Hyperestesia of the pharyngeal mucosa is a common • fi nding mainly in acute infl ammatory conditions

4.1.2. Pharyngeal paresthesias

“Lump in the throat” is a common complaint• Middle-aged women with persistent unpleasant • sensations in the throatTh e sensations are augmented by saliva swallowing, but • do not interfere with the swallowing of foodNo other symptoms, no loss of weight• Cancerophobia• A very accurate local examination should be performed • in order to rule out serious diseases (malignancies of the upper aerodigestive tract)!Gastroesophageal refl ux disease (GERD) investigation• Treatment: tranquilisers, anxiolytics, treatment of • GERD

4.1.3. Glossopharyngeal neuralgia

A very rare condition, similar to that of trigeminal • neuralgiaPresentation is with a stabbing pain related to the area of • the palatine tonsil and base of tongueA careful ENT examination must be performed to rule • out local structural causes (malignancies)

205

In some cases, an elongated stiloid process may be the • cause of the symptoms (Eagle’s syndrome); resection of the process via a tonsillar fossa approach relieves the symptomsTreatment is similar to trigeminal neuralgia: • carbamazepine, symptomaticsSome authors described these neuralgias as a vascular • compression syndrome and reported surgical management by intracranial microvascular decompression45

4.2. Motor disorders

Motor disorders of the pharynx usually present as swallowing problems. Almost 75% of oropharyngeal dysphagia has connection with an underlying neurological etiology46. It is extremely important to eliminate a structural cause, therefore an accurate ENT clinical examination and further imaging evaluation are required in all cases. A detailed history provides valuable informations for the diagnosis. Symptoms associated with neurological dysphagia include: drooling, nasal regurgitation, coughing/choking, throat clearing and wet voice 47.

4.2.1. Myogenic disorders

Myotonia: is characterized by continued muscle • contraction after cessation of voluntary eff ortMyasthenia gravis: acquired autoimmune disorder • determined by the presence of IgG autoantibodies against acethylcoline receptors at the level of the neuromuscular junction. Pharyngo-laryngeal involvement is suggested by the presence of laryngeal weakness, dysphonia and dysphagia

4.2.2. Neurogenic disorders

Stroke: probably the most common neurological disorder • aff ecting the pharynx (after a stroke almost 50% of the patients suff er at least temporarily with aspiration and dysphagia)47

Parkinson’s disease•

206

Demyelinating disorders: multiple sclerosis, Guillain-• Barre syndromeSystemic infections: poliomyelitis, diphtheria, herpes • zoster, rabies, botulismPeripheral nerve lesions: the lower cranial nerves may be • aff ected by local disease involving the brainstem, skull base, neck or thorax (nasopharyngeal carcinoma, temporal bone tumours, metastases, jugular bulb thrombosis, glomus tumours, parotid tumors, lymph node metastases) (Table 2)

Syndrome Cranial nerves involved Site of the lesions

Vernet IX, X, XI jugular foramen

Collett-Sicard IX, X, XI, XII posterior laterocondylar space

Villaret IX, X, XI, XII, cervical sympathetic chain (Horner’s) posterior retroparotid space

Tapia X, XII retromastoid space

Table 2. Eponyms for extra-nevraxial cranial nerve palsies

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5. FOREIGN BODIES IN THE PHARYNX

In most of the cases, fi sh bones are involved. Fish bones lodge in the palatine tonsil, the lingual tonsil, or even in the piriform fossae. Other foreign bodies: chicken bones, toy parts, needles, pins, metal wires. In locating a foreign body, patient’s sensations are sometimes a valuable guide 1:

If the patient points to one side of the throat, then the • foreign body is, or has been thereIf the patient points to the midline, then the foreign body • is probably below the level of the pharynx

Diagnosis is based on an accurate history, oropharyngoscopy, indirect laryngoscopy, endoscopy. Sometimes we don’t fi nd any foreign body during pharyngeal examination, although the patient complains of its presence due to the local irritation determined by the passage of the foreign body to the oesophagus. Treatment is removal, using a special forceps. To aid the foreign body removal, topical anaesthesia is needed.

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6. REFERENCES

1. Colman BH. Th e Pharynx. In: Colman BH, ed. Hall & Colman’s Diseases of the Nose, Th roat and Ear, and Head and Neck. Churchill Livingstone 1992; 93-120.

2. Beasley N. Anatomy of the pharynx and oesophagus. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1942-1953.

3. Logemann JA. Mechanisms of normal and abnormal swallowing. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1437-1447.

4. Bonkowsky V, Gerdemann P. Oropharynx and hypopharynx. In: Bonkowsky V, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 425-442.

5. Chevretton EB. Causes of dysphagia. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2025- 2036.

6. Karkos PD, Wilson JA. Globus pharyngeus. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2037-2042.

7. Malcomson KJ. Globus Hystericus Vel Pharyngis. Journal of Laryngology and Otology 1968; 82: 219-230.

8. Moloy PJ, Charter R. Th e globus symptom. Archives of Otolaryngology 1982; 108: 740-744.

9. Th ompson WG, Heaton KW. Heartburn and globus in healthy people. Canadian Medical Association Journal 1982; 126: 46-48.

10. Hill J, Stuart RC, Fung HK, Ng EKW, Cheung FM, Chung SCS. Gastroesophageal refl ux, motility disorders and psychological profi les in the etiology of globus pharyngis. Th e Laryngoscope 1997; 107: 1373-1377.

11. Couch ME, Blaugrund J, Kunar D. History, physical examination and the preoperative evaluation. In: Cummings CW, Robbins TK, eds. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 3-24.

12. Macnamara M. Acute and chronic pharyngeal infection. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1981-2024.

13. Del Mar C. Managing sore throat: a literature review. I: Making the diagnosis. Medical Journal of Australia 1992; 156: 572-575.

14. Arroll B. Antibiotics for upper respiratory tract infections: an overview of Cochrane reviews. Respiratory Medicine 2005; 99: 255-261.

15. Georgalas C, Kanagalingam J, Zainal A, Ahmed H, Singh A, Patel KS. Th e association between periodontal disease and peritonsillar infection: a prospective study. Otolaryngology and Head and Neck Surgery 2002; 126: 91-94.

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16. Schraff S, McGinn JD, Derkay CS. Peritonsillar abscess in children: a 10-year review of diagnosis and management. International Journal of Pediatric Otorhinolaryngology 2001; 57: 213-218.

17. Papalia E, Rena O, Oliaro A, Cavallo A, Giobbe R, Casadio C. Descending necrotizing mediastinitis: surgical management. European Journal of Cardio-thoracic Surgery 2001; 20: 739-742.

18. Sethi DS, Stanley RE. Deep neck abscesses - changing trends. Journal of Laryngology and Otology 1994; 108: 138-143.

19. Ragnaud JM, Morlat P, Gin H et al. Clinical, biological and developmental aspects of cytomegalovirus infection in immunocompetent patients. La Revue de Medicine Interne 1994; 15: 13-18.

20. Fierstein JT, Th awley SE. Otolaryngologic manifestations of acute leukemia and lymphoma. Th e Southern Medical Journal 1978; 71: 277-280.

21. Ganzer U, Arnold A. Diseases of the nasopharynx. In: Ganzer U, Arnold A, eds. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 319-329.

22. Bonkowsky V, Gerdemann P. Oral cavity. In: Bonkowsky V, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 405-424.

23. Augenbraun MH. Treatment of syphilis 2001: non-pregnant adults. Clinical Infectious Disease 2002; 35: 5187-5190.

24. Windfuhr JP, Remmert S. Extranasopharyngeal angiofi bromas: etiology, incidence and management. Acta Oto-Laryngologica 2004; 124: 880-889.

25. Gleeson M. Juvenile angiofi broma. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2437-2444.

26. Hwang HC, Mills SE, Patterson K, Gown AM. Expression of androgen receptors in nasopharyngeal angiofi broma: an immunohistochemical study of 24 cases. Modern Pathology 1998; 11: 1122-1126.

27. Schiff M, Gonzalez AM, Ong M, Baird A. Juvenile nasopharyngeal angiofi bromas contain an angiogenic growth factor: basic FGF. Th e Laryngoscope 1992; 102: 940-945.

28. Coutinho-Camillo CM, Brentani MM, Butugan O, Torloni H, Nagai MA. Relaxation of imprinting of IGFII gene in juvenile nasopharyngeal angiofi bromas. Diagnostic Molecular Pathology 2003; 12: 57-62.

29. Ho JH. Epidemiology of nasopharyngeal carcinoma. Gann Monograph on Cancer Research 1976; 18: 49-61.

30. Ho JH. Incidence of nasopharyngeal carcinoma in Hong Kong. UICC Bulletin 1971; 9: 5-10.

31. Buell P. Th e eff ect of migration on the risk of nasopharyngeal cancer among Chinese. Cancer Research 1974; 34: 1189-1191.

32. Young LS, Clark D, Sixbey JV, Richinson AB. Epstein-Barr virus receptors on human pharyngeal epithelium. Lancet 1986; 1: 240-242.

33. Niedobitek G. Epstein-Barr virus infection in the pathogenesis of nasopharyngeal carcinoma. Molecular Pathology 2000; 53: 248-252.

34. Shanmugaratnam K, Sobin L. Histological typing of tumors of the upper respiratory tract and ear. WHO International Histological Classifi cation of Tumors. 1991; 7-9, 32-33.

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35. Skinner DW, van Hasselt CA, Tsao SY. Nasopharyngeal carcinoma: a study of the modes presentation. Annals of Otology, Rhinology and Laryngology 1991; 100: 544-551.

36. Tam JS, Murray HGS. Nasopharyngeal carcinoma and Epstein-Barr virus-associated serological markers. Ear Nose and Th roat Journal 1990; 69: 261-266.

37. Woo JKS, van Hasselt CA. Nasopharyngeal carcinoma. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2445-2474.

38. Bradley PJ. Oropharyngeal tumours. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2577-2597.

39. Hammarstedt L, Lindquist D, Dahlstrand H et al. Human papillomavirus as a risk factor for the increase in incidence of tonsillar cancer. Int J Cancer 2006; 119: 2620-2623.

40. Braakhuis BJ, Tabor MP, Leemans CR, van der Waal I, Snow GB, Brakenhoff RH. Second primary tumors and fi eld cancerization in oral and oropharyngeal cancer: molecular techniques provide new insights and defi nitions. Head and Neck 2002; 24: 198-206.

41. Gassner HG, Sabri AN, Olsen KD. Oropharyngeal malignancy. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1717-1757.

42. Sessions DG, Lenox J, Spector GJ, Chao C, Chaudry OA. Analysis of treatment results for base of tongue cancer. Th e Laryngoscope 2003; 113: 1252-1261.

43. Jones AS. Tumours of the hypopharynx and oesophagus. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2633-2662.

44. Blomquist E. Head and neck tumours. In: Anniko M, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 671-685.

45. Resnick DK, Jannetta PJ, Bissonnette D, Jho HD, Lanzino G. Microvascular decompression for glossopharyngeal neuralgia. Neurosurgery 1995; 36: 64-69.

46. Trate D, Parkman H, Fisher R. Dysphagia: Evaluation, diagnosis and treatment. Primary Care 1996; 23: 417-432.

47. Sanderson RJ, Tierney P, Adamson R. Neurological disease of the pharynx. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2074-2083.

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IV. LARYNX

1. LARYNX BASICS


Th e larynx is part of the respiratory system and is located at the upper level of the aerodigestive tract. Th e laryngeal cavity extends from the level of the epiglottis’ tip to the inferior margin of the cricoid cartilage, from where it is continued with the trachea.

1.1.1. Skeletal framework

Th e larynx consists of a cartilaginous framework bound together by ligaments and fi brous membranes, and moved by a number of muscles. It is attached superiorly to the U-shaped hyoid bone by the thyrohyoid membrane and ligaments and thyrohyoid muscles. Th e cartilages of the larynx are paired and unpaired.

1.1.1.1. Unpaired cartilages

Th e epiglottis is a leaf-shaped fi broelastic cartilage which • is attached superiorly to the base of tongue by the glosso-epiglottic folds, and inferiorly to the inner aspect of the thyroid cartilage, immediately above the anterior insertion of the vocal cords. Laterally, its sides are connected to the arytenoids by the aryepiglottic folds

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Th e thyroid cartilage makes the neck’s midline • prominence known as the “Adam’s apple”. It consists of two quadrilateral wings (or alae), joined together in the midline anteriorly. Th e thyroid notch, located at the junction of the alae on the superior margin, can be readily palpatedTh e cricoid cartilage is located below the thyroid. It is a • ring-shaped structure which articulates with the thyroid and the arytenoids by synovial joints. Its anterior part (cricoid arch) is connected to the inferior border of the thyroid cartilage by the cricothyroid membrane. At the level of this membrane, a coniotomy (cricothyroidotomy, inferior laryngotomy) is performed in cases of acute airway obstruction

1.1.1.2. Paired cartilages

Th e arytenoid cartilages are located on the superior • border of the posterior cricoid plate. Th ey are pyramid-shaped cartilages that can rotate and slide on the cricoid (crico-arytenoid joint) and thus play a very important role in the movement of the vocal cords. Th ey have an anterior projection, known as the vocal process, and a posterolateral one, known as the muscular process. Th e

Figure 1. Larynx - anterior view

1 - Epiglottis; 2 - Hyoid bone; 3 - Th yrohyoid membrane; 4 - Th yroid cartilage; 5 - Cricothyroid muscle; 6 - Cricothyroid membrane; 7 - Cricoid cartilage; 8 - Tracheal rings

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vocal process gives insertion to the true vocal cords. Th e muscular process form the main attachment for those muscles activating the vocal cords in phonation and respirationTh e aryepiglottic folds connect the arytenoids with the • epiglottis and forms the upper edge of the laryngeal inlet

1.1.2. The endolarynx

Th e endolarynx is subdivided into the supraglottis, the glottis and the subglottis.

1.1.2.1. Supraglottis

Th e clinical term supraglottis refers to that part of the endolarynx which lies above the horizontal plane of the true vocal cords. It consists of the laryngeal inlet, or aditus (the aperture between the larynx and the pharynx), the laryngeal ventricle of

Figure 2. Endolarynx - frontal view

1 - Epiglottis; 2 - Hyoid bone; 3 - False vocal cord (ventricular band); 4 - Laryngeal ventricle (Morgagni); 5 - True vocal cord; 6 - Vocal muscle; 7 - Cricoid cartilage; 8 - Tracheal ring

Figure 3. Laryngeal levels

I - Supraglottis; II - Glottis; III - Subglottis

1 - Epiglottis; 2 - Pre-epiglottic space; 3 - Hyoid bone; 4 - Th yrohyoid membrane; 5 - Th yroid cartilage; 6 - Arytenoid cartilage; 7 - Cricothyroid membrane; 8 - Cricoid cartilage

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Morgagni (the space between the true and the false vocal cords), the false vocal cords (the ventricular bands), the laryngeal surface of the epiglottis, the arytenoids and the medial aspects of the aryepiglottic folds.

1.1.2.2. Glottis

Th e glottis include the two paired true vocal cords, the anterior and the posterior comissure. Rima glottidis is the horizontal space between the left and the right vocal cord, triangular-shaped, which narrows during phonation (adduction) and widens during breathing (abduction). Rima glottidis is divided into two parts: the anterior two-thirds (the intermembranous part) which participates actively in the phonation, and the posterior one-third (the intercartilaginous part) between the vocal arytenoid processes. Th e true cords (mucosa, vocal ligament, vocal thyroarytenoid muscle), pearly white-coloured, are attached anteriorly in the midline to the posterior surface of the thyroid cartilage, and posteriorly to the vocal process of the arytenoids. Th eir superior aspect corresponds to the fl oor of the Morgagni’s laryngeal ventricle. Th e potential space between the mucosa and the underlying vocal ligament is known as the Reinke’s space. Accumulation of fl uid within this space may lead to abnormal swelling of the true vocal cords (Reinke’s edema).

1.1.2.3. Subglottis

Th e subglottis extends from 1 cm below the free margin of the true vocal cords to the inferior border of the cricoid cartilage.

1.1.3. Muscles of the larynx

Th e muscles of the larynx are divided into two main categories: the extrinsic and the intrinsic muscles. Th e extrinsic muscles connect the larynx to adjacent structures and are responsible for its vertical movements during phonation and swallowing (infrahyoid strap muscles and the inferior pharyngeal constrictor. Th e intrinsic muscles are responsible for altering the length, tension, shape and spatial position of the vocal cords. Th ese

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muscles may be divided in three groups, according to their main actions1:

Vocal cords adductors: thyroarytenoid, lateral • cricoarytenoid and interarytenoid musclesVocal cords abductor: posterior cricoarytenoid muscle• Tensor of the vocal cord: cricothyroid muscle•

1.1.4. Vascular supply and lymphatic drainage

Th e blood supply is derived mainly from the superior laryngeal artery, branch of the superior thyroid pedicle (fi rst collateral branch of the external carotid) and inferior laryngeal arteries, which come from the thyrocervical trunk, branch of the subclavian artery. Th e venous drainage passes to the internal jugular veins. Th e lymphatic drainage of the supraglottis and even the subglottis is much more abundant than the glottis, which has implications for the management of cancer of these sites. Th e incidence of metastatic lymph nodes is higher in supraglottic than in glottic cancer, thus making the prognosis worse.

1.1.5. Nerve supply

Th e nerve supply of the larynx is of great practical importance and comprises the superior and inferior recurrent laryngeal nerves, both being branches of the vagus nerve. Th e superior laryngeal nerve takes origin from the inferior ganglion of the vagus nerve and divides into the internal and external laryngeal nerves, supplying sensitive innervation to the supraglottis and motor innervation to the cricothyroid muscle. Th e recurrent laryngeal nerve is also a branch of the vagus nerve, that has a diff erent course on each side:

On the left side it passes under the aortic arch and ascends • through the mediastinum in the tracheo-oesophageal groove to reach the neck, entering the larynx underneath the inferior constrictor muscleOn the right side it does not descend into the thorax•

Th e recurrent laryngeal nerve is motor to all muscles of the larynx, apart from the cricothyroid.

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1.2. Phisiology

1.2.1. Protection of the airway

Th e most important laryngeal function during deglutition is to prevent food and liquid entering the airway. Th is is performed by means of the sphincteric (closed valve) action of the aryepiglottic folds and the true and false vocal cords, which occurs simultaneously with laryngeal’s elevation under the base of tongue. Epiglottis also protects the airway by covering the laryngeal inlet. Rapid laryngeal elevation, which occurs during the second pharyngeal phase of deglutition, appears to be essential for normal swallowing2. Other important protective refl exes are the coughing refl ex and the eff ort laryngeal closure to increase intrathoracic pressure (Valsalva manoeuvre). Th e last one is essential during diff erent physiologic conditions: defecation, urination, weight lifting 1.

1.2.2. Respiration

During normal breathing at rest, the glottis is open (abduction) and the air passes in and out without an active participation of the larynx in the process itself.

Figure 4. Innervation of the larynx

1 - Hyoid bone; 2 - Internal carotid artery; 3 - External carotid artery; 4 - Right vagus nerve (X); 5 - Common carotid artery; 6 - Th yroid cartilage; 7 - Th yroid gland; 8 - Right recurrent laryngeal nerve; 9 - Trachea; 10 - Right subclavian artery; 11 - Aorta; 12 - Left recurrent laryngeal nerve; 13 - Left subclavian artery; 14 - Left vagus nerve (X); 15 - External branch of the superior laryngeal nerve; 16 - Internal branch of the superior laryngeal nerve

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During high-volume breathing, the glottis performs an active abduction and is also tensed by the contraction of the posterior cricoarytenoid and cricothyroid muscles. Th e rima glottidis is considerably widened, so the resistance to the airfl ow is decresed1.

1.2.3. Phonation

Th e vocal folds, vibrated by the pulmonary air stream, are the source of the voice. Each vibratory cycle of the vocal folds consists of three phases: adduction, aerodynamic separation and recoil. Each cycle is the manifestation of a mucosal wave travelling from the inferior to the superior aspect of each vocal fold (the cover/body theory)2. Th e vocal folds have to be structurally and functionally symmetrical, at the same level and close rapidly in order that a clear vocal note can be initiated and maintained 3. Insuffi cient adduction (glottic insuffi ciency) leads to air wastage and a subsequent breathy voice.

1.3. Symptoms of laryngeal diseases

1.3.1. Dysphonia

Dysphonia may be defi ned as any impairment of the voice or diffi culty in speaking. Hoarseness is characterized by a perceived rough, harsh or breathy quality of the voice. A disordered voice may have one or more of these following features4:

It is not audible, clear or stable• It is not appropriate for the age and gender of the patient• It fatigues easily• It is associated with discomfort or pain during phonation•

1.3.2. Upper airway obstruction

Th e main symptoms of upper airway obstruction are:Dyspnea, or diffi culty in breathing, is the cardinal • symptom of upper airway obstruction. It is accompanied by inspiratory stridor, a high-pitched audible sound resulting

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from the turbulent air passage through a narrowed larynx or trachea. Expiratory stridor is heard in association with obstruction of the lower intrathoracic airwayHoarseness is the eff ect of abnormal vibration of the • vocal foldsSuprasternal retraction is secondary to the maximal • respiratory eff ort performed during inspirationRestlessness may be the result of anxiety or hypoxia• Other symptoms and signs may be associated: drooling, • bleeding, subcutaneous emphysema

1.3.3. Other laryngeal symptoms

Laryngeal pain• Irritative cough•



Inspection may reveal important signs of acute upper airway obstruction: stridor, suprasternal and supraclavicular inspiratory retraction, associated neck masses. Palpation of the anatomical landmarks of the larynx is followed by the evaluation of its passive mobility, by moving it laterally, over the anterior vertebral bodies, producing the normal laryngeal crepitus. Palpation during swallowing evaluates the active mobility (elevation of laryngeal structures in deglutition). Finally, palpation of the entire neck must be performed, for an accurate assessment of the cervical lymph nodes and the thyroid gland lobes.

1.4.2. Laryngoscopy

1.4.2.1. Mirror examination (Indirect laryngoscopy)

Mirror examination is an indirect laryngoscopy performed as an offi ce examination. Since its presentation by Manuel Garcia in 1855, this procedure has changed little5

Instruments:Light source•

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Head mirror• Headlight• Laryngeal mirrors• Spirit lamp•

Th e examiner is in front of the seated patient, light beam is focused on the oral cavity. Previously, the laryngeal mirror is warmed with the spirit lamp, to avoid blurring. Th e temperature of the mirror is checked on the examiner’s back of the hand. Th e patient is asked to lean slightly forward at the waist and to open the mouth. Th e tip of the tongue is grasped by the examiner using a gauze, and the mirror is gently introduced through the oral cavity, with the back of the mirror used to elevate the uvula. An indirect image of the endolarynx, superior aspects of the pyriform fossae and tongue base can be seen. Th e patient is asked to say “eeeeeee”, followed by a

Figure 5. Instruments for indirect laryngoscopy

Figure 6. Endolarynx - superior view

1 - Glossoepiglottic fold; 2 - Epiglottis; 3 - Vallecula; 4 - Pharyngoepiglottic fold; 5 - Aryepiglottic fold; 6 - Pyriform fossa (sinus); 7 - Arytenoid cartilage; 8 - True vocal cord; 9 - False vocal cord (ventricular band)

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deep breath, in order to evaluate the vocal folds mobility in phonation and respiration. Touching the posterior aspects of the pharynx with the mirror causes gagging refl exes, which may compromise the examination. Topical anaesthetics may be applied.

1.4.2.2. Endoscopic examination

Indirect laryngoscopy using rigid or fl exible endoscopes is nowadays the routine gold-standard method for an accurate visualisation of the endolarynx and the hypopharynx. Instruments:

Light source• Fiber optic cable• Rigid Hopkins laryngeal endoscopes with 90º and 70º • angled lensesFlexible fi ber optic endoscopes (naso-pharyngo-• laryngoscopes)Topical anaesthetics and nasal decongestants• Photo and video equipment, if available•

Th e technique of transorally rigid endoscopy is quite similar to that of mirror examination. Although is easier to perform, it still requires patient cooperation. Rigid endoscopy supplies the best quality images for documentation purposes.

Figure 7. Endoscopy of the larynx

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Flexible endoscopy off ers the advantage of being feasible in non-compliant patients such as children, seriously ill, demented or psychiatric patients. Also, it can be used in patients with gagging refl exes, who are not capable to tolerate the rigid technique. After topical decongestants and anaesthetics are applied, the endoscope is introduced through the nasal cavity into the rhinopharynx and then is guided downwards, over the posterior pharyngeal wall, to the laryngeal inlet.

1.4.2.3. Direct laryngoscopy

Direct laryngoscopy is not an offi ce examination. Th is procedure is performed in the operating theater, with patient under general anesthesia in most cases. Th e main indications6 are:

Precise location and diagnosis of laryngeal lesions• Biopsy of laryngeal lesions• Removal of mass lesions by standard microsurgical • techniques or by the use of laserAs an adjunct to other therapeutic procedures•

Th e patient is placed in supine position, with head extension and neck fl exed, eyes covered and dental protection. Th e rigid scope is passed transorally, in order to visualize the tongue base

Figure 8. Direct laryngoscopy

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and the tip of the epiglottis. Th e epiglottis is then elevated and the endolarynx is properly visualized. Suspension of the laryngoscope allows the surgeon to use both hands during procedures. For an accurate assessment, microscopes or rigid Hopkins endoscopes may be additionally used.

1.4.3. Voice evaluation

1.4.3.1. Videolaryngostroboscopy

Videolaryngostroboscopy is the main clinical tool in diagnosing the voice disorders; it may be used to assess the quality of vocal folds vibrations and to evaluate the treatment eff ectiveness. Stroboscopy gives additional information about the vibratory pattern of the vocal folds mucosa and improves the accuracy of diagnosis. It has led to changes in diagnosis in nearly 30 percent of the cases, when compared to examination with continuous light alone7.

1.4.3.2. Other methods

Th e protocol recommended by the European Laryngological Society for functional assessment of voice disorders includes, besides the gold-standard stroboscopic examination, the following investigations8:

Perceptual analysis of voice by an expert/trained listener• Acoustic objective measurements • Aerodynamic objective analysis of voice production: • airfl ow, air pressureSubjective rating by patient•

Other adjuvant procedures: laryngeal electromyography, oesophageal and pharyngeal 24 hours pH monitoring.


Imaging techniques: neck ultrasound, conventional • radiography, CT scans, MRI, conventional swallows with barium or water-soluble contrast mediaBiopsy• Bacteriological examinations•

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2. INFLAMMATORY DISEASES

2.1. Acute laryngitis


Acute laryngitis is a common infl ammatory condition of the laryngeal mucosa. In most of the cases the aetiology is viral, being part of an upper respiratory tract infection. Bacterial superinfection may occur.

2.1.2. Symptoms

Hoarseness, which may progress to aphonia• Dry, irritative cough (the cough becomes productive • when an infection of the lower airways is present)Th roat discomfort, even pain, due to irritation by • coughingFever, • malaiseSymptoms of associated rhinitis and/or pharyngitis •

2.1.3. Diagnosis

History• Local examination: congestion/edema of the entire • laryngeal mucosa, the vocal cords loose their normal light-coloured appearance; mucopurulent secretions are signs of bacterial superinfectionA complete ENT examination should be performed•

2.1.4. Treatment

Acute viral laryngitis is usually a self-limiting infection • that resolves within one weekVocal rest, avoidance of irritants, adequate hydration• Symptomatics: analgetics, antipyretics, antitussive agents/• expectorants

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Antibiotics should be reserved for bacterial superinfections, • persistent laryngeal infl ammation, or in specifi c patients who rely on their voice, professionally9

2.2. Acute edematous subglottic laryngitis


Th is is a particular form of acute laryngitis, aff ecting young children aged 6 months to three years, in which the edema of the narrow subglottic region leads to early respiratory distress. Th e aetiology is viral10: myxoviruses, para-infl uenza viruses 1 and 3, rhinoviruses, adenoviruses, echoviruses. Bacterial involvement is rare.

2.2.2. Diagnosis

Diagnosis is clinical and is based on:History: sometimes the initial context of febrile • rhinopharyngitis is missingDyspnea often occurs during night: inspiratory • bradypneaStridor• Inspiratory retraction of the suprasternal and even • supraclavicular soft tissues of the neckBarking cough• Quite normal voice, normal swallowing• Endoscopic examination, if performed, shows a normal • epiglottis, infl amed vocal folds and edema of the subglottic region

2.2.3. Treatment

Treatment should be started as soon as possible:Hospitalization is recommended• Oxygen, steroids and nebulized epinephrine should be • administered9

Wide-spectrum antibiotics to prevent bacterial • superinfectionSometimes intubation or even tracheostomy are required•

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2.3. Acute epiglottitis


Epiglottitis, or supraglottitis, is an acute infection of the supraglottis that involves primarily the epiglottis. It aff ects both children (mainly between 3 and 6 years old) and adults. Th e main diff erence in children, compared to adults, is that acute epiglottitis progresses more rapidly to severe upper airway obstruction. Aetiology is bacterial, Haemophilus infl uenzae type B being involved in the majority of cases. Th at’s why a signifi cant decrease in children incidence has been noticed since the introduction of vaccination against Haemophilus (Hib vaccine) in 1985, to prevent childhood meningitis11.

2.3.2. Diagnosis

In children: a rapid evolution to acute upper airway • obstruction is characteristic, accompanied by fever, drooling (inability to swallow own saliva because of severe pain), a muffl ed voice, anxietyIn adults: an upper airway obstruction may not be present, • main symptoms being odynophagia, dysphagia, drooling, feverLaryngeal examination (fl exible nasendoscopy in • children, mirror examination and/or rigid endoscopy in adults) reveals a cherry-red swollen epiglottis, like a “thumb sign”10

2.3.3. Treatment

Hospitalization and airway monitoring• Intravenous antibiotics (second and third generation • cephalosporins, active against Haemophilus)Humidifi ed oxygen, steroids, analgetics and antipyretics• Intubation or tracheostomy may be required•

2.4. Laryngeal diphtheria

Although considered an eradicated disease, after mass immunization was applied, isolated cases of diphtheria may still occur nowadays.

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Th e disease is caused by Corynebacterium diphtheriae (the Klebs-Loeffl er bacillus), a facultative anaerobic gram-positive agent, which is spread by air-borne droplets. Th e usual site of infection is the tonsil and fauces, but it can also occur within the nasal cavities or spread into the larynx. Th e bacillus remains at the level of the pharynx, causing the local typical manifestations of the disease. In the meantime, it produces a strong neurotropic exotoxin, which is spread into the organism, causing serious systemic manifestations (cardiac and neurological complications), which may be fatal.

2.4.2. Diagnosis

Symptoms: severe sore throat, • malaise, fever, nasal discharge; extension of the pseudo-membranes within the larynx causes an acute upper airway obstructionPalpation of the neck: multiple cervical adenopathies• Local examination: characteristic pearly, white-greyish, • adherent pseudomembranes in the pharynx, nasal cavities and endolarynxTh roat swab cultures•

2.4.2. Treatment

Early administration of antitoxin is mandatory, based • only on clinical suspicion, without confi rmation of the result of the throat culture!Antibiotics should be also administered: Penicillin G is • requiredTh e patients need to be isolated and reported• Acute airway obstruction requires intubation and/or • tracheostomy

2.5. Acute laryngeal edema

2.5.1. Allergy

Th e vasodilation and increase in capillary permeability caused by abrupt histamine release (type I anaphylactic reaction) causes an acute edematous infl ammation of the laryngeal mucosa.

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Th e most frequently involved factors are10:Exposure to inhaled allergens• Foodstuff s• Medications: angiotensin conversion enzyme inhibitors, • non-steroidal antiinfl ammatory drugs (NSAIDs), PenicillinInsect bites and stings•

2.5.2. Angioneurotic edema (Quincke’s edema)

Th is condition corresponds to a sudden swelling of the face and neck, which may lead to severe upper airway obstruction. A hereditary form exists, caused by a genetic defi cit of C1-esterase inhibitor.

2.5.3. Management

Oxygen, epinephrine, steroids and antihistamines• Androgenic derivatives (Danazol) augment the levels • of C1-esterase inhibitor, and may be considered as a prophylactic treatment in angioneurotic edema10

Sometimes intubation and/or tracheostomy are required•

2.6. Chronic nonspecifi c laryngitis


Chronic nonspecifi c laryngitis is defi ned as a chronic infl ammation of laryngeal structures, most commonly the laryngeal mucosa. Th e most important aetiological factors involved are:

Tobacco smoke• Voice abuse• Gastroesophageal refl ux disease (GERD)• Chronic suppurative infections of the upper respiratory • tractChronic exposure to irritating fumes•

Classically, distinction is made between the “red” types, where infl ammatory phenomena predominate, and the “white” types, otherwise known as keratosis10.

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2.6.2. Symptoms

Progressive hoarseness is the cardinal symptom• Voice is usually worst in the morning (the mucus present • in the larynx has dried during the night), becomes better after throat clearing, and deteriorates again as the day progressesForeign body sensations, persistent cough, throat • clearing

2.6.3. Diagnosis

Local examination: mirror examination, fl exible • nasendoscopy, rigid endoscopy with or without stroboscopyTh e “red” types: mucosal erythema, mild diff use edema • and mucosal swelling, stasis of secretions, granulationsTh e “white” types: leukoplakia (fl at lesion, pearly greyish-• coloured, single or multiple, slightly vague contours, localized on the vocal folds), white pachydermia (the lesion has a warty tumor-like appearance, clearly defi ned margins, irregular surface); in both forms the white-greyish color is due to hyperkeratinizationParticular forms: posterior laryngitis (red infl ammatory • lesions on the posterior aspects of the endolarynx; GERD plays an important role in aetiology), Chevalier Jackson’s contact ulcers (granulative and ulcerative lesions on both arytenoids)If there is any suspicion with regard to malignancy • (mainly in keratotic lesions), direct microlaryngoscopy with biopsy is mandatory12

Keratotic lesions are considered to be pre-malignant. • Table 1 shows the most recognized classifi cation of chronic laryngitis epithelial dysplasia (an alteration of cellular maturation) based only on histological criteria10

Grade

Grade I Hyperplasia and/or keratosis with or without mild dysplasia

Grade II Moderate dysplasia

Grade III Severe dysplasia, carcinoma in situ without infi ltration of the basement membrane

Table 1 Histological grading in chronic laryngitis

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2.6.4. Treatment


Smoking cessation, lifestyle modifi cations• Empirical treatment with proton pump inhibitors for the • GERD is advised13

Voice therapy•


All degrees of epithelial hyperplastic/keratotic lesions • should be treated by microlaryngoscopic removal and histopathological assessment of the specimenRegular follow-up is necessary (pre-malignant lesions)•

2.7. Chronic specifi c laryngitis

2.7.1. Chronic infectious granulomatous diseases

2.7.1.1. Tuberculosis

Laryngeal tuberculosis is almost always secondary to pulmonary tuberculosis. Symptoms are nonspecifi c: dysphonia, pain on speaking and swallowing, referred otalgia. Local examination reveals a diff usely erythematous and edematous larynx, predominantly involving the posterior one-third of the glottis. Granulation tissue is also present nearby the arytenoids. Infected sputum causes, later in evolution, ulcerative lesions mainly in the inter-arytenoid space, the arytenoids and ary-epiglottic folds. Th is clinical picture may resemble a malignant tumor! An accurate diagnosis requires a biopsy from the laryngeal lesions. Histopathological assessment reveals the typical granulomas with caseating necrotic center, Langhans giant cells and acid-fast bacilli. Treatment is primarily focused on the disease in the lung: rest and tuberculostatic drug regimens. Laryngeal lesions may heal leaving scars and sometimes cicatricial stenosis14.

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2.7.1.2. Syphilis

Syphilis is an infectious disease caused by Treponema pallidum. Th e infection may present in various forms that have been classically described as primary, secondary, tertiary and congenital syphilis. Laryngeal involvement is rare, supraglottis is primarily aff ected, the most common form is gummatous laryngitis (tertiary syphilis), which mimics a laryngeal malignancy and needs biopsy for diagnosis! Additional serological testing must be performed. Treatment is with high-doses penicilin.

2.7.1.3. Leprosy

Leprosy (Hansen’s disease) is a chronic granulomatous infection caused by Mycobacterium leprae. Laryngeal involvement is rare, supraglottis is primarily aff ected (edematous and nodular lesions with ulcerations), biopsy is mandatory for diagnosis to rule out malignancy and other granulomatous diseases. Treatment: multi-drug therapy regimens (see Nose, section 3.2.4.)

2.7.1.4. Rhinoscleroma

Rhinoscleroma is primarily a chronic granulomatous infection of the nasal cavities. In evolution, lesions may progress downwards, with subsequent pharyngeal and laryngeal involvement (glottis and subglottis). Th e causative agent is the gram-negative bacillus Klebsiella rhinoscleromatis (Frisch bacillus) (see Nose, section 3.2.3.). Histopathological examination of the biopsy specimens reveals the typical Mikulicz cells (foamy vacuolated histiocytes) and Russell bodies. Bacteriological examinations are also required. Treatment is with aminoglicosides, cephalosporins or tetracycline15.

2.7.1.5. Candidiasis

Laryngeal candidiasis (opportunistic infection caused by the oropharyngeal Candida albicans) may occur in immunocompromised patients (chemotherapy, AIDS), prolonged inhaled or systemic corticosteroid use, long-term wide-spectrum antibiotics, radiotherapy for head and neck cancers13.

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Local clinical appearance: diff use erythema of the mucosa, which is covered by irregular, friable, superfi cial, white exudates. In immunocompromised hosts, lesions tend to be more severe and may extend into the oesophagus. Treatment is with nystatin, fl uconazole. In severe forms, amphotericin B may be required16.

2.7.1.6. Other fungal granulomatous infections

Histoplasmosis (• Histoplasma capsulatum)Blastomycosis (• Blastomyces dermatididis)

2.7.1.7. Laryngeal actinomycosis

Actinomycosis is a chronic suppurative granulomatous infection, caused by the anaerobic bacteria Actinomycoses bovis and Actinomycoses Israeli. Th e larynx is aff ected following involvement of the neck’s soft tissues and adenopathies. Diagnosis is performed by identifi cation of the typical sulphur granules in the biopsy specimen and by bacteriological examinations. Treatment requires penicillin or tetracycline17.

2.7.2. Laryngeal involvement in systemic diseases

2.7.2.1. Wegener’s granulomatosis

Wegener’s granulomatosis is a systemic disease with involvement of the upper and lower respiratory tract and the kidneys, characterized by the presence of necrotizing noncaseating granulomas and vasculitis. Larynx is aff ected in up to 25% of the case (local infl ammatory reactions, granulomatous ulcers located mainly in the subglottic region)13. Diagnosis requires a biopsy specimen and the specifi c anticytoplasmic autoantibody C-ANCA test. Treatment is with corticosteroids or cyclophosphamide.

2.7.2.2. Sarcoidosis

Sarcoidosis is a systemic disease of unknown aetiology, characterized by the presence of non-caseating epitheloid granulomas. It may aff ect any part of the body, but most frequently involves the lymph nodes, the skin, the lungs, the eyes and the liver.

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Laryngeal involvement is reported in less than 5% of all cases, with clinical appearances quite similar to tuberculosis and fungal infections, lesions being located primarily in the supraglottis13. Diagnosis requires a biopsy, chest X-rays, CT Scans, Kveim intradermoreaction Treatment: securing a safe airway, systemic corticosteroids.

2.7.2.3. Amyloidosis

Primary or secondary systemic amyloidosis is characterized by diff use submucosal or small subepithelial masses (deposits of amyloid, with a high fl uid content). In laryngeal involvement, patients’ main complaint is dysphonia, due to the presence of these submucosal deposits in the various subsites of the larynx, and their subsequent eff ect on the vocal folds mobility. Diagnosis is confi rmed by biopsy (the affi nity of the amyloid for Congo Red). Treatment requires microsurgical removal of the deposits. Th e use of CO

2 laser is benefi cial.

2.8. Pseudo-myxomatous laryngitis (Reinke’s edema)


Pseudo-myxomatous laryngitis (Reinke’s edema, smokers’ polyps) is a chronic condition characterized by fl uid accumulation in the subepithelial space of the vocal fold mucosa (Reinke’s space). Th is pathological entity is the result of a chronic irritation of the mucosa due to heavy smoking and voice abuse. It is associated typically with middle-aged women, who have been long-term smokers.

2.8.2. Diagnosis

Dysphonia is the main symptom (low-pitched voice, • often in the masculine range when the condition is seen in female patients)A female patient may complain of being called “sir” on the • phone, or may have problems with increasing hoarseness during the day18

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Local examination: pale, watery bags of fl uid attached • to the superior aspect and margins of the vocal folds; an up-and-down motion of the polypoid edematous tissue is often noticed with respiration

2.8.3. Treatment

Quit smoking!• Microlaryngoscopic surgery procedures: the mucosa is • incised parallel to the vocal ligament, fl uid is suctioned and the redundant mucosa is carefully removed (Gould-Hirano technique)10

Speech therapy•

2.9. Vocal fold polyp


Vocal fold polyp is a benign unilateral lesion, located mainly at the level of the free margin of the vocal folds or the anterior comissure. It is more common in men, particularly men who engage in intermittent severe voice abuse, or who work in noisy environments. Sometimes, a history of aspirin or other anticoagulant use is noticed18. Polyps are the result of a violent voice abuse that leads to capillary ruptures in the submucosa, with subsequent localized haemorrhage. In evolution, the initial haemorrhagic polyp evolves to a fi brotic form19.

2.9.2. Diagnosis

Dysphonia is usually • with sudden, abrupt onset, coinciding with the extreme vocal eff ortIn evolution, polyps may • become large enough to determine an upper airway obstruction syndrome Figure 9. Vocal cord polyp

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Local examination: in early stages, a unilateral red-• bluish lesion is noticed at the level of the membranous part of the vocal folds; long-standing polyps loose their haemorrhagic appearance and become pedunculated, moving in-and-out of the glottis with respiration

2.9.3. Treatment

Microsurgical removal of the lesion, without disruption • of the subjacent vocal ligament; CO

2 lasers may be used

Surgery must be followed by speech therapy, to avoid • recurrences

2.10. Vocal nodules


Vocal nodules are bilateral chronic localized swellings of the vocal fold epithelium, usually placed at the junction of the anterior third with the middle third. It is one of the commonest laryngeal disorders determined by voice abuse and misuse. Children (usually boys), who scream and shout, and adults (mainly females) with professions demanding much of their voice (teachers, lawyers, singers, stock traders) are often affl icted19.

2.10.2. Diagnosis

Permanent or intermittent dysphonia is the main • symptomLocal examination: vocal nodules are always bilateral, • symmetrical, with a wide base; they appear in the middle of the membranous part of the vocal fold, which corresponds with the junction between the anterior 1/3 and posterior 2/3 of the glottis (the point of maximum vibration during phonation) Figure 10. Vocal cord nodules

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Early nodules are usually edematous and translucent, • whereas chronic lesions are white and fi broticStroboscopic examination•

2.10.3. Treatment

Conservative treatment: speech therapy should play a • primary role, initiallySurgical treatment: only in long-standing cases with large • nodules, which do not respond to conservative measures (microsurgical removal)

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3. TUMORS OF THE LARYNX

3.1. Benign Tumors

3.1.1. Papillomas


Squamous papillomas are the commonest benign laryngeal tumors. Th ey are classifi ed in two main histological categories20:

Keratinized papillomas: solitary lesions which occur • mainly in adults and arise at the level of the true vocal cords; the majority of these solitary adult lesions are not related to viral infection, but are connected to smoking and can develop further malignant transformationNon-keratinized papillomas (recurrent respiratory • papillomatosis, RRP), most frequently encountered in children (4 per 100,000 population in children) are multiple benign tumoral lesions, caused by a primary infection with human papilloma viruses (HPV-6 and HPV-11 most commonly found in laryngeal papillomatosis)

3.1.1.2. Diagnosis

Main presenting symptom in the adult form is • hoarsenessTh e juvenile form is more aggressive: besides hoarseness, • progressive dyspnoea, stridor and even acute life-threatening airway compromise may occur Local examination reveals a solitary exophytic lesion at • the level of the true vocal folds (adult form) or multiple pink or white, sessile or exophytic, pedunculated or wide-based lesions, resembling a “cluster of grapes” (juvenile form RRP)In the juvenile form lesions usually regress after puberty, • occur mainly on the true and false vocal cords, anterior

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commissure, epiglottis, often extend in the subglottis, trachea and bronchi; they have a high propensity to recur after treatment

3.1.1.3. Treatment

Surgical options for papillomas removal include • cold microsurgical techniques, CO

2 laser (mostly

recommended), microdebridement and offi ce-based angyolitic laser21

Adjuvant therapies: • α-Interferon, intralesional administration of antiviral agent Cidofovir22

3.1.2. Other benign laryngeal tumours

Hemangiomas occur in children (10%) and adults (90%); • in the paediatric type they are typically subglottic and may lead to severe upper airway obstruction21

Chondromas, leiomyomas, rhabdomyomas, • neurofi bromas, neurilemmomas or benign schwannomas, paragangliomas, benign minor salivary gland tumours


3.2.1. Squamous cell carcinoma (SCC)

3.2.1.1. Defi nition. Aetiology. Epidemiology

Laryngeal cancer accounts for 3% of male cancers in Europe23. It represents also the most common head and neck cancer worldwide. Th e incidence is higher in men (6:1 sex ratio), although an increase in women incidence is noticed in the last decades. Th e peak age incidence is in the 5th and 6th decades of life. Over 70 large case-control studies have investigated the aetiology of laryngeal carcinoma. Tobacco and alcohol are so dominant in these studies that the assessment of risk due to other possible factors (dietary defi ciencies, environmental exposures, HPV infection, GERD) is quite diffi cult24.

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3.2.1.2. Pathology

SCC represents 95% of all laryngeal malignancies. It has characteristic patterns of spread, depending on the site of origin (supraglottis, glottis or subglottis). Tumors of the supraglottis tend to remain locally confi ned (even with pre-epiglottic space or nodal spread) to their sub-site until relatively late. Th ey may extend antero-superiorly to the pre-epiglottic space and the base of tongue, or laterally to the hypopharynx (medial aspects of the pyriform fossae). Th ey involve vocal cords later in evolution, hoarseness is not the fi rst symptom, they tend to be diagnosed in a later stage, also they occur in areas of rich lymphatic drainage, so many patients have palpable neck lymph nodes at the moment of diagnosis (60% in advanced disease). Tumors of the glottis are diagnosed earlier because progressive hoarseness is the fi rst alarm symptom. Th e vocal cord has a poor lymphatic drainage, so these patients have a lower incidence of neck lymph node metastases (20% in advanced disease). Tumors of the subglottis tends to extend caudally and circumferentially, with early cricoid invasion, possible thyroid gland invasion. Upper airway obstruction develops rapidly in evolution. Clinically detectable lymph node metastases are surprisingly uncommon, paratracheal and mediastinal nodes may be involved. Transglottic cancer is defi ned by involvement of all three laryngeal subsites. True primary transglottic cancer originates from the laryngeal ventricle, and subsequently extends upwards and downwards through the paraglottic space24.

3.2.1.3. Symptoms

In glottic cancer, progressive hoarseness is the earliest cardinal symptom. Any patient with hoarseness persisting for three weeks or more should be referred to an otolaryngologist for examination. Advanced disease may lead to upper airway obstruction, haemoptysis, weight loss. Neck nodes are rarely the presenting complaint. In supraglottic cancer early symptoms are nonspecifi c: foreign body sensations, paresthesias. First presentation with a neck mass (lymph node metastasis) is quite common. As tumour

239

bulk increases, voice alterations appear (muffl ed, “hot-potato” voice). Advanced disease may lead to dysphagia, odynophagia with referred otalgia (invasion of the pyriform fossae or tongue base), upper airway obstruction, haemoptysis, weight loss. In subglottic lesions early symptoms are also nonspecifi c: foreign body sensations, paresthesias. Upper airway obstruction develops rapidly in evolution, due to the presence of a bulky tumour within the narrowed spaces of the subglottic region. Any involvement of the glottis or recurrent laryngeal nerves leads to hoarseness.

3.2.1.4. Diagnosis

Offi ce examination: mirror examination, fl exible • nasendoscopy, rigid endoscopy, stroboscopy; tumor site/size and vocal fold mobility must be carefully assessedPalpation of the neck: to evaluate the presence of lymph • node metastases and the status of the thyroid glandDirect pharyngolaryngoscopy: allows for multiple accurate • biopsies for histological examination, provides a precise evaluation of tumor location, size and superfi cial extent; the systematic examination of the entire upper aerodigestive tract (pan-endoscopy) allows the detection of synchronous cancers23 Biopsy is mandatory for • the diagnosisCT is currently the most useful modality to evaluate the • tumor extension and the presence of neck lymph nodes metastases; it is recommended to perform this examination prior to endoscopy and biopsies, to avoid infl ammation, which may lead to overestimation of cancer extent24

MRI, neck ultrasound examination•

Figure 11. Carcinoma of the left hemilarynx

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3.2.1.4. Treatment

Two possible curative treatment modalities are available: surgery and radiotherapy. Th e therapeutic decision depends on the location of the tumor (supraglottis, glottis, subglottis), tumor extent and TNM staging, hysthology of the tumor. All recent guidelines and standards recommend that laryngeal cancer patients should be managed by a multidisciplinary team: a head and neck surgeon, a radiotherapist/oncologist, speech therapist and a dietician25.


Early-stage glottic and supraglottic carcinoma (T1/T2) may be treated by partial laryngectomies. Th e following partial procedures are indicated for surgical treatment of early glottic cancer:

Endoscopic cordectomies, using cold microsurgical • techniques or CO

2 lasers26

Open approach cordectomies (laryngofi ssure/external • thyrotomy)Frontolateral laryngectomies: when the vocal fold tumor • involves the anterior commissure

Figure 12. CT scan: tumor located at the level of the left hemilarynx

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Anterior frontal laryngectomies: for tumors located in • the anterior comissure

For early supraglottic cancer, these partial procedures are recommended:

Horizontal supraglottic laryngectomies• Supracricoid partial laryngectomies with cricohyoidopexy, • if the tumor extends downwards to the ventricle or true vocal folds, but without arytenoid involvement

Near-total/Sub-total laryngectomies combine a supraglottic laryngectomy with a vertical hemilaryngectomy on the site of the tumor27 and may be used as a partial procedure in more advanced disease with vocal fold fi xation (T3). In advanced disease (T3/T4), total laryngectomies are advocated. Partial and total laryngectomies are associated with the surgical treatment of the neck: selective, modifi ed or radical neck dissections. Subglottic lesions may require a wide thyroid gland isthmectomy or even partial or total thyroidectomies.

3.2.1.4.2. Radiotherapy:

Curative radiotherapy (conventional or hyperfractionated •

Figure 13. Laryngeal carcinoma (surgical specimen)

242

techniques): in early glottic cancer, outcomes are similar with surgical treatment28

Postoperative radiotherapy is indicated when tumor • extends beyond the larynx or there is metastatic neck lymph nodes involvement

3.2.1.4.3. Chemotherapy:

Th e most frequently used drugs are cisplatin/carboplatin, • 5-fl uorouracil or, in palliation, methotrexateChemotherapy is used mainly in larynx preservation protocols • (induction therapy, concurrent chemo-radiotherapy, induction followed by concurrent chemoirradiation)29

New agents are under trials: taxanes and targeted therapies • (in particular cetuximab)23

3.2.1.4.4. Voice rehabilitation after total laryngectomy:

Air-swallowing techniques (erigmophonation)• External devices (laryngophones)• Tracheoesophageal voice prosthesis•

In 1998 Strome performed the fi rst true successful laryngeal transplant, in Cleveland, USA30. Further research is required into how this technique might eventually be brought into routine clinical practice.

3.2.1.5. Prognosis

5-year survival rates23 for laryngeal cancer:T1-T2: 70% for supraglottis and 80% for glottis• T3: 60% for supraglottis and 70% for glottis• T4 resectable: 40% for supraglottis and 50% for glottis• T4 non-resectable: below 30%•

3.2.2. Other laryngeal malignancies

Non-Hodgkin lymphomas are the second most common • malignancy of the larynxEpithelial: verrucous carcinomas, adenocarcinomas, • adenoid cystic carcinomas, mucoepidermoid carcinomas, malignant melanomas, carcinoidsMesenchymal: chondrosarcomas, leiomyosarcomas, • rhabdomyosarcomas, fi brosarcomas, Kaposi sarcomas

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4. LARYNGEAL NERVE DISORDERS

4.1. Laryngeal nerve palsy

Th e larynx has three major functions: protection of the upper airway (acting like a sphincter to close the airway during deglutition, thus preventing aspiration of food and saliva), voice production and breathing. A functional larynx requires an adequate sensory and motor innervation of its anatomical structures. Both aff erent and eff erent innervations are supplied by branches of the vagus nerve (10th cranial nerve). Th e vagus nerve exits the skull base through the jugular foramen, and gives rise to some important branches:

Th e pharyngeal nerve divides into multiple branches to • form the pharyngeal plexus in order to supply motor and sensory innervation to the pharynxTh e superior laryngeal nerve takes origin from the • inferior ganglion of the vagus nerve and divides into the internal and external laryngeal nerves, supplying sensitive innervation to the supraglottis and motor innervation to the cricothyroid muscleTh e recurrent laryngeal nerve supplies motor innervation • to all intrinsic laryngeal muscles, except the cricothyroid muscle. On the right side, it arises at the level of the neck base, loops the subclavian artery and ascends to the larynx. On the left side it arises in the upper mediastinum, loops the aortic arch, has a short intrathoracic course and then ascends through the tracheoesophageal groove to the larynx

Impairment of laryngeal nerve function is followed by a variety of phonatory, respiratory and swallowing disorders.

4.1.1. Aetiology

Laryngeal nerves paralysis may be caused by lesions located at the level of the central nervous system (nucleus ambiguous and

244

its supranuclear tracts) or of the main trunk of the vagus nerve and its two laryngeal collateral branches. Surgical procedures in the neck, skull base and upper mediastinum are the main causes of peripheral lesions. Th yroid gland surgery is frequently followed by recurrent laryngeal paresis or palsy. Paresis after primary thyroid surgery for benign disease has been reported to range from 2-7%, permanent palsy from 0.5-4%; after revision surgery and surgery for malignant thyroid disease, laryngeal nerve lesions rates increase to 10-20%31. Malignant tumors of the larynx, hypopharynx, oesophagus, thyroid gland, trachea, upper left mediastinum, lymph node metastases may invade during evolution the vagus nerve or its collateral laryngeal branches. Viral neuronitis probably accounts for most cases of the idiopathic forms. Other conditions involved:

Central nervous system lesions: neurovascular (stroke), • tumors, degenerative disorders (e.g. amyotrophic lateral sclerosis)Lateral skull-base lesions: see Pharynx, chapter 4, table 2• Infectious diseases of the peripheral nerve system: • poliomyelitis, diphtheria, herpes zoster, rabies, botulismDegenerative lesions of the peripheral nerve system: • Guillain-Barre’s syndromePrimary neurogenic tumors: schwannomas, • neurilemmomas Left aortic aneurysms•

4.1.2. Symptoms

Unilateral paresis/paralysis of the vagus or recurrent laryngeal nerve is characterized by impaired abduction/adduction of the vocal fold or immobility in a paramedian position. Th e constant glottic gap, determined by the inappropriate adduction during phonation, results in a typical hoarseness and breathiness of the voice. Bilateral recurrent laryngeal nerve lesions may present with an acute or a chronic insidious onset:

Th e acute form (typically after thyroid gland surgery) • results in a life-threatening upper airway obstruction, which requires emergency measures (intubation, tracheostomy)

245

Th e chronic form is characterized by mild dyspnoea with • inspiratory stridor; superimposed acute episodes of upper airway infections may lead to local infl ammation and oedema with acute upper respiratory distressVoice quality is only mildly aff ected (vocal folds are • paralyzed in adduction)In rare cases, when vocal folds are fi xed in abduction, • symptoms of chronic aspiration are the main complaints

4.1.3. Diagnosis

Th e recommended European standard diagnostic steps31 are:

Detailed history• Inspection and palpation of the neck• Indirect endoscopy with rigid telescopes or fl exible • nasendoscopesTh yroid gland assessment• Ultrasound of the neck•

Additional diagnostic procedures include:Stroboscopy• Direct pan-endoscopy• Laryngeal electromyography• Barium-swallow examination, pulmonary evaluation• CT/MRI of the thorax, neck, skull base and brain• Laboratory tests•

4.1.4. Treatment


In unilateral vocal fold paresis/paralysis: initial voice rest, • oral steroids or non-steroidal antiinfl ammatory drugs (NSAIDs), antiviral/antibiotics (if an infectious aetiology is suspected), speech therapyIn chronic bilateral paralysis: acute episodes of upper • airway infections must be promptly and adequately treated (antivirals/antibiotics, intravenous steroids, NSAIDs) to avoid the development of an acute upper airway obstruction syndrome

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Th e recommended European standard for surgical procedures31:

For voice improvement in unilateral paralysis: medial • vocal fold augmentation using fat, collagen or disperse silicone; medialization thyreoplasty using autologous cartilage or laryngeal titanium implantsFor airway improvement in bilateral paralysis: temporary • intubation/tracheostomy (in acute upper airway obstruction), posterior endoscopic cordectomy, temporary or permanent laterofi xation of one vocal fold, endoscopic arytenoidectomyFor deglutition disorders: laryngeal elevation, medialization • thyroplasty to reduce the glottic gap, myotomy of the crycopharyngeal muscle (upper oesophageal sphincter)

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5. TRACHEOSTOMY

5.1. Defi nition. History

Tracheotomy is the term used to describe the surgical opening of the trachea. Tracheostomy is used to describe the creation of a stoma at the skin surface which leads into the trachea. It may be temporary or permanent32. Tracheostomy is one of the oldest surgical procedures33. It was fi rst depicted on Egyptian tablets around 3600 BC. It was described in the Rigveda, a Sanskrit text in 2000 BC. It is believed that a tracheotomy was performed by Asclepeiades of Bithynia, who lived in Rome around 100 BC. Antonio Brassavola (1490-1554) of Ferrara performed a tracheotomy to a patient with peritonsillar abscess, previously refused by barber surgeons. Th e patient made a complete recovery, and Brassavola published his account in 1546. It is considered the fi rst recorded successful tracheostomy, despite the many ancient references. Sanctorius (1561-1636) was the fi rst to use a trocar and cannula, left in place for 3 days. Th e currently used technique was described by Chevalier Jackson in 190934. He emphasized the importance of postoperative care, and warned of the complications after high tracheostomy (cricothyrotomy).

5.2. Indications

Th e main indications for tracheostomy35 are:Upper airway obstruction • Prolonged intubation• Facilitation of ventilation support• Excessive bronchopulmonary secretions• Inability to intubate• Adjunct to major head and neck surgery procedures• Adjunct to management of major head and neck trauma•

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5.3. Techniques

Cricothyroidotomy/minitracheostomy (coniotomy, inferior • laryngotomy): in extreme life-threatening situations the upper airway is opened at the level of the cricothyroid membrane and the lumen is maintained by the insertion of a mini tracheostomy tube; patient should be referred in the next 24 hours to an ENT department to undergo a classic tracheostomy; if the coniotomy is maintained for a longer time, subsequent subglottic stenosis may occurPercutaneous tracheostomy: minimally invasive alternative • to open tracheostomy; commercial kits based on well-defi ned techniques are available. Th e most commonly used is the dilatational technique36

Open surgical tracheostomy• Open surgical tracheostomy should be performed, wherever possible, in the operating theater under local or general anesthesia. Th e patient is placed in the supine position on the table, with neck extension (a sandbag placed under the shoulders). A horizontal skin incision is performed two-fi ngers above the suprasternal notch. Th e superfi cial anterior jugular veins sometimes must be ligated. Strap muscles and cervical fascias are dissected on the midline, the thyroid gland isthmus is identifi ed, divided and ligated. Th e anterior tracheal wall is completely exposed. Accurate hemostasis and a local intra-tracheal anesthetic injection must be performed before opening the trachea. Th e incision is done at the level of the second/third tracheal rings. An inferiorly based anterior tracheal wall fl ap may be designed and sutured to the skin to ease the change of the tracheostomy tube in the

Figur 14. Cuff ed tracheostomy tube

249

postoperative care. Th e tracheostomy tube, whose cuff integrity has been previously tested by the surgeon, is inserted through the stoma into the tracheal lumen.

5.4. Postoperative management

Humidifi cation and removal of secretions by frequent • suctioningLocal toilet of the surgical wound; changing of the tube • should be performed 3-4 days after the surgery, by the surgeon himselfTh e cuff of the tube must be kept infl ated for the shortest • time possible, to avoid further damage of the tracheal mucosa and the development of subsequent cicatricial stenosisAntibiotics, analgesics, mucolytics•

5.5. Complications

Complications following tracheostomy can be divided into these three categories6:

Figure 15. Tracheostomy

250

Immediate (within 24 hours): haemorrhage (usually • venous), air embolism, apnea, cardiac arrest, local damage of the cricoid cartilage, recurrent laryngeal nerve, oesophagusIntermediate (within 4 weeks): displacement of the • tube, obstruction of the tube with secretions/crusts, surgical subcutaneous emphysema, pneumothorax/pneumomediastinum, infection, necrosis, tracheo-oesophageal fi stulaLate: tracheal stenosis, decannulation problems•

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6. LARYNGEAL TRAUMA

6.1. Defi nition. Aetiology

Laryngeal traumas are characterized by heterogenous lesions secondary to blunt, perforating or endoluminal forces aff ecting the head and neck. Injuries to the larynx are relatively rare, less of 1% of all trauma involves the larynx. Th ere is a risk of other concurrent lesions: intracranial (13%), cervical spine (8%) and oesophagus (3%)37. Laryngeal traumas can be classifi ed in internal and external:

Internal injuries are secondary to endotracheal anaesthesia • procedures, endolaryngeal surgical procedures, ingestion/inhalation of various toxic agents (caustics, acids, gases)External injuries are secondary to blunt or perforating • forces, surgical procedures

Laryngeal trauma may be secondary to accidents, aggressions, suicide or iatrogenic. Lesions are heterogenous in terms of their mechanics and consequences. Internal traumas may lead to soft tissue hematomas and edemas, lacerations, ruptures of the vocal folds, dislocations of the arytenoid cartilages. External blunt traumas may lead to endolaryngeal soft tissue lesions, fractures of the cartilaginous framework, ruptures of the laryngeal membranes and ligaments. External penetrating traumas are associated with lacerations of the larynx, trachea and soft tissues of the neck, laryngeal nerve injuries, bleeding of major vessels, aspiration, air embolism, tracheo-arterial and tracheo-oesophageal fi stulas38. In evolution, besides the acute respiratory and circulatory complications, chronic sequelae must be carefully monitored. Th e major long-term, chronic complication is represented by the development of subsequent laryngotracheal stenosis.

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6.2. Symptoms

Dyspnoea and stridor• Hoarseness• Hemoptysis and irritative coughing, aspiration• External bleedings• Pain, fear, shock• Cardiopulmonary arrest•

6.3. Diagnosis

Th e recommended European standard diagnostic steps38 include:

Documented and accurate history (onset of symptoms, • mechanism, previous surgery)Inspection: hematomas and ecchymosis, subcutaneous • emphysema, penetrating lesions, bleeding, strangulation marks, cyanosisPalpation: crepitations• Indirect endoscopy with rigid or fl exible scopes• Microlaryngoscopy and tracheobronchoscopy•

Additional diagnostic procedures:X-ray of the thorax and neck• CT/MRI of the thorax and neck• Pulmonary functional assessment • Blood oxygen saturation monitoring•

6.4. Treatment

6.4.1. Conservative treatment:

Close clinical observation for up to 48 hours after injury• Bed rest, voice rest,• Humidifi ed air, oxygen, hydration• Steroids, antibiotics, analgesics•

6.4.2. Surgical treatment:

Th e recommended European standard surgical procedures38 are:

Endolaryngeal microsurgery (repairment of soft tissue • lesions, resolution of scars)

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Endoscopic surgery of the trachea• Endo/extraluminal repositioning and fi xation of fragments•

Additional procedures include:Temporary tracheostomy• Laryngeal and tracheal stents• Laryngeal and tracheal enlargement procedures•

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7. FOREIGN BODIES IN THE LARYNX

7.1. Defi nition. Aetiology

Lodging of a foreign body into the larynx causing hoarseness, croupy cough and acute airway obstruction. Most airway foreign bodies aspiration occurs in children younger than 15 years; children aged 1-3 years are the most susceptible39. Children are at risk for putting small toys, candies, or small vegetables (nuts, peanuts, bean, seeds) into their mouth. Th ese objects may be propelled posteriorly, triggering a refl ex inhalation. Among adults, some conditions may facilitate foreign body aspiration: impaired swallow and cough refl ex, mental retardation, alcohol or sedative use, poor dentition, loss of consciousness. After aspiration, the foreign body can settle into 3 anatomic sites: the larynx, trachea or bronchus. 80-90% are lodged in the bronchi. Th e right main bronchus is most frequently encountered, because of its lesser angle of convergence compared with the left one39. Larger foreign bodies tend to locate within the larynx or trachea. Laryngeal foreign bodies occur less frequently than bronchial foreign bodies (8% of aspirations) and tend to fall into two categories based on size and shape40:

sharp, thin and aerodynamic objects are at risk of • embedding in the laryngeal mucosa or of becoming caught between the vocal foldssoft, large, conforming objects may become lodged in the • narrow, funnel shaped glottic or subglottic airway

Acute upper airway obstruction may be due to the foreign body itself, or may be the result of the severe oedema and infl ammatory reaction of the surrounding tissues41.

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7.2. Symptoms

Hoarseness, aphonia• Barking, croupy cough• Inspiratory stridor, cyanosis, acute airway obstruction• Dysphagia, dyspnea, hemoptysis, wheezing, chronic • cough

In the café coronary syndrome, a large foreign body (often, poorly chewed meat) lodges within the larynx or trachea, causing nearly complete upper airway obstruction. Respiratory distress, aphonia, cyanosis, loss of consciousness and sudden death occur in quick succession, unless the foreign body is dislodged42.

7.3. Diagnosis

A history of an acute choking episode, followed by a period • of respiratory manifestations, is extremely important to be identifi edImaging studies: neck X-Rays (lateral and AP views); • foreign bodies are frequently radiolucent, making diagnosis with standard imaging diffi cultEndoscopy: diagnostic and therapeutic•

7.4. Treatment

Heimlich’s maneuver for acute upper airway obstruction • (abdominal thrusts)Removal by direct laryngoscopy, bronchoscopy or • surgeryRespiratory support (intubation, tracheostomy)•

Abdominal thrusts, also known as the Heimlich’s maneuver (after Henry Heimlich, who fi rst described the procedure in 1974), involves a rescuer standing behind the patient and using their hands to exert pressure on the bottom of the diaphragm. Th is compresses the lungs and exerts pressure on any foreign body lodged within the larynx or trachea, hopefully expelling it43.

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8. REFERENCES

1. Piazza C, Ribeiro JC, Bernal-Sprekelsen M, Paiva A, Peretti G. Anatomy and phisiology of the larynx and hypopharynx. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 461-471.

2. Mathieson L, Carding P. Phisiology of the larynx. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2155-2163.

3. Woo P, Colton R, Casper J, Brewer D. Diagnostic value of stroboscopic examination in hoarse patients. Journal of Voice 1991; 5: 231-238.

4. McGlashan J. Disorders of the voice. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2192-2210.

5. Sittel C. Offi ce examination of the larynx. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 473-474.

6. Bradley PJ. Basic surgical procedures on the respiratory tract: laryngoscopy, tracheoscopy and including tracheostomy. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 527-535.

7. Sataloff RT, Spiegel JR, Hawkshaw MJ. Strobovideolaryngoscopy: Results and clinical value. Annals of Otology, Rhinology and Laryngology 1991; 100: 725-727.

8. Dejonckere PH. Voice evaluation and respiratory function assessment. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 563-574.

9. Swift AC. Acute infections of the larynx. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2248-2257.

10. Remacle M. Infl ammatory diseases and lasers. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 475-481.

11. Wurtele P. Acute epiglottitis in children and adults: a large-scale incidence study. Otolaryngology - Head and Neck Surgery: Offi cial Journal of American Academy of Otolaryngology - Head and Neck Surgery 1990; 103: 902-908.

12. Cupic H, Kruslin B, Belicza M. Epithelial hyperplastic of the larynx in biopsy specimens. Acta Oto-Laryngologica Supplementum 1997; 527: 103-104.

13. MacKenzie K. Chronic laryngitis. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2258-2270.

14. Travis LW, Hybels RL, Newman MH. Tuberculosis of the larynx. Th e Laryngoscope 1976; 86: 549-558.

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15. Holinger PH, Gelman HK, Wolfe Jr CK. Rhinoscleroma of the lower respiratory tract. Th e Laryngoscope 1977; 87: 1-9.

16. Vrabec DP. Fungal infections of the larynx. Otolaryngologic Clinics of North America 1993; 26: 1091-1114.

17. Tsuji DH, Fukuda H, Kawasaki Y, Kavaida M, Ohira T. Actinomycosis of the larynx. Auris, Nasus, Larynx 1991; 18: 79-85.

18. Bastian RW. Benign vocal fold mucosal disorders. In: Flint PW, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 2150-2186.

19. Herranz J, Bouzas JG, Barro CV, Lourdes MM. Mucosal disease of the glottis. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 483-486.

20. Barnes I. Diseases of the larynx, hypopharynx and oesophagus. In: Barnes I, ed. Surgical pathology of the head and neck. New York: Dekker 2001; 151-154.

21. Bradley PJ. Benign neoplasms and other tumours of the larynx. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 487-497.

22. Dikkers FG. Treatment of recurrent respiratory papillomatosis with microsurgery in combination with intralesional cidofovir - a prospective study. European Archives of Oto-Rhino-Laryngology 2006; 263: 440-443.

23. Chevalier D, Lefebvre J-L. Laryngeal cancer. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 499-502.

24. Birchall M, Pope L. Tumours of the larynx. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2598-2622.

25. British Association of Otorhinolaryngologists. Role of the multidisciplinary team. Eff ective head and neck cancer management. Th ird consensus document. 2003; 63-65.

26. Eckel HE, Schneider C, Jungehulsing M, Damm M, Schroder U, Vossing M. Potential role of transoral laser surgery for larynx carcinoma. Lasers in Surgery and Medicine 1998; 23: 79-86.

27. Biller HF, Lawson W. Partial laryngectomy for vocal cord cancer with marked limitation or fi xation of the vocal cord. Th e Laryngoscope 1986; 96: 61-64.

28. Dey P, Arnold D, Wight R, MacKenzie K, Kelly C, Wilson J. Radiotherapy versus open surgery versus endolaryngeal surgery (with or without laser) for early laryngeal squamous cell cancer. Cochrane Database of Systematic Reviews 2002; CD002027:

29. Forastiere AA, Goepfert H, Maor M et al. Concurrent chemotherapy and radiotherapy for organ preservation in advanced laryngeal cancer. New England Journal of Medicine 2003; 349: 2091-2098.

30. Strome M, Stein J, Esclamado R, Hicks D, Lorenz RR, Braun W. Laryngeal transplantation and 40-months follow-up. New England Journal of Medicine 2001; 344: 1676-1679.

31. Eckel HE. Laryngeal nerve disorderds. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 519-525.

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32. Pracy P. Tracheostomy. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2292-2304.

33. Pratt LW, Ferlito A, Rinaldo A. Tracheotomy: historical review. Th e Laryngoscope 2008; 118: 1597-1606.

34. Jackson C. Tracheostomy. Th e Laryngoscope 1909; 19: 285-290.35. Goldenberg D, Bhatty N. Management of the impaired airway in adult.

In: Flint PW, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 2441-2453.

36. Ciaglia P, Firsching R, Syniec C. Elective percutaneous dilatational tracheostomy. A new simple bedside procedure; preliminary report. Chest 1985; 87: 715-719.

37. Jewett BS, Shockley WW, Rutledge R. External laryngeal trauma analysis of 392 patients. Archives of Otolaryngology - Head & Neck Surgery 1999; 125: 877-880.

38. Kleinsasser N. Trauma to the laryngeal and tracheal. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 503-506.

39. Bloom DC, Christenson TE, Manning SC et al. Plastic laryngeal foreign bodies in children: a diagnostic challenge. International Journal of Pediatric Otorhinolaryngology 2005; 69: 657-662.

40. Lima JA. Laryngeal foreign bodies in children: a persistent, life-threatening problem. Th e Laryngoscope 1989; 99: 415-420.

41. Brama I, Fearon B. Laryngeal foreign bodies in children. International Journal of Pediatric Otorhinolaryngology 1982; 4: 259-265.

42. Mittleman RE, Wetli CV. Th e fatal cafe coronary. Foreign body airway obstruction. JAMA 1982; 247: 1285-1288.

43. Heimlich HJ. A life-saving maneuver to prevent food-choking. JAMA 1975; 234: 398-401.

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V. SALIVARY GLAND BASICS

1. SALIVARY GLAND BASICS


1.1.1. The parotid gland

Th e parotids are the largest of the major salivary glands. Th ey are compound, tubuloacinar, mepocrine and exocrine glands, composed entirely of serous output-producing acini1. Each gland is located in the parotid space, which lies between the posterior border of the ascending mandibular ramus and the anterior border of the mastoid process of the temporal bone. Th e superior border is closely related to the temporomandibular joint and the external auditory meatus. Th e inferior border reaches the anterior margin of the sternocleidomastoid muscle and the posterior belly of the digastric muscle, at the level of the angle of the mandible. Th e parotid is a unilobular gland through which the facial nerve passes. Th e intraparotid course of the facial nerve classically divides the gland into a superfi cial and a deep lobe. Anatomically, no true superfi cial and deep lobe exists. Th e term superfi cial parotidectomy refers only to the surgically created boundary from facial nerve dissection. Th e deep portion of the gland is located within the anterior prestyloid compartment of the parapharyngeal space. Th at’s why

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tumors located at this level may present as intraoral masses, pushing the palatine tonsils medially and inferiorly. Th e Stensen duct drains the parotid gland. Each duct emerges from the anterior border of the gland and runs horizontally across the masseter muscle, 1 cm below the zygomatic arch. As it crosses the anterior masseter border, it penetrates the buccal fat pad and the buccinator muscle and opens intraorally at the level of the second molar teeth. Th e facial nerve exits the skull base via the stylomastoid foramen and enters the parotid gland. Within the gland, the nerve divides into two major branches: the upper temporofacial and lower cervicofacial divisions. Th is division point is known as pes anserinus. Subsequent branching is variable, but the nerve generally forms 5 branches: temporal and zygomatic (from the upper division), buccal, mandibular and cervical (from the lower division). Arterial supply is provided by branches of the external carotid artery. Venous drainage is provided by the posterior facial

Figure 1. Th e parotid gland

1 - Facial nerve (VII); 2 - Parotid gland; 3 - Marginal branch; 4 - Cervical branch; 5 - Posterior auricular vein; 6 - Retromandibular vein; 7 - External jugular vein; 8 - External carotid artery; 9 - Facial artery; 10 - Submandibular gland; 11 - Submandibular duct (Wharton); 12 - Buccal branch; 13 - Parotid duct (Stensen); 14 - Transverse facial artery; 15 - Zygomatic branch; 16 - Temporal branch; 17 - Superfi cial temporal artery; 18 - Auriculotemporal nerve

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vein to the internal jugular vein. Th e parotid is the only major salivary gland which contains lymph nodes, embedded within the glandular parenchymal tissue. Th e gland receives parasympathetic secretomotor innervation from the inferior salivatory nucleus. Preganglionic fi bers synapse in the otic ganglion. Th e postganglionic fi bers travel with the auriculotemporal nerve (branch of the trigeminal nerve) to the parotid tissues.

1.1.2. The submandibular gland

Th e submandibular gland lies within the submandibular triangle or space. Th is triangle is delineated by the horizontal ramus of the mandible, the posterior and the anterior belly of the digastric muscle. Th e gland is partially enclosed between the two layers of the deep cervical fascia and is in close proximity to the facial vein and artery. Th e fascia’s superfi cial layer is attached to the inferior border of the mandible and covers the inferior surface of the gland. Th e deep layer is connected also to the mandible and covers the medial aspect of the gland. Th e inferior superfi cial surface of the gland is covered by skin, subcutaneous fat, platysma muscle and cervical fascia and is crossed by the facial vein. Th e mandibular branch of the facial nerve loops 1 cm below the horizontal ramus of the mandible to supply motor innervation to the lower lip. Besides the gland, the submandibular space hosts the submandibular lymph nodes. Th e deep lobe of each gland arises at the posterior free edge of the mylohyoid muscle and extends to the back of the sublingual gland. It is in close relationship superiorly with the lingual nerve. Th e Wharton duct opens into the fl oor of the oral cavity at the side of the lingual frenulum. Th e arterial blood supply comes from branches of facial and lingual arteries, the venous drainage is supplied by the deep lingual veins. Th e parasympathetic secretomotor innervation comes from the superior salivatory nucleus. Preganglionic fi bers travel with the facial nerve, the chorda tympani nerve and the lingual nerve to the submandibular ganglion. Postganglionic fi bers innervate both submandibular and sublingual glands.

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1.1.3. The sublingual gland

Th e sublingual gland lies over the mylohyoid muscle and is covered by the mucosa of the fl oor of the mouth. Th e lingual nerve and the Wharton duct lie medially to the sublingual gland.

1.1.4. The minor salivary glands

Th e minor salivary glands are widely dispersed in the submucosa of the head and neck region. Th e highest density is found in the roof of the oral cavity and oropharynx. Th ey are grouped according to their anatomic location: labial, buccal, palatal, tonsillar, nasal cavity, nasopharynx, larynx, etc.

1.2. Physiology

Th e basic unit of a salivary gland consists of an acinus, a secretory duct and a collecting, excretory duct. Acini are classifi ed as serous (parotid gland), mucous (sublingual and minor salivary glands) and mixed (submandibular glands). Saliva is a complex mixture of organic (enzymes, immunoglobulins and mucines) and inorganic compounds (electrolytes)2.

Figure 2. Th e submandibular gland

1 - Hypoglossal nerve (XII); 2 - Glossopharyngeal nerve (IX); 3 - Lingual artery; 4 - Hyoid bone; 5 - Hyoglossus muscle; 6 - Mylohyoid muscle; 7 - Deep lobe of the submandibular gland; 8 - Submandibular duct (Wharton); 9 - Stylohyoid muscle; 10 - Lingual nerve

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Th e main functions of the saliva are3, 4:Lubrication and protection of the structures of the oral • cavity and the oropharynxBuff ering and clearance: saliva has a slightly alkaline pH • and contains some buff er systems, the most important being the bicarbonateMaintenance of tooth integrity• Antibacterial activity: saliva contains both immunologic • mediators (IgA) and nonimmunologic compounds (various proteins, mucins, enzymes)Taste and digestion: saliva aids in the mastication and • deglutition process through its lubricating actions; in addition, salivary amylase initiates the digestion of carbohydrates

Th e secretion of saliva is tightly regulated by various factors, including the autonomous nervous system, humoral factors and disease states.

1.3. Disorders of salivary secretion

1.3.1. Hyposecretion

Salivary glands hypofunction is often associated with a sensation of dry mouth (xerostomia). Th e main causes for hyposalivation5 are:

Iatrogenic: medications (antidepressants, anxiolytics, • diuretics, anti-hypertensive agents, antihistamines), radiotherapy, chemotherapy, surgical traumaChronic infl ammatory/autoimmune disorders: Sjogren’s • syndromeInfections: HIV/AIDS, mumps, EBV, tuberculosis• Neurological disorders: depression, anxiety, Parkinson’s • diseaseEndocrine disorders: diabetes, hyper- and hypothyroidism• Genetic abnormalities: cystic fi brosis• Malnutrition, anorexia, bulimia, alcohol abuse• Others: hypertension, burning mouth syndrome•

1.3.2. Hypersecretion

Th e overproduction of saliva is referred to as hypersalivation, sialorrhoea, ptyalism. Th e symptom is uncommon, as any

264

excessive saliva is usually swallowed. Causes are usually local, due to increased refl exes (pain, infections, dental procedures, new dentures) or systemic (nausea, gastroesophageal refl ux disease)


1.4.1. History

Symptoms related to salivary gland diseases are quite limited in number and often nonspecifi c. Th e main complaints are local swellings, pain, xerostomia, foul taste, sometimes sialorrhoea. Th e most common presenting symptom of benign or malignant disease of the major salivary glands is an asymptomatic chronic swelling. A major salivary gland swelling may be secondary to a duct obstruction, infl ammation or neoplasia. Intermittent episodes of acute painful swelling, related to salivary stimulation during meals, are typical for duct obstruction with calculi. Pain is reported in 2.5-4% of patients with benign parotid neoplasms and 10-29% of patients with parotid malignancies. Th e same symptom is associated with malignant submandibular neoplasms in up to 50% of cases6. A detailed history related to the presence of concurrent systemic diseases, drugs being taken, previous radiotherapy/chemotherapy, dietary and nutrition must be taken into account.

1.4.2. Physical examination

Inspection: symmetry, skin color, possible pulsations, • unilateral or bilateral swellings, signs of facial nerve palsyExternal palpation of the parotid and submandibular • region: assessment of major salivary gland masses (location, size, mobility, fi xation to adjacent structures), the presence of enlarged lymph nodesCombined intraoral and external palpation: may reveal • the presence of a calculus in the draining ductExamination of the oral cavity: dental hygiene, inspection • of the draining orifi ces of the Stensen’ and Wharton’ ducts, inspection and palpation of the tonsillar region (tumors located in the deep lobe of the parotid may present as

265

oropharyngeal swellings, displacing the palatine tonsil medially and inferiorly)A complete ENT examination should be performed: • evaluation of minor salivary glands tissues


1.4.3.1. Imaging techniques

Plain radiographs: may detect a radio opaque calculus• Sialography: lithiasis is the most common indication; • contrast media is injected through the intraoral draining orifi ce of the gland, after a previous topical anaesthesia and a gentle dilation have been performedUltrasound examination• Nuclear medicine: technetium-99m pertechnetate scans • are useful in the assessment of Whartin tumors and oncocytomas, showing high radionuclide uptake; all other tumors are generally radionegative6

CT scans and MRI•

Figure 3. Ultrasound examination (Whartin tumor in the parotid gland)

1.4.3.2. Sialoendoscopy

Sialendoscopy is a modern minimally invasive procedure, which allows direct inspection of the eff erent salivary duct system, by means of a narrow-diameter microendoscope. Th is new technique is performed under local anesthesia on an outpatient basis. A lacrimal probe is used to gently dilate the intraoral orifi ce, then the scope is introduced under direct visualisation7.

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Sialendoscopy has opened up a new frontier for both diagnostic evaluation and treatment, mainly in obstructive salivary gland disease (lithiasis and stenosis of the glandular ducts)8.

1.4.3.3. Fine-needle aspiration (FNA) cytology

FNA provides valuable information about the histopathology of a salivary gland tumor, before surgical treatment. It also helps to diff erentiate a reactive lymph node adjacent to a salivary gland or within the parenchyma of the gland (parotid gland), from a glandular tumor. It supplies accurate data in nearly 90% of the cases6.

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2. INFLAMMATORY DISORDERS

2.1. Infectious infl ammatory disorders

2.1.1. Acute suppurative sialadenitis


Acute suppurative sialadenitis is an acute bacterial infl ammation of the salivary gland parenchyma, the parotid gland being most commonly involved. Th is condition is secondary to retrograde bacterial contamination of the salivary ducts from the oral cavity. It is strongly associated with medically debilitated and post surgical patients9. Mechanical impairment of the salivary fl ow is another factor involved in the development of acute infections. Th e most commonly isolated germs are penicillin-resistant Staphylococcus aureus, but Streptococcus species, Haemophilus infl uenzae, gram-negative and anaerobic bacteria are also involved10.

2.1.1.2. Diagnosis

Rapid onset of local pain and swelling, fever, chills, • malaiseSigns of systemic dehydration, with dry mucous • membranes (mainly in post surgical patients), signs of local infl ammation (rubor, calor, dolor)Bimanual palpation of the gland elicits purulent discharge • from the ductal orifi ceCultures from the suppurative drainage should be • obtainedUltrasound examination, CT scans•

2.1.1.3. Treatment

Wide-spectrum antibiotic therapy, according to the • cultures results

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Analgetics, antipyretics, non-steroidal antiinfl ammatory • drugs (NSAIDs), hydrationRarely, conservative measures fail to eradicate the • infection, and surgical drainage of localized, collected abscesses must be performed

2.1.2. Chronic sialadenitis


Chronic sialadenitis is a localized disease of the salivary glands, characterized by repeated episodes of pain and infl ammation. Th e parotid is the commonest site. Salivary stasis following obstructing disease (sialolithiasis, stenosis and strictures of the ducts, extrinsic compression) predisposes to recurrent episodes of infection and infl ammation. Th is leads to subsequent acinar destruction, fi brous replacement and sialectasis10.

2.1.2.2. Diagnosis

History of recurrent swelling and tenderness of the • glandular tissuePalpation reveals enlargement of the gland• Ultrasound examination• Sialendoscopy•

2.1.2.3. Treatment

No specifi c successful treatment exists• Non-steroidal antiinfl ammatory drugs (NSAIDs), • sialogogues (pilocarpine)Sialendoscopy for obstructive problems• Surgical removal of the gland, when conservative measures • failed, is proved to be safe and eff ective11

2.1.3. Recurrent parotitis of childhood (RPC)


Recurrent parotitis of childhood (RPC) is the most common non-viral infectious condition of salivary glands in children. It usually resolves around puberty12. Th e aetiology is unclear, no specifi c treatment exists. Some authors advocated the role of congenital ductal ectasia in the

269

development of the disease13. Staphylococcus aureus and Streptococcus viridans are the most frequently cultured agents10.

2.1.3.2. Diagnosis

Recurrent episodes of acute or subacute, unilateral or • bilateral parotid swellings, associated with fever and painPalpation: mucopurulent discharge may be expressed • from the erythematous ductal orifi cesUltrasound examination• Sialendoscopy•

2.1.3.3. Treatment

Adequate hydration, gland massage, sialogogues • (pilocarpine)Antibiotics according to culture results, non-steroidal • antiinfl ammatory drugs (NSAIDs)

2.1.4. Mumps


Mumps is an acute nonsuppurative viral parotitis caused by the paramyxovirus, a RNA virus related to infl uenza and parainfl uenza viruses. It is the most common viral infection of the salivary glands, 85% of the cases occur in children below the age of 1510. Th e disease is highly contagious, spreading by airborne droplets from saliva and nasopharyngeal secretions.

2.1.4.2. Diagnosis

History: a viral prodromal period of 1-3 days characterized • by malaise, anorexia, headaches, low-grade feverPainful swelling of the parotid gland, associated with • otalgia and trismus; pain is augmented by eating or chewingIn 75% of the cases, there will be bilateral involvement • causing lateral displacement of the pinna14; commonly, the parotid on one side will enlarge fi rst, followed by the opposite gland in 1-5 daysTh e ductal orifi ce may be oedematous and congested, but • no pus is coming out from the ductLaboratory tests: viral serology, leukopenia•

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Th e most frequent complications are: orchitis (25% in young males), pancreatitis, sensorineural hearing loss (1/20,000 in children, being the fi rst cause of acquired sensorineural hearing loss in children) and meningoencephalitis12.

2.1.4.3. Treatment

Bed rest, oral hygiene, adequate hydration, dietary • modifi cations to decrease secretory activityAnalgetics, sialogogues (pilocarpine)•

2.1.5. HIV

HIV-associated salivary gland disease (HIV-SGD) is a term used to describe salivary glands involvement in AIDS. It is characterized by a diff use swelling of the salivary glands. Th ese diff use enlargements are secondary to various pathological entities: Kaposi’s sarcomas, lymphomas, lymphoproliferative and cystic enlargements accompanied by salivary dysfunction (xerostomia)10. Th e parotid is most commonly involved. As the parotid parenchyma contains lymph nodes, they also may be enlarged.

2.1.6. Granulomatous infections


Mycobacterium tuberculosis• and atypical mycobacteria may aff ect lymph nodes adjacent to the salivary glands or intraparotid lymph nodesPositive PPD skin test, FNA cytology• Chest X-ray, ultrasound, CT scans• Treatment: specifi c tuberculostatic chemotherapy regimens•

2.1.6.2. Actinomycosis

Cervicofacial actinomycosis is a chronic granulomatous • infection caused by Actinomyces israelii, a gram-positive, anaerobic bacillusPainless, indurated enlargement, mimicking a neoplasm, • associated with chronic purulent drainage (sulfur granules); development of multiple cutaneous draining fi stulas is typical15

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Anaerobic cultures, FNA cytology, ultrasound, CT scans• Treatment: long-term high-doses antibiotic treatment • (penicillin, clindamycin); surgical drainage of the collected pus

2.1.6.3. Cat-scratch disease (CSD)

CSD is a granulomatous infectious lymphadenitis, caused • by Bartonella henselae, a gram-negative bacillus; most commonly is the result of cutaneous inoculation through a scratch trauma from a domestic cat10

Typical history is of a papule or pustule at the scratch • site, followed in one or two weeks by the development of enlarged lymph nodes in the regions adjacent to the inoculation site Diagnosis is based on serologic testing and a specifi c • polymerase chain reaction (PCR)In most cases, no active therapy is required; the disease is • self-limited and usually will resolve spontaneously within few monthsAlthough generally prescribed, antibiotics do not shorten • the evolution of disease12

2.1.6.4. Toxoplasmosis

Toxoplasmosis is caused by the organism • Toxoplasma gondii, its usual host being the domestic cat10

A localized, lymphadenopathic and a systemic, • disseminated form are classically describedDiagnosis: lymph node biopsy, serologic testing• Treatment: chemotherapy regimens•

2.2. Noninfectious infl ammatory disorders

2.2.1. Sjogren’s syndrome (SS)


SS is a chronic autoimmune disease of the exocrine glands. Th e most commonly involved sites are the salivary and lacrimal glands. Th e disease is characterized by a diff use lymphocytic infi ltration, with subsequent glandular hypofunction, leading to

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dryness of the mouth and eyes. Th e disorder has a quite broad clinical presentation, ranging from glandular disease to systemic extraglandular involvement and to the development of lymphoid malignancies16. SS is classifi ed as primary (previously healthy person) or secondary, in association with other autoimmune disorders (rheumatoid arthritis, systemic lupus erythematosus)

2.2.1.2. Diagnosis

Dryness of the mouth (xerostomia), diffi culties in • chewing and swallowing food, voice problems, dryness of the noseDryness of the eyes (xerophtalmia), foreign body • sensations, keratoconjunctivitis siccaLocal examination: dry mucosal surfaces, multiple dental • caries, smooth tongue with fi ssures, Candida albicans infections, cheilitisSalivary gland enlargement, mainly in the parotid gland, • is reported in 10-20% of the cases17

Systemic involvement: • malaise, low-grade fever, myalgias, arthralgias, vasculitis, pulmonary, hepatic, renal involvementAssessment of salivary fl ow rate: sialochemistry• Assessment of tear secretion rate: Schirmer’s test• Minor salivary gland biopsy remains a highly specifi c test • for diagnosis of SS, although it is an invasive technique17

Assessment of immunological markers•

2.2.1.3. Treatment

Is mainly symptomatic• Saliva substitutes, chewing sugarless gums or candies, • treatment and prevention of dental caries and fungal infections, sialogogues (pilocarpine)Commercially available eye lubricants• Th e use of systemic steroids or cytotoxic drugs is • reserved for patients with severe systemic extraglandular involvement16

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2.2.2. Sarcoidosis

Sarcoidosis is a systemic disease of unknown aetiology, characterized by the presence of non-caseating epitheloid granulomas. It may aff ect any part of the body, but most frequently involves the lymph nodes, the skin, the lungs, the eyes and the liver. Salivary glands may also be involved, specifi cally the parotids. Symptoms include swellings and xerostomia. Diagnosis requires a biopsy, Kweim intradermoreaction test, pulmonary evaluation (chest X-rays, CT scans). Treatment: systemic corticosteroids. Heerfordt’s syndrome or uveoparotid fever (parotid swelling, uveitis and peripheral facial nerve palsy) is a rare form of sarcoidosis, occurring in young patients12.

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3. OBSTRUCTIVE DISORDERS

3.1. Sialolithiasis


Sialolithiasis is the formation of stones (calculi) at the level of the salivary glands ductal system. Submandibular gland is most commonly aff ected in 60% of the cases, followed by the parotid gland in 40%12. Calculi are composed mainly of calcium phosphate and carbonate, in combination with an organic matrix of glycoproteins and mucopolysaccharides18. No connection have been proven between calculi formation and blood levels of calcium or phosphate19. Th e exact pathogenesis remains uncertain. Salivary stasis and ductal infl ammation and injury contribute to calculi formation. Several factors might account for the higher incidence of calculi formation in the Wharton’s duct: this duct is longer than Stensen’s, has a larger diameter, is angulated against gravity as it crosses the mylohyoid muscle and submandibular saliva is more viscous and has a higher concentration of calcium20.

3.1.2. Diagnosis

Recurrent episodes of postprandial salivary colics (painful • swellings related to meals), which can remain transitory or be complicated by bacterial infectionBimanual palpation may reveal a palpable stone, mainly • in submandibular lithiasisPlain radiographs, conventional and digital sialography, • ultrasound, CT, MRISialendoscopy•

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3.1.3. Treatment


Local heat, adequate hydration, massage of the involved • glandSialogogues (pilocarpine)• Antibiotics and non-steroidal antiinfl ammatory drugs • (NSAIDs), if salivary gland infection is suspected


Palpable stones in the Wharton’s duct, located no more • than 2 cm from the orifi ce, can be removed using an intraoral approach, in local anaesthesia10

Submandibulectomy/parotidectomy• Modern approaches: interventional sialendoscopy, • external lithotripsy12

3.2. Other obstructive disorders

Strictures, stenosis: cause the same symptoms as salivary • stonesTumors: intraductal tumors are very rare; compressions • from adjacent extraductal tumors

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4. SALIVARY GLANDS TUMORS

4.1. Benign tumors

4.1.1. Defi nition. Aetiology. Epidemiology

Th e incidence of salivary glands tumors has been evaluated at 3-4/100,000 population, nearly 75% of these are benign tumors. Most of all develop in the parotid gland, and the vast majority are pleomorphic adenomas. Th ere is a slight female preponderance. All age groups are involved, with a higher incidence in the fourth decade of life21.

4.1.2. Symptoms and signs

Insidious onset, development of a slow-growing, painless • mass, with well-defi ned margins, in the glandular parenchymaSuperfi cial parotid tumors: painless, fi rm swelling in • the preauricular or retromandibular region, mobile over superfi cial and deep adjacent planesDeep lobe parotid tumors: are not accessible to external • palpation; they determine a medial displacement of the lateral oropharyngeal wall (oropharyngoscopy)Submandibular tumors: slow-growing, painless, mobile, • fi rm swellings within the submandibular triangleSublingual tumors: submucosal mass in the anterior fl oor • of the mouth, lateral to the lingual frenulum Minor salivary glands tumors: fi rm, slow-growing • submucosal masses, most commonly located at the level of the palate; ulcerations of the mucosa are rarely seen

Some benign tumors (pleomorphic adenomas) have the potential to transform into a malignant lesion, during evolution. Symptoms and signs suggesting the development of a malignancy are:

277

Rapid growth, sudden changes in mass size• Local pain• Loss of mobility• Facial nerve palsy• Skin invasion• Metastatic neck lymph nodes•

4.1.3. Diagnosis

History, inspection, palpation, complete ENT examination• Ultrasound, CT scans, MRI• FNA cytology: allows the preoperative diff erentiation • between a benign and a malignant lesion with 80-90% accuracy22

Frozen intraoperative sections•

4.1.4. Histopathology

4.1.4.1. Pleomorphic adenomas:

80% of pleomorphic adenomas are found in the parotid gland, 11% in the submandibular gland and a similar proportion in minor salivary glands (mainly in the palate and lips)21.

Figure 4 - Left parotid gland tumor

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On histologic examination, the tumor consists of an epithelial component, embedded in a fi brocollagenous, myxochondroid or chondroid background. Th e tumor has a thin capsule, and in one forth of the cases, satellite tumoral nodules and pseudopodia (extensions of tumoral tissue through the incomplete capsule, within the healthy adjacent parenchyma) have been reported23. Th is is the reason why the recommended surgical treatment is excision with a surrounding cuff of normal tissue, to avoid local recurrences24. Th e risk of malignant transformation is reported to vary between 1.5% (in the fi rst 5 years of evolution) and 10 % (after 15 years of evolution)25.

4.1.4.2. Whartin’s tumor:

It is the second most common benign salivary gland tumor; they account for nearly 10% of parotid tumors. It develops almost exclusively at the level of the parotid region (most commonly at the inferior pole of the superfi cial lobe), it has a slightly male predominance, bilateral lesions occur in 10% of the cases25. Th ey are usually ovoid encapsulated masses; papillary cysts are found on sectioning, fi lled with mucoid brown fl uid. It is suggested that these tumors are the result of a neoplastic proliferation of ectopic salivary duct gland ducts, within intraparotid lymph nodes. Th e absence of lymphoid tissue from other salivary glands parenchyma explains why these tumors develop only at the level of the parotid glands21.

4.1.4.3. Other benign tumors:

Oncocytoma• Myoepithelioma•

4.1.5. Treatment

Th e recommended European standard for surgical treatment26 is:

4.1.5.1. Parotid gland:

Lesion limited to the superfi cial lobe: superfi cial • parotidectomy, with facial nerve dissection and preservation

279

Lesion within the deep lobe: total conservative • parotidectomy, with facial nerve dissection and preservation

4.1.5.2. Submandibular gland and sublingual gland:

Submandibular gland excision• Sublingual gland excision•

Figure 5 - Facial nerve through the parotid gland

Figure 6 - Submandibular gland tumor (surgical specimen)

280

4.1.5.3. Minor salivary glands:

Wide excision of the lesion according to the location • site

All patients must be previously informed about the main complications of this surgery:

Parotid gland surgery: facial nerve paresis/palsy, Frey’s • syndrome (gustatory sweating or fl ushing), salivary cutaneous fi stulasSubmandibular gland surgery: damage to the mandibular • branch of the facial nerve, damage to the lingual or hypoglossal nerve


4.2.1. Defi nition. Aetiology. Epidemiology

Salivary glands malignancies account for 0.3-0.9% of all cancers and 1-3% of all head and neck cancers. Th e mean age is 55-60 years. Nearly 80% of these neoplasms develop in the parotid gland, 10% in the submandibular gland and 10% in the sublingual and minor salivary glands27. Th ere is some evidence that environmental factors such as radiation exposure, diet (polyunsaturated fatty acids seem to exert a benefi cial eff ect), viruses (EBV), certain occupational exposures (livestock feed processing) may elevate the risk for development of salivary gland cancer28.

4.2.2. Symptoms and signs

4.2.2.1. The parotid gland:

One in four parotid tumors are reported to be • malignant29

A rapid enlargement of a previously slow-growing, • painless, mobile swelling in the parotid regionFacial nerve palsy as a presenting symptom appears in • 1/3 of the patients; it is considered to be an independent prognostic indicator, its presence decreasing dramatically the survival rates30

Pain, skin infi ltration, metastatic intraparotid or neck •

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lymph nodes indicate advanced disease and a poorer prognosis

4.2.2.2. The submandibular gland:

40-66% of the submandibular tumors are reported to be • malignant28

A history of a painless, slow-growing, mobile mass in the • submandibular trianglePain, loss of mobility, skin invasion, metastatic lymph • nodes are suggesting malignancyWeakness or numbness of the tongue reveals invasion of • the hypoglossal and lingual nerves

4.2.2.3. Minor salivary glands:

Malignancies developed in minor salivary glands are • reported to vary between 10-50% of all salivary glands cancers28

Submucosal lumps and ulcerations• Rapid enlargement, pain, nerve palsies• Th e most common site is the palate, followed by the lips • and sinonasal tract

4.2.3. Diagnosis

History, inspection, palpation, complete ENT examination• Fine-needle aspiration cytology (ultrasound guided) is • the mainstay of early diagnosis, with a sensitivity of 90% and a specifi city of 100%31

Frozen sections, in cases where FNA is not available• Ultrasound examination• CT, MRI of the head and neck• Chest X-ray, CT, MRI of the chest and upper abdomen, • for distant metastasis assessment

4.2.4. Histopathology

Mucoepidermoid carcinoma: the most common malignant • tumor of the major salivary glands, 80% developing in the parotid27; they are subdivided in low-grade and high-grade tumorsAdenoid cystic carcinoma (cilindroma): nearly one third •

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of adenoid cystic carcinomas occur in major salivary glands, 11% have distant metastasis (lung, bone, liver) at presentation, they are subdivided in three subtypes (solid-poorer prognosis, cribriform and tubular), the disease has a very long natural history and a typical propensity for perineural invasion and spread28

Carcinoma ex pleomorphic adenoma: malignant • transformation of a pleomorphic adenoma is quoted in 3-12% of the cases, 75% occurring in the parotid gland27

Squamous cell carcinoma: primary (0.3-1.5%) or • secondary (invasion from adjacent sites)Acinic cell carcinoma (low-grade adenocarcinoma): best • survival ratesUndiff erentiated carcinoma• Non-Hodgkin lymphoma• Metastasis: skin cancers of the head and neck, lungs, • kidneys and breasts

4.2.5. Treatment


Adjuvant postoperative radiotherapy after surgery off ers superior outcomes than surgery alone. Clear indications for postoperative radiotherapy are: residual tumor, positive resection margins, high-grade tumors (high-grade mucoepidermoid carcinoma, squamous cell carcinoma, carcinoma ex pleomorphic adenoma, undiff erentiated carcinoma), perineural spread, neck node metastasis28. Radiotherapy alone or in association with chemotherapy is reserved for non-resectable advanced lesions.


Primary surgery, followed by postoperative radiotherapy in appropriate cases, is the best treatment modality. Safe, clear oncological margins are mandatory. In advanced disease, wide en-bloc resections are required, followed by various reconstructive techniques. Facial nerve involvement in the parotid malignancies requires the sacrifi ce of only the involved branches, followed by facial nerve reconstruction.

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Surgical treatment of the neck (selective, modifi ed, radical neck dissections) should be performed as a one-step procedure, together with the primary tumor ablation.

4.2.6. Prognosis

5-year survival range varies from 50-70%• 10-year survival range varies from 45-65%• Negative prognostic factors: stadialization, older age, • positive surgical margins27

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5. REFERENCES

1. Bradley PJ. Salivary gland anatomy. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 333-337.

2. Elluru RG, Kumar M. Physiology of the salivary glands. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1293-1312.

3. Edgar WM. Saliva: its secretion, composition and functions. British Dental Journal 1992; 172: 305-312.

4. Humphrey SP, Williamson RT. A review of saliva: normal composition, fl ow and function. Th e Journal of Prosthetic Dentistry 2001; 85: 162-169.

5. Bradley PJ. Saliva, salivation and functional testing. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 339-342.

6. Carlos J. Clinical examination and limited investigations of salivary gland diseases. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 343-347.

7. Gundlach P, Hopf J, Linnarz M. Introduction of a new diagnostic procedure: salivary duct endoscopy (sialendoscopy) clinical evaluation of sialendoscopy, sialography and X-ray imaging. Endoscopic Surgery and Allied Technologies 1994; 2: 294-296.

8. Koch M, Zenk J, Iro H. Algorithms for treatment of salivary gland obstructions. Otolaryngologic Clinics of North America 2009; 42: 1173-1192.

9. Perry RS. Recognition and management of acute suppurative parotitis. Clinical Pharmacy 1985; 4: 566-571.

10. Arrieta AJ, McCaff rey TV. Infl ammatory disorders of the salivary glands. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1323-1338.

11. O’Brien CJ, Murrant NJ. Surgical management of chronic parotitis. Head & Neck 1993; 15: 445-449.

12. Marchal F. Infl ammatory and non-infl ammatory aff ections of the salivary glands. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 351-367.

13. Leake D, Leake R. Neonatal suppurative parotitis. Pediatrics 1970; 46: 203.14. McAnally T. Parotitis: clinical presentation and management. Postgraduate

Medicine 1982; 71: 87-93.15. Burns BV, al-Ayoubi A, Ray J, Schofi eld JB, Shotton JC. Actinomycosis of

the posterior triangle: a case report and review of the literature. Th e Journal of Laryngology and Otology 1997; 111: 1082-1085.

16. Manoussakis M, Moutsopoulos M. Sjogren’s syndrome. Otolaryngologic Clinics of North America 1999; 32: 843-860.

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17. Caballero M. Sjogren’s syndrome and sialosis. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 369-373.

18. Hiraide F, Nomura Y. Th e fi ne surface structure and composition of salivary calculi. Th e Laryngoscope 1980; 90: 152-158.

19. Stanley MW, Bardales RH, Beneke J, Korourian S, Stern SJ. Sialolithiasis: diff erential diagnostic problems in fi ne-needle aspiration cytology. American Journal of Clinical Pathology 1996; 106: 229-233.

20. Williams MF. Sialolithiasis. Otolaryngologic Clinics of North America 1999; 32: 819-834.

21. Gleeson M, Cawson R. Benign salivary gland tumors. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2475-2492.

22. Cohen EG, Patel SG, Lin O et al. Fine-needle aspiration biopsy of salivary glands lesions in a selected patient population. Archives of Otolaryngology - Head & Neck Surgery 2004; 130: 773-778.

23. Stennert E, Guntinas-Lichius O, Klussmann JP, Arnold G. Histopathology of pleomorphic adenoma in the parotid gland: a prospective unselected series of 100 cases. Th e Laryngoscope 2001; 111: 2195-2200.

24. Witt RL. Th e signifi cance of the margin in parotid surgery for pleomorphic adenoma. Th e Laryngoscope 2002; 112: 2141-2154.

25. Hanna EY, Lee S, Fan CY, Suen JY. Benign neoplasms of the salivary glands. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1348-1377.

26. Lombardi D. Salivary glands: benign tumors. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 375-385.

27. Guntinas-Lichius O. Primary and secondary malignant salivary neoplasms. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 387-395.

28. Jones AS. Malignant tumors of the salivary glands. In: Hibbert J, ed. Scott-Brown’s Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2493-2521.

29. Gallia LJ, Johnson JT. Th e incidence of neoplastic versus infl ammatory disease in major salivary gland masses diagnosed by surgery. Th e Laryngoscope 1981; 91: 512-516.

30. Sullivan MJ, Breslin K, McClatchey KD, Ho L, Farrior EH, Krause KJ. Malignant parotid gland tumor: a retrospective study. Otolaryngology - Head and Neck Surgery: Offi cial Journal of American Academy of Otolaryngology - Head and Neck Surgery 1987; 97: 529-533.

31. Roland NJ, Caslin AW, Smith PA, Turnbull LS, Panarese A, Jones AS. Fine needle aspiration cytology of salivary gland lesions reported immediately in a head and neck clinic. Th e Journal of Laryngology and Otology 1993; 107: 1025-1028.

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VI. NECK BASICS

1. NECK BASICS

1.1. Surgical anatomy of the neck

1.1.1. Fascial layers

Th e neck has a superfi cial and a deep fascia1. Th e superfi cial fascia is very thin, adjacent to the overlying skin, and is of less importance. It invests the superfi cial platysma muscle. Th e deep fascia is divided into three layers, extends from the skull base to the upper mediastinum and forms important compartments in the neck. Th e deep fascia layers are:

Superfi cial or investing layer: invests the entire • neck, enveloping the trapezius, the omohyoid, the sternocleidomastoid, the anterior strap muscles and the parotid glandMiddle or visceral layer: encircles the trachea, the • oesophagus and the thyroid glandDeep or prevertebral layer: covers the deep muscles of the • neck and the prevertebral spaceCarotid sheath: is derived from the superfi cial layer of the • deep fascia, medial to the sternocleidomastoid muscle; it contains the carotid artery, the internal jugular vein, the vagus nerve and lymph nodes

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1.1.2. Triangles of the neck

Th e neck is classically divided into an anterior and a posterior triangle, separated by the sternocleidomastoid muscle2.

Figure 1. Neck fascial layers

I - External layer (investing, superfi cial); II - Middle layer (visceral); III - Carotid sheath; IV - Internal layer (prevertebral);1 - Trapezius muscle; 2 - Splenius muscle; 3 - Erector spinae muscle; 4 - Levator scapulae muscle; 5 - Spinal nerve (XI); 6 - Medial scalene muscle; 7 - Medial scalene muscle; 8 - Longus capitus muscle; 9 - Sternocleidomastoid muscle; 10 - Th yroid gland; 11 - Cervical oesophagus; 12 - Trachea; 13 - Common carotid artery; 14 - Vagus nerve (X); 15 - Internal jugular vein; 16 - Phrenic nerve; 17 - Cervical plexus (anterior division); 18 - Spinal nerve (XI); 19 - Posterior ramus of the spinal nerve

Figure 2. Neck triangles

1 - Submental triangle; 2 - Submandibular triangle; 3 - Supraomohyoid (carotid) triangle; 4 - Infraomohyoid triangle; 5 - Lateral neck triangle; 6 - Subclavian triangle

289

1.1.2.1. The anterior triangle

Th e anterior triangle is delineated by the anterior border of the sternocleidomastoid muscle, the inferior margin of the horizontal ramus of the mandible and the midline. It is further subdivided into the submental, submandibular, supraomohyoid (carotid) and infraomohyoid triangles. Th e submental triangle’s boundaries are the anterior belly of the digastric muscle, the hyoid bone and the midline. It contains lymph nodes. Th e submandibular triangle’s boundaries are the inferior margin of the horizontal ramus of the mandible and the two bellies of the digastric muscle. Th e submandibular gland and the adjacent lymph nodes lie within this space. Other important structures are the facial vein, the facial artery, the mandibular branch of the facial nerve (VII), the lingual and the hypoglossal nerve (XII). Th e triangle bordered by the anterior margin of the sternocleidomastoid muscle, the superior belly of the omohyoid muscle and the posterior belly of the digastric muscle is called the supraomohyoid or carotid triangle. Th e carotid sheath, incorporating the carotid artery, the internal jugular vein and the vagus nerve (X), runs through this space. Th e vagus nerve (X) runs within the carotid sheath, between the carotid artery and the internal jugular vein, on their posterior aspect. It gives an auricular branch (Arnold’s nerve), carotid body branches, pharyngeal branches, superior laryngeal branches and recurrent laryngeal branches. Th e accessory nerve (XI) crosses the upper part of the carotid triangle, as it enters the medial aspect of the sternocleidomastoid muscle. It supplies motor innervation to the sternocleidomastoid and the trapezius muscles. Th e hypoglossal nerve (XII) runs anteriorly to the carotid and passes medially below the posterior belly of the digastric muscle to supply motor innervation to the tongue muscles. Th e bifurcation of the common carotid artery lies also within this triangle. It is located nearly at the level of the greater cornu of the hyoid bone. Th e internal carotid ascends posteriorly to the external carotid, and has no collateral branches. Th e external carotid has 3 main anterior collateral branches: the superior thyroid, the facial and the lingual arteries. Th e surgical ligation

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of the external carotid should always be performed above the emergence of the superior thyroid artery. Th e lack of collateral branches of the internal carotid artery is a major surgical and anatomical criteria, in order to help the surgeon to diff erentiate the two carotids, during this procedure.

AnteriorSuperior thyroidLingualFacial

PosteriorOccipitalPosterior auricular

Deep Ascending pharyngeal

TerminalSuperfi cial temporalMaxillary

Table 1. Branches of the external carotid artery

Figure 3. Th e carotid artery

1 - Posterior auricular artery; 2 - Superfi cial temporal artery; 3 - Sternocleidomastoid muscle; 4 - Internal carotid artery; 5 - Ascending pharyngeal artery; 6 - External carotid artery; 7 - Common carotid artery; 8 - Omohyoid muscle; 9 - Superior thyroid artery; 10 - Hyoid bone; 11 - Lingual artery; 12 - Facial artery; 13 - Posterior belly of the digastric muscle; 14 - Maxillary artery;

291

Th e upper and middle jugular lymph nodes lie within this space, embedded in a fi brofatty tissue. Th e infraomohyoid triangle is delineated by the anterior border of the lower portion of the sternocleidomastoid muscle, the inferior border of the superior belly of the omohyoid muscle and the midline. It encompasses the lower carotid sheath, lower jugular lymph nodes, infrahyoid strap muscles, the upper aerodigestive tract and the thyroid gland.

1.1.2.2. The posterior triangle

Th e posterior triangle is delineated by the posterior border of the sternocleidomastoid muscle, the anterior border of the trapezius muscle and the clavicle. It is crossed by the omohyoid muscle, which further subdivides this triangle in the lateral neck and the subclavian triangles. Th e lateral neck triangle’s boundaries are the posterior border of the sternocleidomastoid muscle, the anterior border of the trapezius muscle and the superior border of the inferior belly of the omohyoid muscle. It contains the cervical plexus, fi brofatty tissue, lymph nodes and the spinal accessory nerve (XI). Th e Erb’s point is located on the posterior border of the sternocleidomastoid muscle, at the junction between the superior and the middle one third. It is the point where the cervical plexus (C2 and C3) emerges superfi cially and give rise to four cutaneous branches, supplying sensory innervation to the front and side of the neck, the external ear and the parotid region. Th e subclavian triangle’s boundaries are the lower border of the inferior belly of the omohyoid muscle, the posterior border of the lower portion of the sternocleidomastoid muscle and the clavicle. It contains fi brofatty tissue, lymph nodes, the scalene muscles, the brachial plexus and the subclavian vessels, including the thyrocervical trunk. Th e phrenic nerve is the most important motor branch of the cervical plexus (C4), which runs on the anterior aspect of the scalene muscles and supplies motor innervation to the ipsilateral diaphragm.

1.1.3. Cervical lymph nodes

1.1.3.1. Lymph node groups

Th e cervical lymphatics are divided into superfi cial and deep. Th e superfi cial ones perforate the cervical fascia and drain

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into the deep nodes. Th e deep lymphatics drain the mucosa of the upper aerodigestive tract. Th e deep nodes are divided into four major groups2:

Submental group: lies within the submental triangle/• space and drain the anterior fl oor of the mouthSubmandibular group: lies within the submandibular • triangle/space, related to the submandibular gland and facial vessels; cancers of the fl oor of the mouth, oral cavity and mobile tongue metastasize frequently in these nodesJugular chain: 80% of the neck lymph nodes are closely • related to the internal jugular vein; they are further divided into the superior jugular or jugulodigastric nodes (the fi rst echelon nodes for the drainage of the oropharynx, mainly the tonsillar region), the middle and the inferior jugular nodesPosterior group: lies within the posterior triangle and • are further classifi ed into two chains, one accompanying the spinal accessory nerve (XI), and a horizontal supraclavicular chain, related to the thyrocervical vessels; the spinal nodes are the fi rst echelon for the nasopharynx and the second echelon for the anterior neck nodes

1.1.3.2. Lymph node levels

Th e most widely accepted classifi cation of the neck lymph nodes was developed at Memorial Sloan Kettering Hospital, New York, and was adopted by the American Academy of

Figure 4. Neck lymph nodes levels

IA - Submental; IB - Submandibular; II - Upper jugular (IIA - inferior to the spinal nerve, IIB - superior to the spinal nerve); III - Middle jugular; IV - Lower jugular; V - Posterior triangle group (VA - superior to the inferior belly of the omohyoid muscle, VB - inferior to the inferior body of the omohyoid muscle); VI - Anterior, previsceral.

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Otolaryngology Head and Neck Surgery (AAOHNS) in 1991. Further improvements, including division in subzones, have been subsequently added2.

I (submental and submandibular nodes)

Ia (submental nodes)Ib (submandibular nodes)

anterior fl oor of the mouth, lower lip, ventral tongueoral cavity, anterior nasal cavities

II (upper jugular nodes)

IIa (anteroinferior to the course of the spinal accessory nerve)IIb (posterosuperior to the course of the spinal accessory nerve)

oropharynx, larynx, nasopharynx, hypopharynx and parotid gland

III (middle jugular nodes) larynx, pharynx

IV (lower jugular nodes) larynx, hypopharynx

V (posterior triangle group)

Va (superior to the inferior belly of the omohyoid muscle)Vb (inferior to the omohyoid muscle)

nasopharynxthyroid gland

VI (anterior, previsceral group)

prelaryngeal (Delphian nodes)paratrachealperithyroidal (along the recurrent laryngeal nerve)

larynxthyroid gland

Table 2. Lymph node levels of the neck


1.2.1. History

Examination begins with a detailed history taking. In patients with a primary complaint of a lump in the neck, a history of associated symptoms such as hoarseness, odynophagia, dysphagia, referred otalgia, nasal obstruction must be carefully evaluated. It is important to determine whether the lump is tender, before palpating the region.

1.2.2. Physical examination

Inspection of the neck from the front will notice the presence of any swelling, skin lesions, signs of local infl ammation, wounds, fi stulas, scars.

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Palpation of the neck is recommended to be performed while standing behind the patient, so that both sides can be examined simultaneously and compared. If the patient has an obvious neck lump, it is logical to start palpation at that site, but the rest of the neck should always be carefully palpated as well. When palpating the larynx, trachea, thyroid gland or midline lumps, it is recommended to ask the patient to swallow, in order to evaluate the active mobility of these midline structures. Palpation of the neck nodes will follow all the groups and levels described previously. Th e number, location, tenderness and mobility of the nodes will be assessed. Palpation of the neck is extremely important in the follow-up of the patients with head and neck malignancies. Normal anatomical structures in the neck which may be misdiagnosed during palpation include the carotid bifurcation, the transverse process of the atlas, the greater cornu of the hyoid bone and the submandibular gland3. A complete ENT examination must be performed. Panendoscopy of the upper aerodigestive tract is mandatory to rule out malignancies in patients suspected of lymph node metastases in the neck.

1.2.3. Imaging techniques

1.2.3.1. Ultrasound

High-resolution B-mode ultrasound is an inexpensive, reliable and rapid examination used to evaluate palpable abnormalities and guide biopsies4. It allows to diff erentiate solid from cystic masses, it is especially useful in congenital and developmental cysts, also in vascular lesions.

1.2.3.2. Computed tomography CT

Computed tomography is a quick, widely available and relatively cheap method of imaging the head and neck. It is excellent for evaluating bone destruction.

1.2.3.3. Magnetic resonance imaging MRI

MRI has superior soft tissue resolution with improved demonstration of tumor-muscle interfaces, compared to CT. Direct axial, coronal and sagittal sections are available. It is

295

superior to CT for imaging the skull base, oral cavity, salivary glands and nasopharynx. It is probably no better than CT in evaluation of lymph nodes and the larynx. No radiation is involved. Claustrophobia, the presence of cochlear implants, cardiac pace-makers, metal clips are contraindications to this technique3.

1.2.3.4. Positron emission tomography PET

PET scanning is a modern imaging technique that can map functional and metabolic activity before any structural changes occur. It is capable to diff erentiate malignant from normal tissue based on increased glycolysis or amino acid metabolism by tumor cells, therefore it can identify tumors in normal-sized structures. It appears to be the best method to assess recurrent tumors5. Another advantage is the possibility to perform whole-body scanning, to identify the presence of synchronous tumors, distant metastases and to evaluate the site of the primary tumor in neck metastasis of unknown origin6. A major limitation of this technique is the low-availability and the high expensive costs.

1.2.3.5. Arteriography

It is used to evaluate vascular lesions and masses fi xed to the carotid artery.


Fine needle aspiration (FNA) cytology (ultrasound • guided): the gold standard in diagnosis of neck massesEndoscopy and guided biopsies: to identify primary • tumor as source of a metastatic lymph nodeOpen biopsy: use only after work-up is complete and if • diagnosis is not evidentSkin tests: used when chronic or granulomatous • infl ammatory disorders are suspectedTh yroid gland assessment• Major salivary glands assessment•

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2. NECK MASSES

2.1. Congenital neck masses

2.1.1. Thyroglossal cysts


Th yroglossal cysts are the most common cystic lesions of the neck. Up to 50% will present in young adults, 30% are diagnosed during the fi rst decade of life7. Embryologically, the thyroid gland starts to develop at the level of the tongue base, then descends caudally to its fi nal location. Th e tract that is left usually disappears. Failure of the tract to involute may leave epithelial remnants, which may expand into a cystic lesion. Th e hyoid bone will entrap a portion of this remnant tract8.

2.1.1.2. Diagnosis

Midline, recurrent, painless, supralaryngeal cystic swellings, • adjacent to the hyoid bone, 1-3 cm in diameter Th e cyst ascends during deglutition or with tongue • protrusionCysts may become infected (enlargement, redness, • tenderness); fi stula formation with pus drainage may occur after infectionFNA cytology• Ultrasound examination• Scintigraphy, if an ectopic thyroid is suspected•

2.1.1.3. Treatment

Complete surgical excision is the treatment of choice. It is important to follow the cyst’s tract cranially, and to excise it together with the midportion of the hyoid bone, in order to avoid recurrences. Th e technique was fi rstly described by Sistrunk in 19209.

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Th ere have been some reports of thyroid carcinoma presenting within a thyroglossal tract cyst10.

2.1.2. Branchial cysts


Branchial cysts are lateral cervical cystic lesions. Th ey occur most commonly in young adults. Th ey are classifi ed as fi rst, second, third and fourth branchial cleft abnormalities. Th e second branchial cleft cysts are the most frequently encountered11. Six branchial or pharyngeal arches develop during the fourth week of intrauterine life. By the end of the fi fth week, the second arch grows over the third and the fourth to form the cervical sinus. Th is cervical sinus normally closes during the 6th week, leaving a cervical vesicle. Th is trapped ectoderm is considered to be the origin of branchial cysts development7.

2.1.2.2. Diagnosis

Cystic, oval mass, located anterior to the upper third of • the sternocleidomastoid muscleA recent upper airway tract infection is often reported• Swelling may be intermittent and occasionally local •

Figure 5 - Th yroglossal cyst

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infection occurs (pain, enlargement, erythema, fi stula formation)FNA citology• Ultrasound examination• CT/MRI•

Figure 6 - Branchial cyst

Figure 7 - Branchial cyst ultrasonography

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2.1.2.3. Treatment

Lateral cervical cysts should be surgically removed. Th e presence of local infection requires appropriate antibiotic treatment. Some authors estimated an incidence of malignant transformation (the so-called branchiogenic carcinoma) around 0.3% of all head and neck carcinomas12.

2.1.3. Dermoid cysts

Dermoid cysts develop from ectoderm and mesoderm, along lines of embryonic fusion of the facial processes, mainly in the midline or lateral to the submandibular gland. Th ey grow subcutaneously and contain skin appendages: hair follicles, sebaceous and sweat glands. Th ey must be diff erentiated from epidermoid cysts (epidermal inclusion cysts), which develop out of ectodermal tissue and do not include skin appendages. Surgical excision is the treatment of choice.

Figure 8 - Branchial cyst (intraoperative view)

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2.1.4. Lymphangiomas (cystic hygromas)


Lymphangiomas are benign congenital lymphatic lesions (abnormalities of the jugular lymphatic sac). One third of cases are found in the oral cavity and cheek and about a quarter in the neck. Th ey are divided in three categories7:

Capillary lymphangiomas: are comprised of capillary-like, • thin-walled lymphatic vessels, are usually asymptomatic, presenting as pale, small vesicle-like lesions confi ned to the superfi cial skin or oral cavityCavernous lymphangiomas: dilated lymphatic channels • typically occurring in the tongue, cheeks and lips, presenting as diff use swellingsCystic hygromas: are histologically similar to cavernous • lymphangiomas, presenting as larger cystic masses, which may communicate or be isolated; they develop mainly at the level of the posterior neck

2.1.4.2. Diagnosis

Soft, fl uctuant, cystic, diff use masses found most • commonly within the posterior triangle of the neck; as they grow they may involve the parotid area, the cheek, oral cavity, mediastinum, axillaAlmost always they are present at birth; they can be • diagnosed even prenatallyUsually asymptomatic (only cosmetic complaints)• Bleeding or infection may lead to rapid enlargement, • pain, even life-threatening upper airway obstruction13

FNA cytology• Ultrasound examination• MRI, CT scans•

2.1.4.3. Treatment

Surgery is the treatment of choice. In large lesions, resection is often limited to the neck. Great care must be taken to avoid damaging important vessels and nerves. Another therapeutic option is intralesional injection of sclerosing agents: bleomycin, tetracycline, alcohol, picinabil (OK-432)7.

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2.1.5. Hemangiomas


Like lymphangiomas, hemangiomas are usually considered congenital vascular abnormalities because they are present at birth or they appear within the fi rst months of life14. Histopathologically, these lesions are benign, but show proliferation of endothelial cells and mitoses. Th ree phases are described during their life cycle: proliferative (rapid growth from 2 weeks to 1 year), involuting (slow regression from 1 to 7 years) and involuted (complete regression after 8 years of age)11.

2.1.5.2. Diagnosis

Skin lesion or cystic mass with a bluish-purple appearance, • compressibility followed by refi llingA bruit and a thrill may be noticed• A change in size is seen when the child strains or cries• FNA, ultrasound, MRA (magnetic resonance angiography)•

2.1.5.3. Treatment

Observation: most of these lesions resolve spontaneously • Treatment is indicated if rapid growth occurs, with • pressure on adjacent structures; cosmetic considerations are also importantLasers (pulse-dye, argon, Nd-YAG)• 14

Systemic steroid therapy has been tried with various rates • of success

2.2. Infl ammatory/infectious neck masses

2.2.1. Acute and subacute infections

2.2.1.1. Acute viral and bacterial lymphadenitis

Acute lymphadenitis occurs in nearly every person at some point in life, especially during the fi rst decades15. Viral and bacterial infections of the upper aerodigestive tract cause a reaction in Waldeyer’s ring, followed by the involvement of the upper jugular lymph nodes. If the infection is restricted to the

302

teeth and oral cavity, submental and submandibular nodes are aff ected. Acute infl ammatory nodes are soft, mobile and tender. Infectious mononucleosis is typically associated with bilateral upper jugular and posterior triangle lymphadenopathies (100% of the cases).

2.2.1.2. Brucellosis

Brucellosis is a zoonotic infection transmitted from animals to humans by ingestion of infected food products, direct contact with an infected animal, or inhalation of aerosols. Brucellae are aerobic gram-negative coccobacilli that produce urease. An undulating fever, malaise and development of cervical infl ammatory lymphadenopathies in 20% of the patients is typical Diagnosis is confi rmed by serologic tests. Treatment is with doxycycline and rifampicin for six weeks7.

2.2.1.3. Kikuchi-Fujimoto disease

Kikuchi’s disease (subacute necrotizing lymphadenitis) is a histiocytic necrotizing adenitis without granular cell infi ltration, most commonly found in young women. Th e main complaints are fever, malaise and painless lymphadenopathies especially in the posterior triangle. Evolution is benign and resolves in some weeks or months. Excisional biopsy is required to rule out tuberculosis or lymphomas7.

2.2.1.4. Deep neck infections

Th e great majority are secondary to dental (40%) and oropharyngeal infections, foreign body ingestion, trauma with mandibular fractures. In up to 20% of the cases, no evident cause is revealed16. Most cultures are polymicrobial: Streptococcus viridans, Staphylococcus aureus, Staphylococcus epidermidis, anaerobes. Deep neck infections present as parapharyngeal, lateropharyngeal, retropharyngeal or submandibular space abscesses. Th e commonest complaints are pain, high fever, malaise and the presence of a diff use, tender, indurated swelling, most frequently located along the anterior border of the

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sternocleidomastoid muscle (latero- or parapharyngeal abscesses). If the infection is localized within the submandibular space, a signifi cant oedema of the mouth fl oor is associated (Ludwig’s angina). Deep neck infections are life-threatening conditions. Major complications include airway compromise, internal jugular vein thrombosis, mediastinitis. Evaluation is based on blood tests, ultrasound examination, CT/MRI, needle aspiration, cultures. High doses of wide-spectrum antibiotics must be administered immediately. Surgical drainage of the pus is recommended. Sometimes, in cases of airway compromise, intubation or tracheostomy is required.

2.2.1.5. Cervical necrotizing fasciitis

Cervical necrotizing fasciitis is a rare but serious life-threatening infection, characterized by a rapid, progressive necrosis of the subcutaneous fat and fasciae, and secondary necrosis of the overlying skin. It may develop primarily from an odontogenic or tonsillar infection, or during the evolution of a deep neck abscess. Streptococcus milleri, Streptococcus viridans and anaerobes are most frequently cultured7. Untreated, this condition may be rapidly fatal. High doses wide-spectrum antibiotics and surgical drainage and debridement are the recommended therapies.

2.2.2. HIV infection

Cervical lymph nodes hyperplasia is ubiquitous in patients with HIV infection, and nodal masses in these patients infrequently need to be considered for biopsy. Nearly 70% of AIDS cases develop this persistent generalized lymphadenopathy (PGL) within the fi rst months after seroconversion. If the excision is performed, histology will reveal a reactive follicular hyperplasia17. Enlarging or tender nodes are not typical for PGL. In these cases, nodal involvement may be the result of an opportunistic infection (tuberculosis, Nocardia species) or of a malignancy (non-Hodgkin lymphoma, Kaposi’s sarcoma, metastatic carcinoma)14.

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2.2.3. Chronic granulomatous disorders


Cervical lymphadenopathy is the most common head • and neck manifestation of tuberculosis; only 10-20 per cent of the patients have associated lung disease7

Nodes tend to be tender and sometimes may develop • an overlying erythema or a discharging sinus; they are found mainly in the upper jugular and posterior triangle groupsPPD skin tests, chest X-ray• Ultrasound examination• FNA cytology• Total excision with biopsy is recommended, associated • with specifi c chemotherapy regimens

2.2.3.2. Cat-scratch disease (CSD)

CSD is a granulomatous infectious lymphadenitis, caused • by Bartonella henselae, a gram-negative bacillus; most commonly is the result of cutaneous inoculation through a scratch trauma from a domestic catDiagnosis is based on serologic testing and a specifi c • polymerase chain reaction (PCR)In most cases, no active therapy is required; the disease is • self-limited and usually will resolve spontaneously within few monthsTh ere is some evidence that azithromycin is related with a • rapid resolution of the disease, but in most cases infection will settle without the need for antibiotic therapy18

2.2.3.3. Actinomycosis

Cervicofacial actinomycosis is a chronic granulomatous • infection caused by Actinomyces israelii, a facultative gram-positive, anaerobic bacillus, whose normal habitat is the oral cavityPainless, slow-growing, indurated masses, mimicking • a neoplasm, associated with chronic purulent drainage (sulphur granules); development of multiple cutaneous draining fi stulas is typical19

Anaerobic cultures, FNA, ultrasound, CT scans•

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Treatment: long-term high-doses antibiotic treatment • (penicillin, clindamycin); surgical drainage of the collected pus

2.2.3.4. Toxoplasmosis

Toxoplasmosis is a parasitic infection with • Toxoplasma gondii, the main reservoir being domestic cats; transmission may also occur from cysts in undercooked meat7

A localized, lymphadenopathic and a systemic, • disseminated form are classically describedDiagnosis: lymph node biopsy, serologic testing• Treatment: chemotherapy regimens•

2.3. Benign tumors

2.3.1. Paragangliomas

Paragangliomas are the most common vascular benign neoplasms of the neck. Th ey develop from extraadrenal paraganglionic cells derived from the neural crest (neuroectodermal origin). Historically, they were referred as glomus tumors, chemodectomas and non-chromaffi n tumors. Th e correct terminology is based on their location: carotid paragangliomas, jugulotympanic paragangliomas and vagal paragangliomas20. Most paragangliomas are solitary lesions. In 10% of the cases a syndrome of familial paragangliomas, characterized by multiple locations in the head and neck area, was reported21. Multiple paragangliomas associated with pheochromocytomas are also part of familial multiple endocrine neoplasia (MEN) syndromes. Paragangliomas of the head and neck are rarely vasoactive (1-3%), although they all contain neurosecretory granules22.

2.3.1.1. Carotid paragangliomas


Th e carotid body is a paraganglion located in the adventitia of the posteromedial aspect of the bifurcation of the carotid artery. It has a chemoreceptor role, modulating the respiratory and cardiovascular functions in response to changes in arterial pH, O

2 and CO

2.

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A higher incidence is reported in individuals living at altitudes greater than 2000 meters23. Malignant paragangliomas have been reported in 6% of all carotid body tumors24.


Th e most common presenting sign is a slow-growing, • painless, elastic or fi rm neck mass located at the bifurcation of the carotid arteryPalpation: the mass is mobile laterally, but not in vertical • plane; carotid pulsations may be transmitted through the massSometimes a bruit is audible by auscultation• Very large tumors are associated with dysphagia, • hoarseness and other cranial nerve defi citsCarotid sinus syndrome syncope (loss of consciousness, • bradycardia, hypertension) has been described in carotid body tumors25

Imaging techniques: ultrasound, Doppler ultrasound, • CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography


Surgical excision is the treatment of choice. Tumor removal requires subadventitial dissection. To minimize complications, or when plans of vascular reconstruction are taken into account, the assistance of an experienced vascular surgeon might be strongly recommended. Th e external carotid artery need to be ligated occasionally to gain adequate exposure. Every eff ort should be made to identify and preserve cranial nerves X, XI and XII. Radiation therapy is an alternative when surgery has major contraindications.

2.3.1.2. Vagal paragangliomas


Vagal paragangliomas are tumors developed from paraganglionic tissue associated with one of the ganglia of the vagus nerve26. Vagal paragangliomas most commonly arise from the inferior ganglion (nodose ganglion). Tumors developed from

307

the superior ganglion (jugular ganglion) may extend intracranially through the jugular foramen.


Palpable neck mass with lateral but no vertical mobility• Ipsilateral vocal fold paralysis with hoarseness and aspiration • of liquids secondary to inadequate glottic closureTumors of the jugular ganglion, at the skull base, may • extend intracranially and may be associated with other cranial nerves involvementImaging techniques: ultrasound, Doppler ultrasound, • CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography


Most are treated by surgical excision• Tumors arising from inferior ganglion are removed by • a transcervical approach, while the superior ganglion lesions require a combined transcervical-transmastoid approach Patients must be warned that complete surgical removal • may necessitate the sacrifi ce of the vagus nerve; the subsequent vocal fold paralysis will be managed postoperativelyRadiotherapy is reserved to major surgical contraindications •

2.3.1.3. Jugular paragangliomas


Jugular paragangliomas are classically divided in two categories: the glomus tympanicum tumor and the glomus jugulare tumor. Glomus tympanicum typically develops within the middle ear cavity from a branch of the glossopharyngeal nerve (tympanic branch of Jacobson) or the vagus nerve (posterior auricular branch of Arnold). Glomus jugulare tumors originate at the level of the jugular bulb20.


Pulsatile tinnitus, hearing loss, cranial neuropathies • involving cranial nerves IX, X, XI, XII

308

Otoscopic examination may reveal a vascular mass behind • the eardrumImaging techniques: ultrasound, Doppler ultrasound, • CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography


Surgical management requires a multidisciplinary team • including head and neck surgeons, neurosurgeons, neurootologists Radiation therapy, when surgical removal is not feasible•

2.3.2. Peripheral nerves tumors


Benign tumors of the head and neck arising from peripheral nerves include schwannomas and neurofi bromas. Schwannomas (neurilemmomas) are typically slow-growing, well-encapsulated tumors that arise from the Schwann cells of peripheral nerves. Most commonly, these are solitary lesions, but may be multiple and in 50% of the cases occur in the head and neck area27. Schwannomas may develop from cranial nerves, the sympathetic chain, the cervical and the brachial plexus. Neurofi bromas are benign nerve sheath tumors that may present as solitary lesions or as multiple tumor nodules as part of the autosomal dominant disorder von Recklinghausen disease (café au lait spots, multiple neurofi bromas, gliomas, spina bifi da, acoustic neuromas, pedunculated skin lesions). Unlike schwannomas, these are non-encapsulated tumors; cystic and degenerative changes are uncommon20. Malignant transformation of peripheral nerve tumors is rare.

2.3.2.2. Diagnosis

Most commonly appears as slow-growing lateral neck • mass; pain and neurologic dysfunction are unusualLesions arising from cranial nerves or sympathetic chain • may present as parapharyngeal space tumors, displacing the lateral pharyngeal wall mediallyImaging techniques: ultrasound, Doppler ultrasound, •

309

CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography

2.3.2.3. Treatment

Surgical excision is the treatment of choice.

2.3.3. Lipomas

Lipomas are benign tumors derived from adipose tissue. Th ey present as superfi cial, subcutaneous, soft, encapsulated, painless neck masses. Palpation gives the clinical diagnosis. Ultrasound and FNA cytology may be useful. Lipomas are best treated by complete surgical resection. Madelung’s disease (Lanois-Bensaude syndrome, benign symmetric lipomatosis, “fat neck”) is characterized by the excessive formation of multiple non-encapsulated lipomas with a symmetrical distribution at the level of the neck, supraclavicular fossa, shoulder and even upper mediastinum, with sparing of distal arms and leggs. Th e disease is largely more prevalent in middle-aged Mediterranean male subjects. A history of high ethanol intake (red wine) is frequently associated28.

Figure 9 - Madelung disease

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2.4.1. Primary malignant neck tumors

Most neck malignant lesions in adults are lymph node metastases from a carcinoma of the upper aerodigestive tract. Primary tumors are encountered less frequently. Th ey include:

Salivary gland carcinoma•

Figure 10 - Neck metastases

Figure 11 - Neck metastases

311

Th yroid gland carcinoma: papillary carcinoma, follicular • carcinoma, Hurthle cell carcinoma, medullary carcinoma, anaplastic carcinomaParathyroid carcinoma• Branchial cleft cyst carcinoma/thyroglossal duct cyst • carcinomaSarcomas• Cutaneous malignancies: basal cell carcinoma, squamous • cell carcinoma, malignant melanoma

2.4.2. Lymphomas

2.4.2.1. Defi nition. Classifi cation

Malignant lymphomas originate from the lymphatic system, which consists of a widespread tissue with complex maturation. Th e precursor cells are located in the bone marrow. Th e lymphoid stem cells diff erentiate into two distinct lineages of T- and B-cell types, respectively29. Malignant lymphomas were classically divided into Hodgkin’s and non-Hodgkin lymphomas. Th e latest 2008 World Health Organization (WHO) classifi cation30, separates the malignant lymphomas into immature and mature lymphoid neoplasms. Immature, precursor lymphoid neoplasms of T- and B-cell type (pre-acute B-/T-cell lymphoblastic leukaemia or lymphoma) frequently present with nodal involvement in children, but the clinical picture is dominated by the leukaemia. Mature lymphomas are divided into B-cell and T-/NK-cell neoplasms, and further subdivided into 30 and 18 subtypes, respectively. Th e most common B-cell lymphomas in Western countries are diff use large B-cell lymphoma (DLBCL) and follicular lymphoma (FL), followed by mucosa-associated lymphoma (MALT). Hodgkin’s lymphoma is considered a distinct entity, with B-cell origin. It arises almost exclusively in nodal tissue. Th e most frequent T-cell lymphomas are the peripheral T-cell lymphoma (PTCL), angioimmunoblastic lymphoma (AILT) and anaplastic large-cell lymphoma (ALCL)29.

312

2.4.2.2. Diagnosis

Th e ENT area is a common site of presentation in malignant lymphomas. Th is region has a dense and well-developed lymphatic tissue. Extranodal involvement may be found in the Waldeyer’s ring, salivary glands, oral cavity, thyroid gland, nasal cavities and paranasal sinuses, larynx. Neck nodes involvement can occur as a part of a generalized lymphadenopathy or isolated in almost all cases of Hodgkin’s and non-Hodgkin lymphomas (painless, fi rm, multiple nodes). Th e gold standard diagnostic procedure is an open biopsy. Th is method should supply enough material for an extensive pathological work-up with morphology, immunohistochemistry, fl ow cytometry and ancillary techniques (cytogenetics, molecular genetics and gene-expression arrays)31. Stage of the disease is assessed according to the Ann Arbor staging system. For an accurate stadialization, these routine procedures must be performed32:

Imaging techniques: CT, MRI, PET of the head and • neck, thorax and abdomenBone marrow biopsy and aspiration • Hematology, blood chemistry•

Th e presence (B) or absence (A) of general symptoms (drenching night sweats, fever, 10% or more weight loss within three months) must be evaluated.

2.4.2.3. Treatment

Surgery has its role only as a diagnostic procedure. Chemotherapy is the treatment of choice in all non-Hodgkin lymphomas. In B-cell subtypes addition of antibodies against B-cells (anti-CD20, rituximab) to chemotherapy regimens increased cure rates with 10-15%29. In Hodgkin’s lymphomas stage I and II, radiotherapy is still mandatory as consolidation after chemotherapy. Th e site of disease has little importance for choice of treatment, the decisive factors are the histology and the stage of the disease.

313

2.4.3. Neck metastases

2.4.3.1. Neck metastases with known primary tumor

In the greatest majority of cases, metastatic neck disease is secondary to a carcinoma of the upper aerodigestive tract. It is important not to overlook the possibility of metastatic disease from other head and neck sites (the skin of the scalp, ear, face and neck, the major and minor salivary glands, the thyroid gland) or from distant sites (lungs, stomach, kidneys, prostate, ovaries). Th e probability of lymphatic spread of carcinomas in the head and neck area is related to the abundance of capillary lymphatics at the level of the primary site. Th e greatest density of lymphatic capillary networks belongs to the nasopharynx, tongue, tonsils and hypopharynx. Th us, the rate of metastases from these sites is higher when compared to other sites: larynx or paranasal sinuses33. Tumors located in midline areas (base of tongue, posterior pharyngeal wall, supraglottis) may present with bilateral nodal involvement. Th e neck mass in a patient with known primary tumor should be managed according to the principles described for each primary site.

2.4.3.2. Neck metastases with unknown primary tumor


Th e presence of neck lymph nodes metastases without clinical evidence of a primary tumor is an unusual, but not rare, condition. It accounts for nearly 2-3% of head and neck malignancies. Metastases develop mainly at nodal levels II and III, with less frequent involvement of levels I, IV, V and VI. Squamous cell carcinoma is the most common histological fi nding34.


When a patient presents with an isolated neck mass, a detailed and accurate clinical history and examination is essential. A complete and repeated full ENT examination, including offi ce endoscopy (fl exible or rigid) of the upper aerodigestive

314

tract, with particular attention to the nasopharynx, tongue base and tonsils, must be performed. Additionally, the skin in the head and neck region, including the scalp should be carefully assessed, together with the external ear canal, pre- and retroauricular areas. Fine needle aspiration (FNA) cytology from the abnormal neck lymph node off ers clear indications of the pathological nature of the lesion. Th e only tumor marker of clinical value is Epstein-Barr virus (EBV) serology. Microamounts of tissue obtained by FNA have also been studied to detect the EBV in neck metastases, using PCR (polymerase chain reaction)35. Positive outcomes should recommend multiple biopsies from the nasopharynx. Imaging techniques (CT scans, MRI, PET scans of the head and neck) should be performed before biopsy, in order to assist in directing subsequent biopsies and to avoid false-positive results determined by local swelling and infl ammation induced after the tissue specimen is excised34. Th yroid gland and major salivary glands must be carefully assessed. Chest and abdominal images are helpful, mainly if the neck mass is located in the supraclavicular region. Direct panendoscopy of the upper aerodigestive tract, under general anaesthesia, is the next step. If no evident primary tumor is visible, guided biopsies should be performed from the most logical sites for the occult primary lesion, based on known lymphatic drainage. Th ese areas are the nasopharynx (Rosenmuller fossa), the base of tongue, the tonsil and the pyriform sinus14. Ipsilateral or even bilateral tonsillectomy, instead of a simple incisional biopsy, is recommended in these cases, because up to 25% of occult tumors are found at this site36, 37. Open excisional biopsy of the metastatic lymph node is performed only after workup is complete and no primary tumor is found. If the specimen for histologic frozen section reveals a metastatic squamous cell carcinoma, a defi nitive neck dissection should be performed during the same intervention. Th erefore, the patient must be counselled before undertaking the open biopsy about the possibility of a concomitant neck dissection14. Th ere is no clear evidence in the literature that an open biopsy alters the prognosis, as long as appropriate treatment is initiated within 6 weeks38.

315


Despite extensive and accurate investigations, in nearly 60% of the cases, the primary tumor is not revealed. Controversies in the management of these patients range from types of neck dissections to be performed, fi elds of radiotherapy (ipsilateral neck only, whole neck, panmucosal), the role of chemotherapy. A defi nitive modifi ed radical neck dissection should be performed in all patients with positive metastatic carcinoma after FNA or open excisional biopsy34. Radiotherapy is recommended after surgery. Th ere is evidence to support the use of radiotherapy alone for management of the neck following excisional biopsy, without neck dissection, with 88-100% control rates39,40. Panmucosal radiotherapy has demonstrated a decrease in primary site occurrence, with no improvement in overall survival34. Benefi t of chemotherapy and chemoradiotherapy is still under investigation. Nodal stage is the most important risk factor for local control.

2.4.4. Neck dissection

Th is surgical procedure dates back to 1906, when George Crile described for the fi rst time the classical radical neck dissection. Subsequently, Hayes Martin was a strong proponent of the radical en bloc technique, which was similar to the radical surgery that had evolved for breast cancer. During that period, radiotherapy had not been developed as an eff ective adjuvant therapy, and radical surgery was the only hope for survival. Th e signifi cant morbidity of the radical procedure and the further development of radiotherapy led to an interest in more conservative approaches. Th e following neck dissections are now defi ned41:

Classic radical neck dissection• Modifi ed radical neck dissection (types 1-3)• Selective neck dissection• Extended radical neck dissection•

316

2.4.4.1. Radical neck dissection

Th is procedure removes lymph nodes from level I-V, and all three non-lymphatic structures: the internal jugular vein, the spinal accessory nerve and the sternocleidomastoid muscle.

2.4.4.2. Modifi ed radical neck dissection

Th e operation consists of lymph node removal from level I-V, with preservation of one or more non-lymphatic structures:

Type 1: preservation of the spinal accessory nerve• Type 2: preservation of the spinal accessory nerve and the • internal jugular veinType 3: preservation of the spinal accessory nerve, the • internal jugular vein and the sternocleidomastoid muscle

When all 3 non-lymphatic structures are preserved, the neck dissection is also called functional or comprehensive. Th e principles of this functional procedure were presented by Suarez in 1963, and subsequently popularized by Ettore Bocca42.

2.4.4.3. Selective neck dissection

Selective neck dissection involves the removal of selected

Figure 12 - Radical neck dissection

1 - Common carotid artery; 2 - Vagus nerve; 3 - Internal carotid artery; 4 - Parotid gland; 5 - Horizontal ramus of the mandible

317

lymph node levels (see Table 2), with preservation of the 3 non-lymphatic structures1:

Selective neck dissection levels I-III (formerly called • supraomohyoid neck dissection): most commonly used in cancers of the mobile tongue, fl oor of the mouthSelective neck dissection levels I-IV (formerly called • anterolateral neck dissection): mobile tongue, fl oor of the mouthSelective neck dissection levels II-IV (formerly called • lateral neck dissection): oropharyngeal, hypopharyngeal and laryngeal cancerSelective neck dissection levels II-V (formerly called • posterolateral neck dissection): nasopharyngeal cancer, melanoma of the scalp, sometimes oral and hypopharingeal cancerSelective neck dissection level VI (formerly called anterior • or central neck dissection): thyroid cancer, subglottic carcinoma

Selective neck dissections are only indicated in the N0 neck, when the natural history of the disease is predictable. It’s not recommended to be performed following previous surgery or radiotherapy41.

2.4.4.4. Extended radical neck dissection

Besides the removal of all the structures described in a radical neck dissection, this procedure involves resection of additional lymph node groups or non-lymphatic structures (retropharyngeal, parapharyngeal, occipital, paratracheal or parotid nodes, parotid gland, mandible, mastoid tip, carotid artery, skin).

Nx Regional lymph nodes cannot be assessed

N0 No regional lymph nodes metastases

N1 Single ipsilateral lymph node, 3 cm or less in greatest dimension

N2a Single ipsilateral lymph node, 3-6 cm in greatest dimension

N2b Multiple ipsilateral lymph node, none more than 6 cm in greatest dimension

N2c Bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension

N3 Lymph node more than 6 cm in greatest dimension

Table 3. TNM classifi cation of regional neck lymph nodes

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