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Facial nerve schwannomas R3 康焜泰 Introduction Uncommon disease Originate any segment of facial nerve CP angle peripheral branch in the parotid gland Varying clinical symptoms Diagnosis: difficult Anatomy of facial nerve Segmental Description of the Facial Nerve and Central Connections Segment Location Length, mm Supranuclear Cerebral cortex NA?/td> Brain stem Motor nucleus of facial nerve, superior salivatory nucleus, tractus solitarius NA?/td> Meatal segment Brain stem to IAC 13-15 Labyrinthine segment Fundus of IAC to facial hiatus 3-4 Tympanic segment Geniculate ganglion to pyramidal eminence 8-11 Mastoid segment Pyramidal process to stylomastoid foramen 10-14 Extratemporal segment Stylomastoid foramen to pes anserinus 15-20 Intracranial segment brainstem in a recess adjacent to the pons, cross CP angle medial to vestibuloacoustic nerve Meatal segment (13-15 mm) 由 Brain stem IAC Internal auditory canal Labyrinthine segment (3-4 mm) Within narrowest part of fallopian canal to geniculate ganglion Tympanic segment (8-11 mm) Geniculate ganglion to pyramidal eminence Mastoid segment (8-11 mm) Pyramidal process to stylomastoid foramen Extratemporal branch: stylomastoid foramen 1. Temporal 2.Zygomatic 3. Buccal 4. Mandibular 5. Cervical Brain stem : facial nuclei Facial nerve function 1.Brachial motor: facial expression 2.Visceral motor: lacrimation, salivation (submandibular, sublinguar, nose, palate) 3.Special sensory: taste (tongue ant. 2/3) 4.General sensory: auricular sensation Branches of facial nerve GSPN: geniculate ganglion GSPN sphenoplalatine ganglion lacrimal and palatine gland 支配 lacrimation & salivation Nerve to stapedius muscle : 由 proximal mastoid segment 分出 Chorda tympani: 由 distal mastoid segment 分出 , 和 lingual nerve 一同支配 sublingual gland 和 submandibular gland, Taste Sensory auricular branch Sensation of posterior external auditory canal & inferior pinna of ear Some axons may arise from vagus (Arnold's nerve) Motor: Facial muscles Facial nerve anatomy FIGURE LEGEND 1. Facial nerve nucleus 2. Trigeminal nerve: Spinal nucleus 3. Superior salivary nucleus 4. Solitary tract 5. Porus acusticus internus 6. Meatal foramen 7. Greater petrosal nerve 8. Sphenopalatine ganglion 9. Maxillary nerve 10. Lacrimal gland 11. Deep petrosal nerve 12. Vidian nerve 13. Nerve to glands of nose and palate (motor fibers to levator palati muscles) 14.Minor petrosal nerve anastamosis 15. Stapedial nerve 16. Chorda tympani 17. Auricular branch 18. Stylomastoid foramen 19. Lingual nerve 20. Submandibular ganglion 21. Submandibular gland 22. Sublingual gland Facial nerve imaging-CT Facial nerve imaging-MRI T1 T2 Facial nerve schwannoma Rare 1931 Schmidt: First report 1972 Saito and Baxter: report 0.8% occult FNS incidence in a study of 600 temporal bone Now, more than 400 reported cases Benign, encapsulated, slow-growing tumor Age: 7-81 y/o, mean age 41.7 y/o No sexual predominant No side preference No bilateral FNSs reported Locations Arise from any segment of facial nerve from CP angle to peripheral branch in parotid gland The geniculate ganglion is frequently involved and is often affected proximally or distally. Labyrinthine: 43.5% Tympanic: 42.8% Mastoid: 36.7% IAC: 24.3% CP angle: 17.8% Extratemporal peripheral: 15% Sherman et al, 2002 Symptoms Progressive facial paresis 73% Hearing loss 50% Tinnitus 13% Dizziness 11% EAC mass 11% Pain 11% Otorrhea 6% Pre-operative evaluation Detailed history, clinical symptoms, and radiologic data However, diagnosis is difficult Pre-operative histopathologic diagnosis Fine needle aspiration: Not possible for some FNS cases Limited value for diagnosis Balle and Greisen(1984): repeated FNA not contribute to correct preoperative diagnosis in their 2 intraparotid FNSs. Chong et al(2000): only 1 patient had preoperative diagnosis suspicious of schwannoma among 5 intraparotid FNS Image study CT and/or MRI provide important for determining the localization, nature, and extent of tumor. CP angle and IAC Characterized by sensorineural hearing loss Because more thinly myelinated sensory fibers of the acoustic nerve are more vulnerable to compression than more thicky myelinated motor fiber of the facial nerve May cause both acoustic and facial nerve symptoms May clinically and radiologically indistinguish from acoustic neuroma CP angle and IAC Labyrinthine segment Slowly progressive facial paresis and sensorineural hearing loss Schwannoma in the geniculate ganglion grow silently before facial nerve symptoms and hearing loss present Geniculate ganglion Tympanic segment Progressive facial paresis, fullness in the ear and conductive hearing loss Tend to first cause conductive hearing loss by ossicular interference Tympanic segment Tympanic segment Mastoid segment Progressive facial paresis, otorrhea, conductive hearing loss Peripheral segment Mass in the parotid gland Slowly progressive facial paresis Multi-segment Usually accompanied by facial nerve symptoms Multi-segment Facial nerve schwannoma are often sausage-shaped, expanding long segment of facial nerve Differential Diagnosis Acoustic neurinoma Chronic otitis media with cholesteatoma Dermoid cyst Benign parotid tumor Treatment Controversial because facial paralysis is inevitable after surgical resection. Complete surgical resection+facial nerve reconstruction is the most recommended modality. Treatment Some authors advise observation rather than surgical excision 1. Slow growing, benign tumor 2. Many patient do not present significant facial paralysis suggest delay surgery until patient exhibit > H-B grade III tumor followed by radiologic imaging Treatment Some authors advise facial nerve decompression Angeli and Brackmann(1997): 4 patients treated with decompression sugery HB grade I and grade II postoperatively Treatment Surgical approach is based on tumor location, extent, and hearing level Prepare to explore tumor from CPA to stylomastoid foramen Treatment Tumor intracranial : retrosigmoid approach Tumor from CPA, IAC, or labyrinthine segment No serviceable hearing: translabyrinthine approach or transotic approach Good hearing: middle fossa approach Tumor from tympanic or vertical segment: transmastoid apraoch Tumor from peripheral segment: superficial parotidectomy Treatment-Gamma knife 1783 schwannoma of CP angle treat by Gamma knife surgery from 1992-2003 in Timone University Hospital, France 11 patients: 9 involve tympanic or mastoid segment of facial nerve 2 previous microsurgery Prognosis Excellent Morbidities: facial paralysis, hearing loss, CSF leak, meningitis, and recurrence Post-operative facial nerve paralysis Sataloff et al(1995): remove tumor with facial nerve preservation in 25% cases Interposition nerve graft: - greater auricular nerve: obtain from neighboring - hypoglossal nerve: - direct anaastomosis without a graft: rare Recurrence Rare Janecka and Conley(1987): report only 2 recurrences after surgery among 30 FNS cases Reference 1.Shirazi MA, Leonetti JP, Marzo SJ, Anderson DE.Surgical Management of Facial Neuromas: Lessons Learned.Otol Neurotol. 2007 Jul 26 2.Lee JD, Kim SH, Song MH, Lee HK, Lee WS.Management of facial nerve schwannoma in patients with favorable facial function.Laryngoscope. 2007 Jun;117(6):1063-8. 3.Litre CF, Gourg GP, Tamura M, Mdarhri D, Touzani A, Roche PH, Regis J.Gamma knife surgery for facial nerve schwannomas.Neurosurgery. 2007 May;60(5):853-9. 4.Park HY, Kim SH, Son EJ, Lee HK, Lee WS.Intracanalicular facial nerve schwannoma.Otol Neurotol. 2007 Apr;28(3):376-80. 5.Kreeft A, Schellekens PP, Leverstein H.Intraparotid facial nerve schwannoma. What to do? Clin Otolaryngol. 2007 Apr;32(2):125-9. 6.Weber PC, Osguthorpe JD.Facial nerve neuroma management.Skull Base Surg. 1998;8(1):51-5. 7.Chi FL, Wang ZM, Chen ZY, Wu YZ.Diagnosis and management of facial nerve neuromas 8.Senthilvel E, McConn Walsh R, Brett FM, Rawluk D.Facial nerve schwannoma of the internal auditory canal.Ir J Med Sci. 2006 Jan-Mar;175(1):74-6. 9.Wiggins RH 3rd, Harnsberger HR, Salzman KL, Shelton C, Kertesz TR, Glastonbury CM.The many faces of facial nerve schwannoma.AJNR Am J Neuroradiol. 2006 Mar;27(3):694-9. 10.Kirazli T, Oner K, Bilgen C, Ovul I, Midilli R.Facial nerve neuroma: clinical, diagnostic, and surgical features.Skull Base. 2004 May;14(2):115-20. The end