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Case Studies

Melanotic Schwannoma – An unusual pathology in the internal auditory meatus addressed by translabyrinthine approach

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A 50-year-old non-diabetic, non-hypertensive woman presented to Bangalore Skull Base Institute with history of continuous, non pulsatile tinnitus 4 years duration on the right side with gradually progressive hearing loss with inability to understand speech on the right side on 1 year duration. She also gave history of imbalance of 6 months duration. There was no history of facial weakness or numbness, ear discharge, ear pain, falls, headache or vomiting.

On examination, bilateral ear were normal.

 

Cranial nerve examination revealed reduced sensation of V1,V2 and V3 segments of trigeminal nerve was noted. Facial nerve examination was normal. Rombergs test was positive to the right side. Unterbergers test revealed displacement and positional change to the right side.

Pure tone audiometry showed a moderately severe hearing loss on the right side with minimal hearing loss on the left side

MRI with contrast revealed a non-restricting T1 hyper-intense / T2 hypo intense lesion in the right

Internal auditory meatus measuring 12 x 4mm  with no significant change post contrast.

The patient underwent translabyrinthine approach for removal of the tumor. Intra-operatively a blackish thick tarry-like material was noted in the right internal auditory meatus. Total clearance was achieved. Histopathological examination revealed the diagnosis of melanotic schwannoma. Patient is currently on follow-up and recovering well.

Giant Vestibular Schwannoma – Translabyrinthine Approach Achieves Total Removal

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A 46-year-old man presented to Bangalore Skull Base Institute with history of hyperaesthesia of the left side of the tongue, hypoaesthesia of the left side of the face, recurrent headache, blurring of vision of 4 months duration with on and off episodic vertigo. He also had history of slurring of speech of 1 month duration. He did not have history of hearing loss, tinnitus or facial weakness. He did not have complaints of aspiration, cough or change in voice. 

On examination, tympanic membrane was normal on the both the sides. He had delayed blinking on the left side. Rest of the cranial nerves were within normal limits. Pure tone audiometry showed a mild, mixed hearing loss on both the sides. 

 MRI with contrast showed a lobulated, well-defined, irregular, extra-axial mass with cystic component in the left cerebellopontine angle with heterogenously enhancing solid component and peripherally enhancing cystic component, overall measuring 3.5 x 2.0 x2.7cm, combined measuring 5.2 x 4.5 X 3.4 cm, with extension through the internal auditory meatus causing widening of porus acousticus involving the VII and VIII nerve complex and in close relationship with IX cranial nerve inferiorly which was not separately seen. Portions of left 7th and 8th cranial nerve within lateral portion of left IAC appeared normal. Root entry zone of left 5th cranial nerve is seen displaced superomedially by the lesion.

Lesion was not only seen causing mass effect on the pons and middle cerebellar peduncle causing displacement to opposite side, left cerebellar hemisphere posteriorly but also lateral cystic component of the lesion was seen causing focal infiltration of right MCP. Lesion was causing effacement of the fourth ventricle and widening of left half of prepontine cistern. Multiple curvilinear blooming areas noted suggesting calcifications. Mild tonsillar descent across foramen magnum was also noted.

Patient underwent translabyrinthine approach for removal of left vestibular schwannoma under general anaesthesia. Internal acoustic meatus was exposed from the fundus to the porus acousticus and the canalicular component of the tumor was identified. Dura was opened to identify the cisternal solid component and the cystic component of the tumor. The tumor was found involving the vestibulocochlear bundle which was traced upto the root entry zone into the brainstem. Facial nerve integrity was maintained during the entire procedure. The tumor was removed in toto. Post-operative scan done at one year follow-up showed no evidence of residual/recurrent tumor.

Facial Nerve Schwannoma: Early Removal, Better Outcome!

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A 40 year old lady came to Bangalore Skull Base Institute with the history of left side ringing sensation which was continuous whooshing sound & non pulsatile since 1 year, left ear hard of hearing since 5 months and left earache since 4 months. There was no history of facial weakness, drooling, change in voice, difficulty swallowing, difficulty in speech articulation.

On clinical examination, whitish pink mass was seen arising from the posterior wall and floor of the left external auditory canal obscuring the posterosuperior and inferior quadrant of the tympanic membrane. Associated grade 2 retraction of the membrane was also noted. Right ear was relatively normal. On cranial nerve examination, slight lid lag over the left was noted and rest of the facial and other cranial nerves were normal.

 

PTA showed mild mixed hearing loss on the left ear. HRCT and MRI with contrast showed a heterogenously enhancing T2 iso to hyperintense lesion arising from the left facial nerve involving geniculate ganglion, tympanic and vertical mastoid segment seen bulging into the adjacent middle ear cavity abutting medial aspect of middle ear & ossicles. A diagnosis of facial nerve schwannoma was made. Patient underwent a transmastoid approach for its excision with sural nerve interposition grafting with deep lobe parotidectomy under General anesthesia. In the surgery,Facial nerve tumor was identified involving the horizontal segment including the geniculate ganglion and partly the labyrinthine segment & was found extending into the deep lobe of the parotid. Sural nerve graft placed. Temporalis fascia graft harvested, wrapped around the inferior portion of sectioned nerve. Post operatively the patient did well with grade 4 facial palsy and is on regular follow up with progressive signs of improvement.

Long Duration Epistaxis – Catch Tumors Early For Complete Endoscopic Resection

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A 60-year-old woman complained of left nasal discharge and on and off epistaxis of one year duration. Patient also complained of heaviness of the head of 3 months duration.
On examination, she had a deviated nasal septum to the left side with spur. A reddish, slough covered friable mass was noted at the anterior end of the middle turbinate which could be probed medially but not laterally. Provisional diagnosis of left inverted papilloma was made.

MRI with contrast showed a well defined homogenous T1 hypointense, STIR hyperintense avidly enhancing lesion measuring 24 x 24 x 10 mm filling the anteromedial and superior nasal cavity on the left side, medially lying flush with the posterior nasal septum and posteriorly extending into the anterior olfactory cleft causing widening. Inferolaterally, it is seen abutting and scalloping the inferior turbinate and posteroinferiorly it is seen abutting  the left middle turbinate. Posterosuperiorly, it is related anterior to the crista galli extending into the anterior ethmoid sinus. HRCT with contrast revealed an intact bony skull base.

Patient underwent left Draf type 2b surgery with clearance of the tumor under general anaesthesia. Friable slough covered mass was seen arising from the skull base anterior to the olfactory plate extending into the nasal cavity and impinging on the middle turbinate and nasal septum but not involving it.

Uncinectomy,  middle meatal antrostomy, anterior and posterior ethmoidectomy, sphenoidotomy and frontal recess clearance were done on the left side. Multiple biopsies were taken from the tumor. Tumor was debulked using a microdebrider. Middle turbinate was excised on the left side. Coblator was used to mark the margins of the tumor origin. Mucosa at the site of the tumor was removed. Drilling of the nasal bone and anterior wall of frontal sinus was done. First olfactory fiber was identified. Hemostasis was achieved with coblation and bipolar cautery.

In histopathology, the final diagnosis was capillary hemangioma.

There is never an age to treat skull base osteomyelitis.
Quality of life improves instantaneously after surgery!!

Pre Op Facial Nerve Examination (4)
Pre Op Facial Nerve Examination (4)

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Pre Op Facial Nerve Examination (3)
Pre Op Facial Nerve Examination (3)

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Pre Op Facial Nerve Examination (4)
Pre Op Facial Nerve Examination (4)

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An 85-year-old gentleman presented to BSBI with progressive right ear pain of 3 months duration, facial weakness, and reduced hearing of 2 months duration, with difficulty in swallowing, aspiratory spells, change in voice, and choking spells of 10 days duration. He was a diabetic and hypertensive on medication.

Clinical examination revealed extensive granulations in the right external auditory canal obscuring the view of the right tympanic membrane. There was tenderness around the right temporomandibular joint. Cranial nerve examination revealed a right lower-motor-neuron facial palsy(House Brackmann Grade IV) with IX, X, XI, and XII cranial nerve palsies.

HRCT of the temporal bone revealed a soft tissue density in the right external auditory canal and middle ear with the erosion of the floor of the right external auditory canal lateral to the right jugular bulb. MRI with contrast also revealed involvement of the parotid space, superficial and sub-temporal space, and right infratemporal fossa.

A clinical diagnosis of Right Skull Base Osteomyelitis was made and the patient underwent right subtotal petrosectomy with infratemporal fossa type A extension. Histopathological examination revealed chronic granulation tissue with osteomyelitic changes. Bacterial culture grew pseudomonas which was treated with antibiotics. The fungal and mycobacterial culture was negative.

Post-operative follow-up showed clearance of disease and improvement in symptoms with residual lower cranial nerve palsies.

Giant Vestibular Schwannoma – Translabyrinthine Approach With Transapical Extension Offers A Wide Exposure Without Brain Retraction

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A 59-year-old hypertensive man presented to Bangalore Skull Base Institute with a history of tinnitus and progressive hearing loss in the right ear of 5 years duration, numbness on the right side of his face of 5 months duration, swaying to the right side of 2 months duration and taste change and reduced sleep of 15 days duration.

 

The patient did not give a history of facial weakness or lower cranial nerve weakness. He did not complain of fever, ear discharge, or ear pain.

On clinical examination, there was evidence of fifth cranial nerve weakness and delayed blinking on the right side. There was swaying to the right side on the Romberg test. Other Cerebellar signs were negative. Motor and sensory systems were normal. Ear examination was normal.

Pure tone audiometry revealed severe mixed hearing loss in the right ear with normal hearing in the left ear. Videonystagmography was normal. MRI revealed a 3.7 x 2.9 x 3 cm heterogeneously enhancing extra-axial soft tissue mass with a cystic component in the right cerebellopontine angle which was hyperintense on T2W and hypointense on T1W. Prominence was noted in the ventricular system with widening of the sulci and cerebellar folia.

The patient underwent translabyrinthine approach with transapical extension for the excision of the right vestibular schwannoma. Intra-operatively, a 3.6 cm vestibular schwannoma was noted indenting the brainstem, lower cranial nerves, and cerebellum with extension into the IAC.

Sutures were removed on day 10 and the patient is on follow-up and recovering well with no residual symptoms and complete improvement from the 5th nerve symptoms. 

Giant Petroclival Neurofibroma – Endoscopic Endonasal Access To The Jugular Foramen

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Post Op CT Sep 21 (Facebook Post)
Post Op CT Sep 21 (Facebook Post)

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A 27-year-old woman presented to Bangalore Skull Base Institute with complaints of left hemicranial headache extending to the neck of 8 months duration. She had no improvement with medications. The patient also complained of occasional giddiness.

There was no history of blurring of vision, nausea, and vomiting. On endoscopic examination, the patient had a fullness in the left nasopharynx stopping short of the fossa of Rosenmuller. There was a deviated nasal septum to the left side. Cranial nerves, and sensory and motor function were normal.
 

CECT revealed a mildly enhancing heterogenous mass located in the petroclival region displacing the internal carotid artery. CEMRI revealed an isointense petroclival mass on T1W, and hyperintense mass on T2W with T1W contrast enhancement. The patient underwent pre-operative Intra carotid stenting with DSA and embolization of feeding vessels.
 

The patient underwent a transnasal endoscopic approach(combined medial maxillectomy and endoscopic infratemporal fossa approach) for the removal of the petroclival mass. Neuronavigation and lower cranial neuromonitoring were placed to safeguard critical structures. A prelacrimal approach was used to remove the medial wall of the maxilla. The sphenoid floor was made flush with the posterior wall of the nasopharynx. The base of the pterygoids was drilled completely along with the pterygoid wedge after identification and sectioning of the V2 and Vidian nerve. The infraorbital nerve was identified and followed up to the pterygovaginal canal.
 

Tumor resection started from the clivus followed up between the Eustachian tube and paraclival, petrous carotid to the parapharyngeal carotid with a coblator. V3 was identified entering the foramen ovale. V3 and its branches were sectioned. Positioning of the parapharyngeal carotid confirmed with doppler. Eustachian tube resected. Remnant the tumor on the lateral part of the parapharyngeal carotid coblated.
 

Post-op CT scan revealed in toto removal of the tumor. Histopathological examination revealed the presence of a petroclival neurofibroma.
 

The patient is on regular follow-up and is symptom-free.

TYMPANOJUGULAR PARAGANGLIOMA – CAREFUL COMPLETE BLOODLESS DISSECTION OF THE TUMOR IS THE MANTRA; DURATION OF SURGERY IS IRRELEVANT!!

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A 40-year-old woman from Mangalore was presented to Bangalore Skull Base Institute with reduced hearing in the left ear of 2 years duration with pulsatile tinnitus and giddiness of 1 year duration. Patient also had a history of voice change of 6 months duration. Patient did not give a history of ear discharge, bleeding from the ear or facial weakness. There was no evidence of difficulty in breathing, swallowing or regurgitation of food. There was no history of facial weakness.

Examination revealed a pulsatile, reddish mass in the left external auditory canal. Tympanic membrane was obscured by the pulsatile mass. Right ear was normal. Cranial nerve examination was normal.

Pre-operative audiometry revealed a moderate mixed hearing loss in the left ear with a “B” type curve on tympanometry. CECT of the temporal bone revealed an enhancing lesion arising from the left jugular foramen, protruding into left CP angle measuring 7.5 X 14 X 20mm with similar enhancement in the adjacent sigmoid sinus and internal jugular vein.

Bony erosions were noted involving the left jugular foramen. Bony erosion was noted in the proximal aspect of the left carotid canal and posterior wall of the left eustachian tube. Soft tissue densities were noted in the left eustachian tube and left middle ear suggesting a likely infiltration by the lesion.

CE-MRI revealed a T1W contrast-enhancing tumor originating from the left jugular foramen extending into the hypoglossal canal on the left side. Tumor extension was noted in the left internal jugular vein. Extradural extension into the left internal auditory meatus was noted. Tumor enhancement was also noted on T2W sequences.

Diagnosis of Left tympanojugular paraganglioma Fisch Classification De1 was made.

DSA and embolization revealed feeders from the left ascending pharyngeal artery and left occipital artery. Both vessels were embolized prior to surgery.

The patient underwent Infratemporal Fossa A approach for removal of left tympanojugular paraganglioma with OSIA implantation for rehabilitation of hearing. Intra-operatively the tumor was involving the middle ear(epi, meso, and hypotympanum extending into the Eustachian tube). Inferiorly the tumor was visualized involving the jugular foramen area and abutting the petrous carotid destroying the caroticojugular spine and medially extending extradurally into the IAM. The tumor was involving the jugular bulb with the involvement of the IJV lumen. Partial anterior transposition of the facial nerve resulted in a Grade III House Brackmann facial nerve palsy post-operatively.

The patient was discharged on postoperative day 5 and is currently on follow-up and recovering well.

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