Imaging Approach To & Interpretation of Findings in

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IMAGING APPROACH TO & INTERPRETATION OF FINDINGS IN LESIONS IN THE CEREBELLO PONTINE ANGLE & INTERNAL AUDITORY CANAL
BY DR (MRS) YANJU AKINOLA MBChB, FWACS, FMCR, FICS ASSOCIATE PROFESSOR OF RADIOLOGY / HONORARY CONSULTANT LASUTH / LASUCOM
CNS UPDATE 2013 LUTH 02/08/2013
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Objectives
1. To review the process of imaging patients suspected of harbouring CPA-IAC lesions. 2. To review the anatomy of the CPA-IAC. 3. To identify imaging findings of the common lesions found in the CPA-IAC.
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Introduction
CPA tumours are the most common neoplasm in the posterior fossa, constituting 5-10% of intracranial tumors. Mostly benign and extra-axial Vestibular schwannomas (acoustic neuromas), 85% Lipomas, vascular malformations, hemangiomas.
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Introduction (Contd.)
The most frequent non-acoustic CPA tumours include, meningiomas epidermoids (primary cholesteatomas), facial or lower cranial nerve schwanomas. Primary malignancies or metastatic lesions account for less than 2% of neoplasm in the CPA
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DD of CPA LESIONS
Vestibular schwannoma (85%) Meningiomas (3-13%) Epidermoids (2-6%) Facial and lower cranial nerve schwannomas (1-2%) Arachnoid cysts (1%) Rare tumors Lipomas Dermoid tumors Neuroepithelial cysts Trigeminal schwannoma Endolymphatic sac carcinomas Ependymoma choroid plexus papillomas, metastatic adenocarcinomas, metastatic neuroblastoma, ependymoma, lymphoma, cholesterol cyst, angioleiomyoma, venous hemangioma, cavernous angioma, and pontine glioma.
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OTHER DD.
Brainstem glioma Astrocytoma Medulloblastoma Choroid plexus papilloma Large or giant aneurysms of anterior inferior cerebellar artery (AICA), superior cerebellar artery (SCA), or the midbasilar artery Brainstem arteriovenous malformation (AVM)
Brainstem cavernous malformation originating in the brain stem (may reach a pial surface along the CPA) Petroclival chondrosarcoma Clivus chordoma (usually arise in midline but may extend laterally into the CPA) Osteosarcoma of the lateral skull base Metastatic tumors (can reach CPA by hematogenous spread or direct extension) Granulomatous inflammatory masses
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Anatomy of the CPA & IAC

CPA is the anatomic space between the cerebellum and the pons. A common site for the growth of acoustic neuromas or schwannomas. Neurologic syndrome / deficits occur due to the proximity of the CPA to specific cranial nerves
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Anatomy of the CPA & IAC (Contd.)

8th cranial nerve enters the cranial cavity through the internal auditory meatus, which is found in the temporal bone also entering through this canal are the facial nerve and the labyrinthine artery(which provides the blood supply to the inner ear) the close relationship between the VII and VIII cranial nerves can explain some of the complications of an ACOUSTIC NEUROMA 8th nerve fibres enter the ventrolateral surface of the medulla at the cerebellopontine angle, anterior to the inferior cerebellar peduncle (25 on the brainstem) and lateral to the facial nerve
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Lateral view of the brainstem: 1. medial geniculate body 2. brachium of the inferior colliculus 4. inferior colliculus 25. Cerebellar Peduncle
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The internal acoustic meatus (also internal auditory meatus, IAM) is a canal within the temporal bone that transmits nerves and vessels from within the posterior cranial fossa to the auditory apparatus. Contents of the IAM include: Facial nerve, V11 Vestibulocochlear nerve, V111 Vestibular ganglion Labyrinthine artery (usually a branch of the Anterior Inferior Cerebellar Artery or basilar artery) The opening of the IAM is located within the cranial cavity, near the posterior surface of the temporal bone.
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IAC
Normal anatomy of the temporal bone. MPR/CT
EAC =External auditory canal, Carotid canal = CC Jugular bulb = JB, Malleus = M, Facial nerve = FN, Cochlea = C, Semicircular canals = SCC, Internal auditory canal = IAC, Incus = I, Vestibule = V, Vestibular aqueduct = VA, Mastoid air cells = MAC.
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1. 2. 3. 4. 5. 6. 7. 8. 9. 10. Ear drum Malleus Incus Stapes Semicircular canals Auditory nerve Facial nerve Vestibular nerve Cochlea Eustacian tube
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The margins of the opening are smooth and rounded and the canal is short (1 cm), running laterally to the bone. At the distal end of the canal, it splits into three distinct openings, one of which is the facial canal.
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Nerves There are five nerves that run through the IAC: 1. nervus intermedius (sensory component of CN V11) 2. facial motor root (motor component of CN V11) 3. cochlear nerve (component of CN V111) 4. inferior vestibular nerve (component of CN V111) 5. superior vestibular nerve (component of CN V111) Their position is most constant in the lateral portion of the meatus which is anatomically divided by the falciform crescent. This horizontal ridge divides the canal into superior and inferior portions.
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Superior: facial nerve and superior vestibular nerve (SVN); the facial nerve is anterior to the SVN and is separated from it laterally by Bills bar, a vertical ridge of bone Inferior: cochlear nerve and inferior vestibular nerve (IVN); the cochlear nerve is situated anteriorly Ganglion In addition to the three nerves which enter it, it also contains the vestibular ganglion (Ganglion of Scarpa) From here three bundles emerge (superior and inferior division of the vestibular nerve and the nerve from the posterior semicircular canal
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Imaging
The IAC is housed by the petrous temporal bone which is a complex structure with many tiny bones (crura of stapes, vestibular acqueduct, <1mm) with poor spatial resolution CT Bony details MRI Inner ear & its central connections Conventional angio MR & CT Angio Metabolic imaging (FDG-PET) to differentiate radiation damage from recurrent tumour
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Plain radiography, now obsolete except for postop. assessment of cochlear implants Views include, Stenvers view (Oblique PA) Periorbital view best for IAM, if tomography is not available CT shows intracranial lesions & its extensions Contrast enhancement required except if lesion is confined to petrous pyramid Thin slices with wide window setting, 3000-4000HU Starts with lateral scout film, then axial, coronal & reformatted images
Imaging (Contd.)
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Imaging (Contd.)
MRI Bone Negligible signal. Appears same as air Bone details better seen by CT Soft tissue details on MRI. Cranial Nerves well seen TIW intense signal with yellow bone marrow (high fat content especially in petrous apex) CSF & Labyrinthe fluid low signal intensity on T1W, Spin echo & high signal on T2W Flowing blood gives no signal blood vessels are normally black
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Imaging (Contd.)
Standard MRI studies using TI & T2 Protocol T1W - better spatial resolution T2W - poorer spatial resolution & take longer to acquire images. T2 gives higher signal from water containing structures (as in tumours) With Gadolinium DTPA, there is significant enhancement
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Imaging (Contd.)
Angiography Has a limited role now for vascular anomalies and tumours. MRA & CTA have replaced it Rarely used as diagnostic means now. More for pre operative assessment and for therapeutic embolization Aneurysm and vascular malformations require selective angio. Gradient echo sequences used
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Imaging Studies
Adults suspected to have CPA-IAC disease, usually complain of sensorineural hearing loss (SNHL), can be imaged either with conventional enhanced T1 fat-saturated MR or high-resolution T2 MR imaging. CPA-IAC diseases that may be found include congenital lesions such as epidermoid cyst, arachnoid cyst and CPA-IAC lipoma. Benign tumors found in this location include acoustic schwannoma, meningioma, facial nerve schwannoma and IAC hemangioma.
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Acoustic Neuroma (Vestibular Schwanoma)

Benign, primary intracranial tumours of the myelin forming cells of the vestibulocochlear nerve (CN VIII). involves the vestibular portion of the 8th cranial nerve, arises from Schwann cells, which are responsible for the myelin sheath in the peripheral nervous system. 3,000 cases are diagnosed each year in the US, with a prevalence of about 1 in 100,000 worldwide. 510% of all intracranial neoplasm in adults. Incidence peaks in the fifth and sixth decades both sexes are affected equally.
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Imaging Studies (Contd.) Site of acoustic N/Intracanalicular Schw.

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Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Ipsilateral SNHL/deafness, disturbed sense of balance and altered gait, vertigo with associated nausea and vomiting, pressure in the ear, all of which can be attributed to the disruption of normal vestibulocochlear nerve function. Tinnitus in > 80%, (usually unilateral, high-pitched ringing, sometimes a machinery-like roaring or hissing sound, like a steam kettle). Large tumours that compress the adjacent brainstem may affect other local cranial nerves.
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7th cranial nerves are rarely involved preoperatively Involvement of the trigeminal nerve (CN V loss of sensation in the involved side's face and mouth. The glossopharyngeal and vagus nerves are not usually involved, but their involvement altered gag or swallowing reflexes. Larger tumors increased intracranial pressure, with its associated symptoms such as headache, vomiting, and altered consciousness.
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Pathogenesis May occur idiopathically or as part of von Recklinghausen neurofibromatosis, in which case the neuroma may take on one of two forms. In Neurofibromatosis type I, schwannoma may sporadically involve the 8th nerve, usually in adult life, but may involve any other cranial nerve or the spinal root. Bilateral acoustic neuroma are rare in this type. In Neurofibromatosis type II, bilateral acoustic neuroma are the hallmark and typically present before the age of 21. Tend to involve the entire extent of the nerve and show a strong autosomal dominant inheritance. Incidence is about 5 to 10%.
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Usually in the adult, presents as a solitary tumour, originating in the nerve. From the vestibular portion of the 8th nerve, just within the internal auditory canal. Usually extends into the posterior fossa to occupy the angle between the cerebellum and the pons (cerebellopontine angle). may also compress the 5th, 7th, and less often, the 9th and 10th cranial nerves.
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Later, may compress the pons and lateral medulla, causing obstruction of the CSF and increased intracranial pressure. Schwannomas can occur in relation to other cranial nerves or spinal nerve roots, resulting in radiculopathy or spinal cord compression. Trigeminal neuroma are the second most common form of schwannomas involving cranial nerve. Schwannomas of other cranial nerve are very rare.
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Contrast-enhanced CT will detect almost all acoustic neuromas that > 2.0 cm in diameter and project further than 1.5 cm into the cerebellopontine angle. smaller tumors may be detected by MRI with gadolinium enhancement.
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VESTIBULAR SCHWANOMA VESTIBULAR SCHWANOMA
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VESTIBULAR SCHWANOMA VESTIBULAR SCHWANOMA
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Meningioma
Pathophysiology 2nd most common tumour of CPA Arise from the meninges, the membranous layers surrounding the CNS. According to the American Brain Association, they are the most common primary brain tumour, representing 1/3 of all such tumors. Arise from the arachnoid "cap" cells of the arachnoid villi in the meninges. Usually benign but may be locally aggresive; however, a small percentage are malignant
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Pathophysiology Meningioma (Contd.)

Many are asymptomatic, producing no symptoms throughout a person's life, and require no treatment other than periodic observation. The 4 histologic variants, (1) meningothelial, (2) fibroblastic, (3) transitional, (4) angioblastic. Angioblastic variant is more aggressive. usually arise on the posterior surface of the petrous bone, near the porous acusticus, or along the lateral venous sinus. Oval / hemispheric broad dural-based, attached to the tentorium/petrous bone dura
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Pathophysiology Meningioma (Contd.)

Displace but do not invade adjacent neural tissue. Meningiomas usually have a thin investing capsule. They can invade bone without destruction by extension along haversian canals. 60% extend into the middle fossa. Intense contrast enhancement Usually does not extend into the IAC
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Epidermoid
Pathophysiology Originate from epithelial rests within the temporal bone or CPA. Usually grow very slowly with undulating margins. Patients often do not present until the 2nd to 4th decade of life. Tend to envelop rather than displace adjacent neural and vascular structures. Variable shapes, with irregular patterns. Usually develop multiple fingerlike projections between cranial nerves and brainstem fissures, sulci and blood vessels.
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Pathophysiology - Epidermoid (Contd)

Lamellated / onion skin appearance May extend through the tentorium into the middle fossa, forming a dumbbell shape. Imaging signal is similar to that of CSF May show a peripheral rim of calcification No enhancement with Gadolinium on MRI T1W Hypo / Iso intense T2W & Flair Iso / Hyperintense DWI Marked restriction bright appearance
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Pathophysiology - Epidermoid (Contd)

Apparent diffusion coefficient (ADC) values of epidermoid are significantly lower than that of CSF and higher than that of deep white matter Unusual imaging findings include, Hyperdensity on CT, T1W, T2W MRI which may be due to the high protein content of the cyst
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Facial and lower cranial nerve schwannoma:

Arise from Schwann cells anywhere along the course of the nerve. May be cystic / solid Cystic schwanomas are larger, with a thin rim enhancement of the wall Funnel shaped in axial MRI and club shaped on coronal images Half of the solid tumours strong homogenous enhancement
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Arachnoid cysts
CPA is the 2nd most common site for arachnoid cyst to occur, sylvian fissure being the 1st These are thin-walled sacs containing yellow cerebral spinal fluid (CSF) that most likely originate as congenital developmental anomalies. Smooth walled with CSF intensity on MRI Dont enhance with contrast
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Arachnoid cysts(Contd.)
Similar to epidermoid cysts in that they are well defined, homogenous low density, similar to CSF on CT, with no contrast enhancement and on MRI Hypointense on T1W and hyperintense on T2W On FLAIR, similar intensity to CSF unlike epidermoid which is strongly hyperintense Echo planar diffusion scanning, helps differentiate : Arachnoid cyst is hypointense but epidermoid is hyperintense
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Presentation of CPA tumours

CNS UPDATE 2013 LUTH
02/08/2013
Hearing loss - 95% Tinnitus - 80% Vertigo/unsteadiness - 50-75% Headache - 25% Facial hypesthesia - 35-50% Diplopia - 10% Symptoms can vary according to the size and location of the lesion.
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Presentation of CPA tumours (Contd.)

Meningioma Trigeminal or facial nerve symptoms are likely to occur earlier than hearing loss. Patients with larger tumours can present with obstructive hydrocephalus and/or symptoms of brainstem compression. Epidermoid These can become quite large without symptoms. Facial twitching (hemifacial spasm) and progressive facial paralysis is more prominent than with other tumors in the CPA. Patients may present with cranial nerve or cerebellar dysfunction that develops over a number of years.
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Presentation of CPA tumours(Contd.)

Facial nerve schwannoma Findings depend on the portion of the nerve affected Conductive hearing loss from middle ear involvement, parotid mass from extratemporal involvement, or sensorineural loss from internal auditory canal (IAC) or CPA involvement. CPA lesions do not cause facial weakness until tumor is very large (hemangiomas of the facial nerve usually cause symptoms at an earlier stage). Hemifacial spasm is relatively common. Lower cranial nerve schwannoma Patients may have weakness and hypesthesia of the palate, vocal cord, and shoulder (cranial nerves [CN] IX, X, XI) or hemiatrophy of the tongue (CN XII). Large tumours may cause deficits of all the lower cranial nerves
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Imaging Studies
Meningioma Computed tomography (CT) scanning findings
Hyperdense compared with the cerebellum on NCE images May demonstrate calcifications within or at the periphery of the tumor May be a broad attachment to the petrous ridge Less likely to show enlargement of the internal auditory canal (IAC) than vestibular schwannomas
MRI findings
Variable intensity on T2W images and either isointense or slightly hypointense to brain on T1W images Can be heterogeneous as a result of calcifications and cystic foci within the tumor Dural tail sign, characterized by enhancement of the dura adjacent to an enhanced primary lesion, possible on contrast-enhanced MRI
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CT
MRI FINDINGS
DURAL TAIL
LEFT IAC MASS WITH CALCIFIC.
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Imaging Studies (Contd.)

IAC MENINGIOMA WITH DURAL T.
IAC MENINGIOMA
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Epidermoid CT scanning findings
Hypointense to brain Do not enhance (enhancement suggests an associated malignancy or infection) Irregular margins with variable involvement of IAC Heterogeneous and hypointense to brain on T1-weighted images Homogeneous and isointense or hyperintense to brain on T2-weighted images Nonenhancing (allows them to be differentiated from schwannomas, meningiomas, and chondromas, which have similar intensity)
MRI findings
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EPIDERMOID CYST
NCE CT T1W MRI
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EPIDERMOID CYST (Contd.)

T1W MRI / T2W MRI
CNS UPDATE 2013 LUTH
02/08/2013
T2W MRI
DWI
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Facial nerve schwanoma CT scanning findings
Identical to vestibular schwannoma within the IAC except for possible anterosuperior IAC erosion or erosion of facial nerve canal in labyrinthine segment May have enlargement of geniculate ganglion and fallopian canal (more common)
MRI findings
Isointense or mildly hypointense to brain on T1WI Mildly hyperintense to brain on T2WI Enhances with gadolinium contrast
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Lower cranial nerve schwannoma Enlargement of jugular foramen with cranial nerve IX, X, and XI tumours Enlargement of hypoglossal canal with cranial nerve XII tumours
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Arachnoid cyst CT scanning findings
Typically smooth surface lesion isointense with CSF Non enhancing
MRI findings
Isointense or hypointense with brain on T1weighted images Hyperintense to brain on T2-weighted images
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Lipoma
Lipomas develop from mesenchymatous cells In the IAC bone erosion, but is atypical for CPA lipomas CT scans show a typical low density mass that has attenuation characteristics similar to adipose tissue (-50 to -100HU). At MRI lipomas appear similar to fat, hyperintense, compared to brain, on T1-weighted images and hypointense on standard T2-weighted spin echo frequencies. With gadolinium no enhancement. DD, hemorrage or highly proteinaceous fluid. Fat suppression techniques in T1-weighted scans can be used to confirm the diagnosis, when it changes from the high signal intensity to an isointense aspect
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Lipoma (Cond.)
T1W T1W
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Lipoma (Contd.)
Coronal STIR MRI. Fat suppressed
Contrast enhanced T1W + Fat Suppression / T1W
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Cerebral angiography
Cerebral angiography is used when involvement of a large vessel is suspected or preoperative embolization is required. It is used to assess patency in vessels encased by tumor. Balloon test occlusion can aid in determining likelihood of catastrophic stroke if internal carotid artery sacrifice is contemplated. Preoperative embolization can be performed in vascular tumors to effectively decrease amount of blood loss
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Treatment
Surgical intervention remains the most definitive means for complete tumour removal for most of these lesions. Radiation therapy has gained popularity to arrest tumour growth with specific tumour types and is also an option for patients who are unwilling or medically unable to undergo surgery
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Contraindications
Occasionally, complete resection is impossible because of the intimate involvement of surrounding structures, which may cause unnecessary morbidity if complete excision is attempted, and planned partial resection is considered.
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WORK UP OF PATIENTS WITH CPA/IAC TUMOURS

Audiovestibular assessment. Audiology and vestibular tests should be concurrently evaluated using air conduction and bone conduction threshold testing to assess for sensorineural versus conduction hearing loss. MRI: T1W & T2W with or without contast; DWI CT Scan: CE / NCE
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MORE IMAGES Ac.Neur.
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IAC MASS
Glomus tympanicum (arrow) in a patient with right-sided pulsatile tinnitus. Axial CT image demonstrates lobulated soft-tissue attenuation in the middle ear overlying the cochlear promontory.
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Aberrant course of the ICA presenting with pulsatile tinnitus. A, CECT shows an abnormal lateral course of the right ICA through the middle ear ( white arrow), destruction of the overlying bony plate. B, AP from the MR angiogram shows decreased caliber and lateral deviation of the aberrant ICA on the right reversed-7 sign (black arrow).
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Paget disease of the skull. Axial CT scan demonstrates diffuse expansion and sclerosis of the bones of the skull base. Note the sparing of the maxillofacial bones, which is a helpful in differentiating it from fibrous dysplasia.
Temporal bone Meningioma. Axial CT image demonstrates diffuse sclerosis of the temporal bone and soft-tissue attenuation (black arrows) in the left mastoid and middle ear cavity. B, Axial enhanced MR image showing enhancement within the left middle ear (white arrow).
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THANK YOU & BEST OF LUCK

Imaging Approach To & Interpretation of Findings in

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Anatomy of the CPA & IAC

Anatomy of the CPA & IAC (Contd.)

Anatomy of the CPA & IAC (Contd.)

Anatomy of the CPA & IAC (Contd.)

Anatomy of the CPA & IAC (Contd.)

Anatomy of the CPA & IAC (Contd.)

Anatomy of the CPA & IAC (Contd.)

Anatomy of the CPA & IAC (Contd.)

Anatomy of the CPA & IAC (Contd.)

Acoustic Neuroma (Vestibular Schwanoma)

Imaging Studies (Contd.) Site of acoustic N/Intracanalicular Schw.

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Acoustic Neuroma (Vestibular Schwanoma)(Contd.)

Pathophysiology Meningioma (Contd.)

Pathophysiology Meningioma (Contd.)

Pathophysiology - Epidermoid (Contd)

Pathophysiology - Epidermoid (Contd)

Facial and lower cranial nerve schwannoma:

Presentation of CPA tumours

Presentation of CPA tumours (Contd.)

Presentation of CPA tumours(Contd.)

LEFT IAC MASS WITH CALCIFIC.

Imaging Studies (Contd.)

Imaging Studies (Contd.)

EPIDERMOID CYST (Contd.)

CNS UPDATE 2013 LUTH

Imaging Studies (Contd.)

Imaging Studies (Contd.)

Imaging Studies (Contd.)

WORK UP OF PATIENTS WITH CPA/IAC TUMOURS

MORE IMAGES Ac.Neur.

THANK YOU & BEST OF LUCK

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