MORAN - Neuro-ophth OKAP

What are the contents of the superior orbital fissure...
- Above the annulus of Zinn?
- Within the annulus of Zinn?
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Terms in this set (32)
What are the contents of the superior orbital fissure...
- Above the annulus of Zinn?
- Within the annulus of Zinn?
Superior to the annulus
- Branches of V1 (Lacrimal + Frontal)
- CN4
- Superior ophthalmic vein

Within the annulus
- CN3 (inferior division + superior division)
- V1 (nasociliary nerve)
- CN6
- Sympathetic root of ciliary ganglion
What are the structures that travel within the cavernous sinus?
CN3, 4, 6, V1, V2, ICA and sympathetics
What are the 3 most common intraconal tumours?
- Optic nerve meningioma
- Cavernous malformations
- Optic nerve glioma
What is the typical appearance of an optic nerve sheath meningioma on imaging?
Tram-track sign
Optic nerve glioma
- What is the most likely type of tumour in a child with optic nerve glioma?
- What is the most likely cell in adults? What is the prognosis?
- What is the systemic association in children with an optic nerve glioma?
- What is the classic cell type that you see in optic nerve gliomas?
- Astrocytoma in kids
- Glioblastoma in adults, usually fatal within 1 year
- Systemic association = NF1 in children (10-50%)
- Rosenthal spindle fibers
What kind of mass do you think of when you see a, intraconal mass displacing the optic nerve with a dark pseudocapsule and progressive enhancement?
- Cavernous hemangioma
What kind of VFD does an anterior communicating artery aneurysm typically cause?
- Isolated VFD inferiorly
Dominant optic atrophy
- When does it tend to present?
- What is the inheritance?
- What is the gene associated?
- What do the nerves typically look like?
- What kind of VFD tends to occur with dominant optic atrophy?
- Tends to present before 10 years of age
- Inheritance is autosomal dominant
- OPA1
- Temporal disc pallor OU
- VFD = bitemporal VFD, central/cecocentral scotoma
LHON
- What do you classically see at the optic disc in this disease?
- What are 5 systemic associations with LHON?
- What is the important thing to watch out for?!
- Circumpapillary telangiectasia
- Systemic: paraplegia, dementia, deafness, migraines, vertigo, spacticity
- Imporatnt to watch out for Wolff-Parkinson-White syndrome
ODD
- What is the typical inheritance?
- What is the typical pathology describing the ODD?
- What is the most common VFD in ODD? What are 2 other common VFDs?
- What are two disease with a higher incidence of optic nerve drusen?
- Usually autosomal dominant, but can be sporadic
- Basophilic calcareous, laminated acellular bodies of different sizes and shapes
- Inferonasal defect most common. Others: concentric narrowing, arcuate scotoma
- Pseudoxanthoma elasticum + RP
What do you think of when you hear an alcoholic with stocking and glove peripheral neuropathy?Vitamin B12 deficiencyWhat are 5 medication-related causes of IIH?- Tetracyclines, lithium, accutane, vitamin A, steroid withdrawalWhat is your differential if you are suspecting atypical optic neuritis?Atypical optic neuritis - NMO - SLE - GPA - Syphilis - Sarcoid - Lyme - InfiltrativeONTT - What was the objective of the ONTT? - What was the 3 main findings of the study? - What is the risk of developing MS by 15 years after ON episode if you have a) no lesion on MRI? b) 1 or more lesions on MRI?- Objective = evaluate role of corticosteroids in tx of unilateral ON 3 Main Findings: - IV steroids 1 g daily x 3 days followed by 11 days oral taper results in FASTER recovery - IV steroids did NOT reduce rate of additional attacks or incidence of MS at 3 years compared to placebo - Oral prednisone alone had a higher risk of relapse than IV steroids - No lesion = 25% - 1 or more lesions = 72%Review the NMOSD diagnostic criteria for: 1) With AQP4-IgG 2) Without AQP4-IgG or Unknown AQP4-IgGWhat are 4 differentiating features between an optic nerve glioma and optic nerve meningioma on imaging?In a nuclear 3rd... - When is 1 pupil only involved? - Which superior rectus would be involved in a right nuclear 3rd? Which levator would be involved?- Nuclear 3rd = either both are involved, or neither! The EW nucleus supplies both pupils - Contralateral SR paresis, but bilateral ptosis because same subnuclears feeds both levatorsWhat are the 3 types of aberrant regeneration of CN 3?1) Muscle to muscle = co-contraction of MR and IO 2) Muscle to pupil = constriction on downgaze/adduction 3) Muscle to lid = Adduction/elevation/depression and eyelid retractionWhich is the only cranial nerve that decussates?CN4Where does CN6 exit the brainstem, and what is it adjacent to? What other similar structures exit here? - Briefly go over the rest of the course of CN6 until it reaches the cavernous sinus - Where does the petrous bone fit in to all of this? - What is Gradenigo's sign? What is Gradenigo's syndrome triad?- CN6 exits the PONS adjacent to the CEREBELLOPONTINE angle, where CN7 and CN8 exit as well - CN6 climbs over the clivus, through Dorello's canal, into cavernous sinus through the superior ophthalmic fissure - Part of Dorello's canal is in contact with the adjacent petrous bone - Gradenigo's sign = abscess of petrous apex (mastoiditis) - Gradenigo's syndrome triad —Otitis Media —CN6 palsy —Retro-orbital pain/trigeminal nerve pain- What do you see on histology when you biopsy someone with CPEO? - What would you see on electron microscopy?Histology = Ragged red fibers + mitochondrial proliferation Electron microscopy = inclusion body anomalies in mitochondriaWhat are the components of Kearns-Sayre Syndrome?CPEO + Pigmentary retinopathy + cardiac conduction abnormality (prolonged QT/heart block)Neuroblastoma - What is the definition of this? - Where does it originate? - What % of these patients have orbital involvement?Neuroblastoma = malignant tumour arising from any neural crest element of the sympathetic nervous system - Originates in the adrenal gland or sympathetic ganglion chain in the retroperitoneum or mediastinum - Orbital involvement in 20%A kid with proptosis, bruising, and nystagmus presents. What do you think of? - What are 2 tests you can do to diagnose it and what would you expect to see? - What age are neuroblastomas usually diagnosed?Neuroblastoma! Tests - Urinalysis —> Catecholamines in 95% - Incisional biopsy —> small round blue cells Usually diagnosed at age 2What are poor prognostic factors in neuroblastoma? (2)- Age of onset < 1 year - Bony metastasesWhat are the 3 locations of an aneurysm if you're suspecting a pupil-involving 3rd nerve palsy?- Anterior communicating artery - Posterior communicating artery - ICAHow does lyme disease normally present?- Optic neuritis or follicular conjunctivitisWhat CT finding are you classically looking for in a CC-fistula?Enhancing cavernous sinus + dilated SOVWhat vitamin deficiency is most likely to cause optic neuropathy?B12What vitamin deficiency is most likely to cause nyctalopia?Vitamin AWhat type of nystagmus is paraneoplastic syndrome associated with?OpsoclonusWhat is the inheritance of VHL?Autosomal dominant