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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4518_Библиотеки_им_академика_М_И_Перельмана
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16 The Facial Nerve andLateral Skull Base Disorders
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– Fisch and Mattox glomus staging system
Includes both glomus tympanicum and jugulare
Type A involves promontory only
Type B involves hypotympanum but no erosion over jugular bulb
Type C erodes bone over jugular bulb
• C1 erodes carotid foramen, C2 involves vertical carotid canal, C3 extension to horizontal carotid canal but not through foramen lacerum, C4
involves entire petrous carotid with extension to cavernous sinus
Type D intracranial extension
• De extradural, Di intradural
• D1 <2cm intracranial, D2 >2cm intracranial, D3 unresectable
– Glasscock–Jackson staging system
Glomus tympanicum
• I: Small mass on promontory
• II: Completely lls middle ear
• III: Fills middle ear and extends to mastoid
• IV: Fills middle ear, extends to external auditory canal
Glomus jugulare
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• I: Involves jugular bulb, middle ear, and mastoid
• II: Extends under internal auditory canal
• III: Extends to petrous apex
• IV: Extends to clivus or infratemporal fossa
• Types II–IV may have intracranial extension
– Treatment
Glomus tympanicum: Transcanal approach for small tumors, tympanomastoidectomy with extended facial recess for larger tumors
Glomus jugulare
• May observe if not growing, older patient
• Surgery favored for younger patients with + cranial nerve decits
• Primary radiotherapy (stereotactic or external beam radiotherapy) can
arrest tumor growth
• Recent trend is toward radiotherapy with/without subtotal resection to
spare functioning cranial nerves
• Multiple surgical approaches described
– Transmastoid jugular foramen approach with extended facial recess
– Add canal wall down mastoidectomy and external auditory canal
overclosure to increase anterior exposure near carotid
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– Can anteriorly reroute facial nerve to further increase exposure
(Fisch type A infratemporal fossa approach); causes some degree of
permanent facial weakness
– All approaches need neck dissection for proximal internal carotid
artery and internal jugular vein control
– Preoperative embolization may decrease tumor blood ow; embolize
external carotid artery branches commonly including ascending pharyngeal artery
• Prognosis: favorable control rates reported with primary surgery or
radiation therapy
• Facial nerve tumors
– Schwannoma: Most common facial nerve tumor; can affect any portion of
nerve, some series have reported highest incidence in perigeniculate region
– Hemangioma: Very rare, usually involves perigeniculate region
– Clinical: Slowly progressive facial twitching and/or weakness; hearing loss in
50%, can be conductive or sensorineural depending on tumor location
– Imaging: CT shows smooth expansile fallopian canal mass, hemangioma may
show bony spicules; MRI with enhancement on T1 with contrast for both
schwannoma and hemangioma
– Management
S. Pelosi
Observe until patient’s facial nerve function reaches House–Brackmann
grade 3 or 4, or if large cerebellopontine angle component that is compressing brainstem
Consider middle fossa decompression once House–Brackmann grade 3 to
give tumor more room to grow into middle fossa and decrease facial nerve
compression
Surgical resection with interpositional graft once worse than House–
Brackmann grade 3
• Translabyrinthine route can access the entire extent of facial nerve, but
sacrices hearing
• Middle fossa approach for hemangioma involving perigeniculate
region; some authors describe peeling of small hemangiomas off
facial nerve
Stereotactic radiotherapy may be considered for growing tumor but still
with good (House–Brackmann grade 1–3) facial function
• Differential diagnosis of cerebellopontine angle masses
– Schwannoma: (Vestibular 80%, facial 1%) T1/T2 isointense, enhances on T1
with contrast, greater internal auditory canal involvement
– Meningioma (3%) T1/T2 isointense, enhances on T1 with contrast, dural tail,
often eccentric to internal auditory canal, hyperostosis at base on CT
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– Epidermoid (2%) T1 hypointense, T2 hyperintense (high uid content), does
not enhance with contrast, has a high signal (restricted diffusion) on diffusionweighted imaging
– Paraganglioma: T1/T2 heterogenous hyper/hypointense foci, enhances on T1
with gadolinium “salt and pepper” appearance from ow voids
– Arachnoid cyst: T1 hypointense, T2 hyperintense, does not enhance, low sig-
nal (limited restriction) on diffusion-weighted imaging
– Metastatic tumors: T1/T2 isointense focal meningeal thickening, T1 with
contrast bilateral linear or nodular meningeal enhancement
– Endolymphatic sac tumor: T1 and T2 hyperintense, centered in the retrolaby-
rinthine presigmoid space; enhances with contrast
– Lipoma: T1 hyperintense, T2 hypointense, does not enhance and has signal
suppression with fat saturation technique
• Vestibular schwannoma
– Most common cerebellopontine angle tumor
– Very rare malignant degeneration, typically sporadic and unilateral except
when associated with neurobromatosis type 2
– Neurobromatosis type 2
Autosomal dominant
Diagnosis requires one of the following: bilateral vestibular schwannomas,
unilateral vestibular schwannoma with a family history of neurobromatosis type 2, or multiple brain tumors (schwannoma, meningioma, glioma)
with a family history of neurobromatosis type 2
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– Clinical ndings: Asymmetric hearing loss, tinnitus, disequilibrium (less
common since slow growth allows for vestibular compensation), headache if
large tumor size
– Audiometry: Asymmetric sensorineural hearing loss, word discrimination
may be disproportionately worse than pure tone thresholds; rollover (increased
sound intensity results in decreased word discrimination), tone decay (sus-
tained signal with decreased perception)
– Balance function testing: Unilateral caloric weakness
– Auditory brainstem response: May have prolonged wave I–III, I–V, III–V
latencies, not as sensitive as MRI
– Imaging
MRI: T1 with contrast shows homogenous enhancing mass in internal
auditory canal and cerebellopontine angle
CT with contrast may miss smaller tumors
– Histology: Antoni A: cells with parallel palisading nuclei; Antoni B: histo-
logically less uniform
– Management
Observe non-growing tumors (~40%)
For growing tumor, options include surgery or radiotherapy
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S. Pelosi
Stereotactic radiotherapy
• Gamma-knife: Single fraction (commonly 12–14Gy at tumor margin)
given using stereotactic frame rigidly xed to patient’s head
• Linear accelerator (LINAC, includes cyber-knife): Nonrigid imageguided system used to give stereotactic radiotherapy in hypofractionated doses
• Goal is to prevent tumor growth
• Better for older patients with multiple comorbidities
• Intensity-modulated radiotherapy (non-stereotactic) has also been used
with hypofractionated treatment schedules
Surgery
• Preferred for younger healthy patients, larger tumors
• Surgical approaches
– Translabyrinthine: Most direct access route to internal auditory canal
and provides consistent facial nerve identication distally, avoids
cerebellar retraction, sacrices hearing
– Retrosigmoid: Potential for hearing preservation but increased cere-
bellar retraction, potentially higher risk of headaches
– Middle fossa: Potential for hearing preservation but limited to intra-
canalicular tumors; higher risk of facial nerve paresis
Outcomes
• Increased tumor size associated with decreased rates of facial nerve
function and hearing preservation
• Cerebrospinal uid leak risk 10% (similar across approaches)
• Stereotactic radiotherapy often results in delayed sensorineural hearing
loss; overall long-term outcomes similar to surgery
Management in neurobromatosis type 2 patient
• Screen relatives with MRI
• Remove larger tumor with less hearing rst
• Consider placement of auditory brainstem implant at the time of initial
surgery if cochlear nerve sacriced (generally gives poor hearing
results)
• If able to preserve cochlear nerve at the time of surgery, consider
cochlear implant
• Observe the only-hearing ear with serial MRI
• Bevacizumab (Avastin)
– Anti-VEGF monoclonal antibody
– Can stop tumor growth/hearing loss progression in NF2 patients
– Option for NF2 patients with progressive bilateral vestibular schwan-
noma (growth and/or worsening SNHL)
– High incidence of toxicity/adverse events (GI perforation, bleeding,
poor wound healing)
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• Endolymphatic sac tumor
– Association with von-Hippel–Lindau syndrome (seen in 10–30% of VHL
patients)
– Locally destructive and capable of intracranial seeding but does not metasta-
size distantly
– Imaging: Presigmoid retrolabyrinthine lesion, posterior to internal auditory
canal; T1 hyperintense foci within tumor, T2 heterogenous signal, T1 with
contrast reveals heterogenous enhancement
– Histology shows papillary features
– Management: Surgery with or without radiotherapy
• Differential diagnosis of petrous apex masses
– Cholesterol granuloma: T1 and T2 hyperintense from both high uid and fat
(cholesterol crystal) content, does not enhance
– Asymmetric marrow: T1 hyperintense, does not enhance
– Effusion/trapped uid: CT shows air cell septations present; T1 hypointense,
does not enhance; T2 hyperintense
– Petrous apicitis: CT shows air cell coalescence; T1 intermediate signal, may
have enhancing ring with abscess; T2 high signal
– Aneurysm (carotid): Smoothly marginated bone-eroding lesion in the region
of carotid canal, may have thrombus making contrast-enhanced scans appear
heterogenous
– Chondrosarcoma: Presents with headache and diplopia; CT shows irregular
bone destruction, may have “popcorn” calcications; enhances on T1 with
contrast; treatment is surgical resection; radiotherapy (proton beam) may be
of benet in cases of subtotal resection, recurrent tumor
– Chordoma: Usually midline with extension from clivus to petrous apex; CT
shows destructive lesion with calcication foci, enhances on T1 with contrast,
may be difcult to differentiate from chondrosarcoma
– Schwannoma (trigeminal): T1 with contrast demonstrates homogenous
enhancement in the region of trigeminal (Gasserian) ganglion
– Metastasis
– Cholesterol granuloma
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Pathogenesis: Obstruction of air cell drainage pathways resulting in inammation/hemorrhage, red blood cell breakdown, and foreign body reaction
to cholesterol crystals
Slowly expansive
Clinical ndings: Usually asymptomatic, may expand to compress cranial
nerves (CN VI), brainstem, or temporal bone structures
Imaging: MRI T1/T2 hyperintense, no enhancement with contrast
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Management
• Observe if not causing symptoms
• Surgical decompression for cranial neuropathies, brainstem compression
– Transnasal approaches provide the widest access for lesions with
extension medial to carotid
– Lateral approaches
Infracochlear and infralabyrinthine affords only narrow access,
but provides route for aeration and drainage through connection
with middle ear/mastoid
Middle fossa approach does not provide a route for drainage or
aeration
Translabyrinthine approach provides the widest exposure, but
does not preserve hearing
• Differential diagnosis of diffuse temporal bone/skull base lesions
– Fibrous dysplasia: Usually monostotic, progressive external auditory canal
occlusion with conductive hearing loss, rarely causes sensorineural hearing
loss, temporal bone with uniform “ground glass” appearance; treatment is
generally observation, may consider canalplasty for conductive hearing loss
or cholesteatoma formation behind canal stenosis
– Paget’s disease (see causes of conductive hearing loss)
– Eosinophilic granuloma
S. Pelosi
Mildest form of Langerhans cell histiocytosis
Affects older children and young adults
Typically affects mastoid, external auditory canal, petrous apex; may
involve entire temporal bone
Presents as painful postauricular swelling, or with granulation and otorrhea of external auditory canal
CT shows areas of bony destruction; MRI T1 with contrast shows
enhancement
Treatment
• Conservative surgical excision
• Low-dose radiotherapy
– Rhabdomyosarcoma: Most common temporal bone malignancy of children;
affects middle ear/mastoid, presents with chronic otalgia/otorrhea; treat with
chemotherapy+surgery/radiotherapy depending on group and stage
– Osteopetrosis (Albers–Schonberg disease): Symmetrical increase in bone
density, narrows internal auditory canal and causes sensorineural hearing loss,
narrows fallopian canal and causes facial weakness/paralysis, spares otic cap-
sule; middle fossa decompression of facial nerve may be of benet
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327
Further Reading
1. Arriaga M, Curtin H, Takahashi H, Hirsch BE, Kamerer DB.Staging proposal for external
auditory meatus carcinoma based on preoperative clinical examination and computed tomography ndings. Ann Otol Rhinol Laryngol. 1990;99:714–21.
2. Baugh RF, Bausra GJ, Ishii LE, etal. Clinical practice guideline: Bell’s palsy. Otolaryngol
Head Neck Surg. 2013;149:S1–27.
3. Benecke JE Jr. Management of osteomyelitis of the skull base. Laryngoscope. 1989;99:1220–3.
4. Bennett M, Haynes DS.Surgical approaches and complications in the removal of vestibular
schwannomas. Otolaryngol Clin North Am. 2007;40(589–609):ix–x.
5. Brodie HA, Thompson TC.Management of complications from 820 temporal bone fractures.
Am J Otol. 1997;18:188–97.
6. Fisch U, Mattox D.Classication of glomus temporal tumors. In: Fisch U, Mattox D, editors.
Microsurgery of the skull base. Stuttgart: Thieme; 1988. p.149–53.
7. Gantz BJ, Rubinstein JT, Gidley P, Woodworth GG. Surgical management of Bell’s palsy.
Laryngoscope. 1999;109:1177–88.
8. Gottfried ON, Liu JK, Couldwell WT.Comparison of radiosurgery and conventional surgery
for the treatment of glomus jugulare tumors. Neurosurg Focus. 2004;17:E4.
9. Gronseth GS, Paduga R. Evidence-based guideline update: steroids and antivirals for Bell
palsy: report of the Guideline Development Subcommittee of the American Academy of
Neurology. Neurology. 2012;79(22):2209–13.
10. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg.
1985;93:146–7.
11. Jackson CG, Glasscock ME III, Harris PF.Glomus tumors. Diagnosis, classication, and management of large lesions. Arch Otolaryngol. 1982;108:401–10.
12. Limb CJNJ.The acute facial palsies. In: Jackler RK, Brackmann DE, editors. Neurotology.
Maryland Heights: Mosby; 2004. p.1230–57.
13. Ling SS, Sader C.Fungal malignant otitis externa treated with hyperbaric oxygen. Int J Infect
Dis. 2008;12:550–2.
14. Lockhart P, Daly F, Pitkethly M, Comerford N, Sullivan F.Antiviral treatment for Bell’s palsy
(idiopathic facial paralysis). Cochrane Database Syst Rev. 2009;(11):CD001869.
15. Lu VM, Ravindran K, Graffeo CS, etal. Efcacy and safety of bevacizumab for vestibular
schwannoma in neurobromatosis type 2: a systematic review and meta-analysis of treatment
outcomes. J Neurooncol. 2019;144:239–48.
16. McKennan KX, Chole RA. Facial paralysis in temporal bone trauma. Am J Otol.
1992;13:167–72.
17. Mulder JJ, Kaanders JH, van Overbeeke JJ, Cremers CW.Radiation therapy for vestibular
schwannomas. Curr Opin Otolaryngol Head Neck Surg. 2012;20:367–71.
18. Ridgway JM, Crumley RL, Kim JH.Rehabilitation of facial paralysis. In: Flint PW, Haughey
BH, Lund VJ, Niparko JK, Richardson MA, Robbins KT, Thomas JR, editors. Flint: Cummings
otolaryngology: head & neck surgery. Maryland Heights: Mosby; 2010.
19. Rosenfeld RM, Schwartz SR, Cannon CR, et al. Clinical practice guideline: acute otitis
externa. Otolaryngol Head Neck Surg. 2014;150:S1–24.
20. Schwartz SR, Magit AE, Rosenfeld RM, etal. Clinical practice guideline (update): earwax
(cerumen impaction) executive summary. Otolaryngol Head Neck Surg. 2017;156:S1–S29.
21. Semaan MT, Megerian CA.Current assessment and management of glomus tumors. Curr Opin
Otolaryngol Head Neck Surg. 2008;16:420–6.
22. Sunderland S.A classication of peripheral nerve injuries producing loss of function. Brain.
1951;74:491–516.
23. Wilkinson EP, Hoa M, Slattery WH III, etal. Evolution in the management of facial nerve
schwannoma. Laryngoscope. 2011;121:2065–74.
Part V
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Head and Neck
Chapter 17
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Thyroid andParathyroid Diseases
NoelM.Phan, AnthonyDel Signore, BrettA.Miles, andMohemmedKhan
Pearls
• Stage affected by age (≥55)
• Cervical metastatic disease, very common in papillary thyroid cancer, does not
usually affect prognosis
• Thyroid malignancy is usually treated surgically
• Key to successful parathyroid adenoma is accurate preoperative localization
• Intraoperative parathyroid hormone (PTH) assay is used at most centers to verify
adequate treatment
N. M. Phan (*) M. Khan
Department of Otolaryngology - Head and Neck Surgery, Icahn School of Medicine at the
Mount Sinai Hospital, New York, NY, USA
e-mail: noel.phan@mountsinai.org; mohemmed.khan@mountsinai.org
A. Del Signore
Department of Otolaryngology, Icahn School of Medicine at the Mount Sinai Hospital,
New York, NY, USA
e-mail: Anthony.delsignore@mountsinai.org
B. A. Miles
Otolaryngology Head and Neck Surgery, Oral and Maxillofacial Surgery, Northwell Health
System, New Hyde Park, NY, USA
e-mail: bmiles4@northwell.edu
© Springer Nature Switzerland AG 2023
F. Y. Lin, Z. M. Patel (eds.), ENT Board Prep,
https://doi.org/10.1007/978-3-031-26048-3_17
331
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N. M. Phan et al.
Thyroid
Embryology
• Development begins between the 2nd and 11th week of gestation
• Three pharyngeal bodies: the median anlage and two lateral bodies
• Median anlage is derived from invagination of endoderm at foramen cecum
• The descent occurs along the thyroglossal duct (TGD) to the anterior trachea at
the level of the second and fourth tracheal rings
– Incomplete descent: Ectopic thyroid (lingual thyroid)
70% without cervical thyroid→establish functional thyroid tissue
– Incomplete involution of TGD: Pyramidal lobe
• Calcitonin-secreting parafollicular C cells arise within the ultimobranchial bod-
ies from neural crest cells
Anatomy
Macroscopic
• The thyroid gland is a bilobed structure connected by central isthmus
• Enveloped by deep cervical fascia and covered by strap muscles→ posterior
condensation of fascia→suspensory ligament of Berry
Microscopic
• Each lobe is contained within a brous capsule
• Within each capsule, there are a collection of follicles, composed of thyroglobulin-
producing cells surrounding manufactured colloid material
– Each follicle is surrounded by a basement membrane with a ne capil-
lary network
– Parafollicular cells (C cells) can also be found within the basement membrane
Vascular
Arterial supply (2 vessels)
• Superior thyroid artery from external carotid artery
• Supercial to external branch of superior laryngeal nerve
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