P-neuro

Question 1

young child w ET and RAPD
-description
-ddx
-eval

Answer 1

anomalous (small) optic disc w double ring sign

-optic nerve hypoplasia:
–VA nml to LP depending on if macular fibers are involved -> VF defects, strab, amblyopia, nystagmus
–can be associated w CNS abnormalities (if absent septum pellucidum, then DeMorsier syndrome -> severe endocrine abnormalities)
–can be idiopathic or caused by maternal DM1 or ingestion of phenytoin/quinine/LSD/EtOH
- optic nerve atrophy (genetic or tumor)
- optic nerve coloboma

hx: onset, progression (?prior exams), sxs (vision, strab, nystagmus, FTT, stunted growth, developmental delays, seizures) and RFs (maternal DM1 or drug ingestion during pregnancy)

exam: may need EUA
-ocular VS (alignment, nystagmus)
-nerve exam
-foveal hypoplasia

testing: OCT nerve (thin), neuroimaging of brain and orbit to look for a myriad of cortical malformations, including absent septum pellucidum (picture)

Question 2

Young child w optic nerve hypoplasia and this MRI
- dx?
- management
- counsel

Answer 2

optic nerve hypoplasia + absent septum pellucidum = DeMorsier Syndrome

maximize vision: CRx, patching, strab

Endocrine eval and pituitary hormone replacement (growth hormone, TSH, ACTH)

VA can range from 20/20 to NLP
-If hypoplasia alone, usually can lead productive lives
-If CNS abnormalities, more likely intellectual and other disabilities. Need close f/u. Can stop worrying about hormonal derangements after puberty.

Question 3

description
ddx
eval

Answer 3

blurring of disc margins without vessel obscuration or disc heme

-pseudopapilledema (optic disc drusen): examine fundus of parents
-papilledema: TVO x sec, pulsatile tinnitus, positional headaches, weight gain, drugs (steroids, OCP, vit A, Li, tetracycline / cipro / cyclo)
-papillitis: pain w movement

-exam: ocular VS, nerve exam (color vision, RAPD)
-testing: VF (arcuate defect), Bscan on low gain (hyperfluorescence at nerve head). Can also consider CT to look for calcifications, optic disc autofluorescence (picture-hyperfluorescent nodules in normally dark disc)

Question 4

dx
mgmt

Answer 4

optic disc drusen

-normal VA but VF defect and at rare risk for NAION / vein occlusion
-follow annually w VF and disc photos

Question 5

description
ddx
eval

Answer 5

abnormal disc vessels that appear to be optociliary shunts

ddx = causes of optociliary shunts = chronic poor drainage of CRV
- optic nerve sheath meningiomas (> sphenoid wing meningioma, optic nerve glioma though gliomas are more common in children): chronic progressive loss of vision, decreased color vision, RAPD, VF defects, proptosis, decreased motility, pain, lower lid edema and disc edema
- chronic papilledema
- prior CRVO: acute vision loss
- glaucoma

History: onset, progression (acute vs. chronic), sxs (pain, lower lid edema, vision loss, color vision, VF defects, proptosis, decreased EOM), RFs (FHx, NF, h/o IIH / CRVO / glaucoma)

exam:
-ocular VS
-nerve exam (color, hertel / resistance to retropulsion, VF)
-NF exam: CALBORN (2/6 for dx)
-?glaucoma/CRVO

testing: MRI (tram track = ONSM, a/w NF2; fusiform / kinked optic nerve = optic nerve glioma, a/w NF1)

Question 6

middle aged woman with optociliary shunt + MRI
- can worsen w what condition?
- dx?
- mgmt
- counsel

Answer 6

pregnancy

optic nerve sheath meningioma

- no biopsy as it can compromise vision
- observe if vision is stable and proptosis is not disfiguring
- Otherwise, fractionated radiation to improve vision and slow tumor progression / improve disc edema
- If tumor is extending intracranially / encroaching the chiasm -> neurosurgery
- counsel: natural hx is slowly progressive vision loss and proptosis over yrs / decades, VA 20/20-LP, usually observe w visual fields and neuroimaging

Question 7

description
ddx
eval

Answer 7

corkscrew conjunctival vessels

-carotid-cavernous fistula (no pain or discharge): whooshing sound, diplopia
-low flow: older patient w HTN
-high flow: old head trauma

-cavernous sinus thrombosis
-TED
-conjunctivitis: glaucoma gtts? discharge?
-episcleritis / scleritis: pain
-IOIS

hx: onset, progression, sxs (above), RFs (trauma, HTN)

exam:
-ocular VS
-exposure keratopathy due to proptosis
-nerve exam (color, Hertel, disc edema, tortuous vessels)
-auscultate orbit for bruit
-blood in Schlemm’s canal

test: CTA / MRA to look for enlarged superior ophthalmic vein. If negative, refer for angiography.

Question 8

corkscrew vessels + MRI (description)
dx
mgmt

Answer 8

enlarged superior ophthalmic vein

carotid-cavernous fistula

refer to neuro-radiology for closure of CCF
if HTN: mmanage
if exposure keratopathy from proptosis: lubrication
if elevated IOP: glaucoma gtts
potentially life threatening but good prognosis if managed

Question 9

DDX for various CN palsies:
1. CN3
2. CN4
3. CN6
4. CN7

Answer 9

Common:
- Ischemic: DM, cardiovascular, stroke, GCA
- Infectious: syphilis, lyme, TB
- Inflammatory / Infiltrative: IOIS, sarcoid, collagen vascular diseases (lupus, RA, Sjogren, scleroderma), mets
- Compressive: tumor
- Traumatic
- Congenital

CN3/4/6
-cavernous sinus syndrome (2/2 tumor, thrombosis, vascular), mucor

CN3
- pcomm aneurysm

CN6
- intracranial pressure

CN7
- temporal bone fracture, FN block, parotid surgery
- otitis, Ramsay-Hunt (VZV reactivation that affects facial nerve), Guillain-Barre
- cerebellopontine angle mass (tinnitus, vertigo, hearing loss, nystagmus)
- Bell’s

Question 10

Parks 3 step test

e.g.1 RHT, worse on right head turn and tilt, excyclotorsion
e.g.2 LHT, worse on left head turn and tilt, incyclotorsion

Answer 10

1. which eye is hyper
2. worse on left or right head turn
3. worse on left or right head tilt

e.g. 1: right SO
1. RHT = problem w right depressors or left elevators
2. worse on right head turn = problem with left gaze muscles
3. worse on right head tile = problem w OD intortors or OS extorters; SINRAD: superior intort, rectus adducts

e.g. 1: would be left SO except incyclotorsion so actually skew (vertical misalignment 2/2 asymmetrical otolithic input to ocular motor nucleus)

Question 11

binocular diplopia

• ddx
• eval

Answer 11

• nerve palsy: use parks 3 step to determine which nerve. Etiology congenital, ischemic or traumatic.
• skew deviation: vertical misalignment 2/2 problems w inner ear and its afferent projections, most commonly MS or brainstem lacunar infarct
• myasthenia gravis: variable strabismus.
• TED

-hx: onset (old photos), progression, sxs (monocular vs. binocular, horizontal vs. vertical, intermittent vs. variable vs. constant; head tilt), RFs (trauma / stroke / MS / cardiovascular disease / DM / HTN / thyroid / MG)

-exam:
–ocular VS, hertel
–Parks 3 step + double maddox to meausre cyclotorsion (5 if unilateral CN4 palsy, 10 if bilateral; record if the eye is in / ex: read straight off the device so that if patient rotated 10 out from vertical, that’s 10 ex), vertical fusion (10PD if congenital, 2PD if acquired),
–other CN: lid function
–macular dragging

-testing:
-if new onset in <45yo w/o trauma and vasculopathy or if multiple CNs involved -> neuroimaging
-T3, free T4, TSH, TSI
-anti-Musk, anti-AchR, ice/rest/ten

Question 12

CN4 palsy: mgmt
when to image?

Answer 12

Congenital
- decompensates in 4th or 5th decade. Same strategies as traumatic

Traumatic
- unlikely to self improve; offer strab surgery (SO tuck, harada ito to correct torsional component) once measurements stabilize for >6mo
- symptomatic mgmt: patch, fogging, prism

• if cause of CN palsy is unknown or presumed to be vasculopathic, follow every month. If no improvement within 3mo / new problems, then image.

Question 13

sudden onset Down and out eye + ptosis

ddx
eval
mgmt

Answer 13

CN3 palsy: ischemic vs. compressive (pcomm, cavernous sinus syndrome [CN3-6], mucor)
- hx: onset, progression, sxs (fever, headache), RFs (trauma, cardiovascular disease, recent illnesses, immunocompromise)
- exam: ocular VS (motility spares abduction due to CN6), anisocoria (if enlarged pupil, then likely compressive such as PCOM aneurysm because the parasympathetic fibers are externally located), neuro exam
- testing: unless complete (ophthalmoplegia + ptosis) + pupil sparing + clear vasculopathic, emergent CTA and MRI brain and orbit
-mgmt:
– if mucor (answer): ENT, ID, NSGY
– if pcomm (prompt): IR, NSGY

TED: never ptosis
- exam: ?RAPD, hertel, lid lag

MG: never pupil involvement
- hx: variable; worst at night
- exam: cogan lid twitch, lid weakness, upgaze fatigue, variable diplopia
- testing: ice, rest, tensilon (have atropine ready for side effects), anti-Musk, anti-AchR

Horner: small pupil w ptosis, not large

Question 14

sudden onset painful CN3 palsy with anisocoria - dx and mgmt

Answer 14

PCOMM aneursym
- stat CTA/MRA
- neuro consult for immediate intervention to prevent massive subarachnoid hemorrhage

Question 15

60M w DM and HTN p/w diplopia
ddx and eval
imaging?

Answer 15

-CN6 palsy: congenital or acquired (ischemic, traumatic, increased intracranial pressure, cavernous sinus)
-MG
-TED
-orbital fracture of medial wall causing restriction

-hx:
–onset, progression, recurrence, constant vs. variable vs. intermittent
–Sxs: diplopia - monocular, binocular, horizontal, vertical, constant, intermittent, variable with fatigue or throughout day; scalp tenderness, jaw claudication, fever, weight loss; pain w motility
–RFs (trauma, cardiovascular disease, diabetes, HTN, trauma, thyroid disease, recent illness, immunocompromise)

-exam: ocular VS, hertel, lid, saccade + smooth pursuit, upgaze fatigue, VF, disc swelling or pallor, neuro exam, blood pressure
-labs: acetylcholine receptor antibody, thyroid lab, ESR/CRP +/- temporal artery biopsy
-testing: if bilateral CN6 palsy or multiple CNs involved, h/o cancer or optic disc edema, then order imaging

Question 16

mgmt of CN6 palsy

Answer 16

-medicine referral for management of underlying disease
-symptom mgmt: patching, fogging, prisms
-90% of ischemic CN6 resolve in 90 days so follow monthly to resolution. If no improvement within 90 days or if progression of symptoms (involvement of new nerves), then image
-if symptomatic and negative workup, then strab surgery after stable measurements for >6mo

Question 17

ddx
eval

testing if:
-infectious
-inflammatory
-stroke
-trauma
-when to image?

Answer 17

• bell’s palsy (dx of exclusion)
• infection (lyme, syphillis, otitis, Ramsay Hunt, Guilain-Barre)
• infiltrative (sarcoid, mets, collagen vascular disease like lupus, RA, AS)
• ischemic (HTN, DM, stroke)
  - cerebellopontine angle mass: tinnitus, vertigo, hearing loss, nystagmus
• trauma (temporal bone fracture)
• iatrogenic (FN block, parotid surgery)

eval: follow algorithm
–image if lower face only or bilateral

• hx: onset/progression/recurrence, sxs (pain, hearing loss, tinnitus, vertigo, diplopia), RFs (trauma/surgery/disease
• exam: ocular VS, lid position / lag / orbic strength, K health, upper vs. lower face, unilateral vs. bilateral, complete neuro exam including 5, 6, 7, 8, motor weakness, ataxia, balance, lymph nodes, skin rashes
• test:
  –if infection: lyme, EBV, HIV, HZV, RPR, VDRL
  –if infiltrative: sarcoid (ACE, lysozyme, chest CT), collagen vascular disease (ESR, ANA, ANCA)
  –if stroke: MRI, carotid dopplers, ECHO, neuro consult
  –if trauma: CT
  –if cancer or duration >3mo = MRI

Question 18

mgmt of bell’s palsy

Answer 18

acyclovir + pred x 14 days if within 3 days of symptoms onset (MUST ENSURE TB NEGATIVE PRIOR TO STEROIDS)

K: lubricate, gold weight / tarrsorrhaphy

most regain use over 3-6mo. Follow monthly until then. If no improvement or new symptoms after 3mo: image + neuro consult

Question 19

Anisocoria algorithm and eval
mgmt for Horner’s

Answer 19

hx: onset, progression, sxs, RFs

Horner:
-hx: congenital vs. acquired, trauma, chiropractic, pain, neuro deficits, SOB / cough
-exam: ptosis with small pupil, dilation lag, heterochromia suggests congenital
-ddx: hydroxyamphetamine: dilates 1/2, not 3
–1st order (hypothalamus -> C8/T2): stroke (vertebrobasilar insufficiency), MS, brain tumor
–2nd order (exit spinal chord -> lung apex -> superior cervical ganglion): lung tumor (pancoast), mets
–3rd order: migraine, carotid dissection, herpes, trauma / forceps birth, Tolosa-Hunt (idiopathic inflammatory -> headaches and ophthalmoplegia),
-testing: emergent CTA/MRA, include lung (if non-revealing, gold std for dx carotid dissection is angiography)
-mgmt: if carotid dissection -> neurosg or IR for intravascular stenting w anticoag vs. observation
-prognosis: horner’s may or may not resolve upon correction of carotid dissection

CN3:
-hx: diplopia
-exam: ptosis, ophthalmoplegia

For Adie’s:
-exam: parasympathetic denervation -> vermiform movement, LND
-mgmt: observe

iris tear/iritis
-exam: AC cell, posterior synechiae

Question 20

DDX for disc edema

Answer 20

Disc edema
- Ischemic: NAION, GCA, diabetic papillitis, malignant HTN, CRVO
- Infectious: syphilis, lyme, TB, bartonella
- Inflammatory / Infiltrative: IOIS, sarcoid, collagen vascular diseases (lupus, RA, Sjogren, scleroderma), mets
- Idiopathic: IIH
- Sinus venous thrombosis
- Compressive: glioma, meningioma, other tumors
- MS

Question 21

description
ddx and eval

Answer 21

disc edema

-idiopathic intracranial hypertension (IIH)
–RFs: weight gain, vit A, steroid (use AND withdrawal), OCP, Li, tetracycline / cipro / cyclo
–s&s: positional headache, TVO (x secs), diplopia (2/2 CN6 palsy), pulsatile tinnitus, no spontaneous venous pulsation
–modified dandy criteria for dx: S&S + papilledema + normal MRI + elevated opening pressure

-intracranial mass
-venous sinus thrombosis

-malignant HTN: check BP

-papillitis (optic neuritis, diabetic, uveitis, leukemic infiltration): pain w eye movement, other intraocular inflammation

exam: ocular VS, nerve exam (color plates, edema, SVP)

-testing:
–RNFL and HVF 30-2
–MRI/MRV, if wnl, then LP for opening pressure (>25 in adults, >28 in children) and cytology
–B-scan to r/o drusen

Question 22

mgmt of IIH

Answer 22

• decrease RFs: weight loss, stop tetracycline/OCP/vitA
• diamox 1-2g/day -> if fails, then ONSF (for visual symptoms) or VPS (for systemic symptoms)
• RNFL HVF 30-2 for monitoring rolls eyes @ fat: arcuate > enlarged BS

-Usually good prognosis with weight loss and arrest of offending agent and diamox.
-Counsel side effects of diamox (polyuria, metallic taste, parethesias, GI upset)

Question 23

pallid disc edema
ddx
eval

Answer 23

AAION = GCA
-hx: onset, progression, sxs (fever, weight loss, malaise, arthralgia/myalgia, proximal muscle weakness, temporal pain, scalp tenderness, jaw claudication, contralateral episode)
-exam: ocular VS, temporal cords, VF (GCA is a big deal - altitudinal defect), nerve palsies, CRAO / cherry red spot
-testing: elevated ESR/CRP/platelet though normal labs do not exclude diagnosis (ESR cutoff age/2 for M, (age+10)/2 for F); FA with patchy and delayed choroidal filling

NAION: must exclude GCA first in this age group.

optic neuritis: rare in the elderly

retinal vein occlusion

infectious (syphillis, TB, lyme) / infiltrative (ONSM)

Question 24

Mgmt of GCA

Answer 24

-PO pred 1mg/kg/daily -> tocilizumab
-bilateral temporal artery biopsy 2-3cm within 14days of starting PO pred
-rheum referral
-taper based on symptoms and ESR, typically months-1 year
- prognosis poor for involved eye but treatment important to protect fellow eye (95% involvement without treatment) and other medium-large vessels such as cardiac ones

Question 25

young female w decreased vision and pain w eye movement x 2 days, normal nerve -ddx -eval

Answer 25

retrobulbar optic neuritis retrobulbar infiltrative process PION optic nerve compressive lesion hx: onset, progression, recurrence, family / personal h/o MS, symptoms worse w exercise or heat? recent illnesses, rashes exam: -ocular VS -nerve exam (color plates, hertel, RNFL, visual field [**MS is a big deal - altitudinal or enlarged blind spot]**, -neuro exam testing: --MRI brain/orbits with and without contrast --CBC, FTA/RPR, TB, ACE, lysozyme, chest CT

Question 26

Mgmt of optic neuritis

Answer 26

- **if symptom onset within 14 days, optional IV steroids 1g x 3days -> PO pred 1mg/kg/day x 11 days -> taper over 1 wk** - NO PO steroids alone as they can increase recurrence Counsel **- 90% return to baseline vision within 90 days** - IV steroid can hasten recovery but no effect on ultimate VA; can decrease MS within 2 yrs but no effect by 3yrs - if 0 lesions on MRI: 25% develop MS - if 1+ lesions on MRI: 75% develop MS - if 2+ lesions on MRI: IFNb can decrease MS by 50% @3yrs - **follow weekly** then extend as patient improves

Question 27

Painful red and swollen left eyelid ddx eval

Answer 27

orbital cellulitis preseptal cellulitis orbital pseudotumor orbital **mass** -hx: onset, progression, --sx: fever, pain, discharge, VA change, **diplopia** --RFs: trauma, sinusitis, DM or other forms of immunocompromise -exam: -- ocular VS -- orbital signs (decreased VA, RAPD/dyschromatopsia, proptosis, painful motility) -- temp, **nasal** exam to look for black eschar (Mucor) - testing: CBC, **Bcx**, CT (orbital fat stranding, subperiosteal abscess, mass)

Question 28

painful red left eye lid: dx and mgmt

Answer 28

orbital cellulitis (fat stranding, proptosis) likely due to sinusitis (no periosteal abscess) - IV unasyn, monitor daily, **once improving** - > PO augmentin - **ENT** consult for sinusitis - **if periosteal abscess, consider draining** - good prognosis

Question 29

ddx for proptosis

Answer 29

VEIN vascular: CC fistula, hemangioma, hemangiopericytoma (faster growing, heterogenous cysts) lymphangioma, orbital varix (intermittent proptosis that is position dependent and increased with Valsalva maneuver, intralesional calcifications) Endocrine: thyroid Infection/inflammatory: **orbital cellulitis / abscess**, IOIS, sarcoid Neoplasm: nerve (glioma, ONSM, **neuroblastoma), muscle (rhabdo)**, lacrimal gland (pleomorphic adenoma / adenocarcinoma, adenocystic carcinoma), mets

Question 30

description ddx eval

Answer 30

bilateral proptosis + conj injection + scleral show + lid retraction w lateral flare TED - hx: onset, progression, sxs (VA, pain, DES, hyperthyroid [heat intolerance, palpitations, weight loss]), RFs (smoking, level of control of thyroid disease) - exam: --ocular VS --nerve exam (color vision, disc swelling / pallor) --orbital exam (hertel, resistance to retropulsion, prism) --**K exam (lid lag, exposure)** --**CAS (baseline orbital pain, motility orbital pain, swelling/redness of eyelid / conj / caruncle; change in VA / proptosis / motility)** - tests: TSH, T3, **free** T4, thyroid stimulating immunoglobulin, CT to look for muscle enlargement with tendon sparing, RNFL and HVF to monitor nerve orbital tumors/ mets **Parinaud** dorsal midbrain syndrome = sylvian aqueduct syndrome - exam: upward gaze palsy, convergence retraction nystagmus, **light-near dissociation**, and bilateral lid retraction - tx the underlying cause (**pinealoma, congenital aqueductal stenosis, demyelination, thalamic stroke**) Idiopathic orbital inflammatory syndrome

Question 31

TED mgmt counsel

Answer 31

-symptomatic management: K lubrication, **HoB elevation + cold compresses to decongest lid and conj, lid tape, prisms for symptomatic diplopia, gtts for IOP** - if inactive / no optic nerve compromise (no decreased VA, no RAPD, normal color plates, no disc swelling or pallor): endo referral for mgmt of underlying thyroid disease - if significant active disease without nerve compromise: **teprotumumab infusions** - if nerve compromise: stat IV steroid -> orbital wall decompression, radiation - follow **monthly** to ensure no nerve compression - **generally gradual progression x1-2yrs before stabilizing.** - STOP smoking - Long term complications: strabismus, cornea -sequence of repair: orbital wall decompression -> strab repair -> lid repair

Question 32

young boy w upper eyelid fullness that enlarged over 14 days

Answer 32

ddx - rhabdomyosarcoma (violet appearing): sudden onset painless proptosis - chalazion - lymphangioma (pic): fever, changing proptosis w valsalva or URIs - metastatic neuroblastoma - cellulitis - plexiform neurofibroma hx: onset, duration, progression, associated symptoms (fever, pain, lid swelling, discharge, vision change), triggering events (URI, sinusitis, trauma, h/o cancer) exam: -ocular VS -lid exam (position, lag, painful or warm to touch) -nerve exam (color vision, VF, nerve swelling or pallor) -orbital exam (hertel, **cranial nerve including K and trigeminal hypoestehsia 2/2 concern for orbital tumor, chorioretinal folds 2/2 compression of globe**) -systemic (temperature, lymph nodes in head and neck) testing: CBC (cellulitis), CT for mass/infection

Question 33

young boy w sudden proptosis -image description -dx -mgmt

Answer 33

CT scan showing homogenous nasal mass without cyst, globe displacement and possible bony erosion rhabdomyosarcoma (2/2 hx + location on CT) -excisional biopsy (necessary for dx) -mets workup (liver, bone and lungs) -refer to onc for radiation and/or chemo -**prognosis dependent on type but usually good (95% cure rate) if no extraorbital involvement.** Emphasize need for collaborative care

Question 34

post trauma constricted VF w crossing isopters ddx eval mgmt

Answer 34

- non-organic - glaucoma - retinal dystrophy - **chronic papilledema** hx: onset, duration, progression, severity, associated symptoms, triggering events, **mental health**, **neuro** exam, meds exam: full exam to rule out organic causes testing: - OKN drum, CF funneling, stereopsis, - if any suspicion, given h/o trauma, consider imaging (OCT, MRI, ERG, VEP) Counseling: share the good news that there is no structural abnormalities so we anticipate a full recovery f/u 2wks if uncertain

Question 35

Localization of visual fields A. central scotoma B. Monocular vision loss C. Bitemporal hemianopia D. Homonymous hemianopia E. superior quadranopia F. inferior quadranopia G. central sparing homonymous hemianopia

Answer 35

A. ipsilateral retina B. ipsilateral ON C. optic chiasm (**posterior lesions are more central**) D. contralateral optic tract and beyond (**bowtie atrophy**) E. contralateral inferior radiation and bank F. contralateral superior radiation and bank G. contralateral occipital lobe (posterior cerebral artery is occluded; if no central sparing then tract is also involved)

Question 36

top left VF defect description ddx eval mgmt

Answer 36

Junctional scotoma - chiasmal tumor (pituitary - pushes nerve from below, craniopharyngioma - pushes nerve from above and posterior, meningioma, glioma) - **vascular: aneurysm or stroke** hx: onset, progression (?prior VFs), sxs (), RFs (endocrine abnormalities [galactorrhea, weight gain/loss, buffalo hump change in menstrual cycle], NF1 CALBORN, **neuro deficits [speech, mental status, sensory, motor functions]**, cancer) exam: -ocular VS -nerve exam: color vision, nerve pallor/edema testing: **-pregnancy test (tumor can enlarge)** -MRI to look for mass / vascular abnormalities **-pituitary labs (GH, PRL, ACTH, FSH, LH, TSH)** mgmt: - nsgy for excision - onc for adjunct radiation / chemo - endo for pituitary abnl - f/u 6mo - counsel: benign tumors w good prognosis but risk of recurrence

Question 37

mgmt of chiasmal tumor

Answer 37

-neurosurgery referral for biopsy and potential surgical resection -heme/onc to determine if chemo/radiation is needed -if pale nerves: discuss likelihood of permanent vision loss and refer to low vision services -monitor w serial HVFs -discuss need for collaborative mgmt and regular followup meningioma or glioma: -**genetics referral to rule out NF** - glioma: chemo/radiation; surgery only if no vision remains - mengingioma: radiation, surgical debulking (no chemo 2/2 limited blood supply) pituitary: -co-management with endocrine and neurosurgery -counsel that the visual field defect may or may not be permanent upon excision of pituitary mass

Question 38

NF1 dx criteria

Answer 38

2+ of CALBORN cafe au lait axillary freckling / **astrocytic hamartoma (mulberry like - prompt)** lisch nodules bony deformity (no greater sphenoid wing) optic glioma relative affected neurofibromas

Question 39

optic nerve glioma vs. ONSM mgmt

Answer 39

Both: - If not visually significant, observe - monitor w VF and neuroimaging - Counsel: Both are slowly progressive, **VA 20/20-LP** + proptosis If encroaching on chiasm or visually significant: - glioma: chemo/radiation; surgery only if no vision remains - mengingioma: radiation, surgical debulking (no chemo 2/2 limited blood supply)

Question 40

description ddx eval

Answer 40

s-shaped deformity of right upper eye lid causing mechanical ptosis plexiform neurofibroma rhabdomyosarcoma / sarcoma neuroblastoma (proptosis, ecchymotic eyelid, Horner's pupil, image) hx: onset, duration, progression, associated sx (pain, vision change) exam: ocular VS, color vision, VF, refraction because of mechanical astigmatism, CALBORN, optic nerve pallor/edema **testing: urine/blood catecholamines and metabolites HVA/VMA (r/o neuroblastoma)**, CT (sphenoid wing dysplasia, optic glioma)

Question 41

Plexiform neurofibroma mgmt

Answer 41

- Rx to maximize vision given mechanical eyelid deformity. Discuss amblyopia and importance of wearing glasses - **biopsy**/excision of mass but counsel regarding difficulty of complete excision / need for additional procedures given vascularized and infiltrative nature of lesion. - **refer to onc for radiation/chemo** - discuss associate w NF1 and genetic workup

Question 42

description ddx eval

Answer 42

left eye **hypoglobus**, proptosis, no eyelid erythema mucocele (above medial tendon) encephalocele (above medial tendon, image) dacryocystocele (below medial tendon) orbital mass (e.g. dermoid) **sinus-originating tumor (e.g.SCC)** -hx: onset, duration, progression, associated sx (pain, diplopia, decreased vision), triggering events (trauma, sinusitis, dacryocystitis) -exam: ocular VS, hertel, strab measurement, **optic pallor/edema, choroidal folds (globe compression)** -testing: CT/MRI

Question 43

description dx mgmt

Answer 43

**circumscribed cystic mass that originates from the sinus with bony erosion** mucocele -refer to ENT for endoscopic evacuation: urgent if RAPD/HVF. -If pyocele: intraoperative culture and sensitivities -Counsel risk of recurrence, residual strab needing surgery and need for sinus management

Question 44

MVA, unconscious w OS sluggish pupil description ddx eval

Answer 44

axial CT showing tenting of the posterior globe = "tear drop" sign -retrobulbar hemorrhage -**orbital mass/tumor** hx: mode of injury exam: ocular VS (demars for accurate IOP if eyelids swollen), forced ductions if no suspicion for open globe, lid injury, K injury, iris injury, uveitis, hyphema, lens dislocation, VH, RD/commotio, nerve pallor/edema testing; **CT for foreign body (metallic, organic)** and fractures

Question 45

mgmt of retrobulbar hemorrhage

Answer 45

- if IOP >45: canthotomy + cantholysis -> if persistent IOP elevation, make sure canthal tendon is indeed cut -> if still persistent, fracture inferior orbital wall (transconjunctival or through the sinus) - counsel risk for re-bleeds; admit and observe for 48hrs

Question 46

7yo M s/p trauma OS p/w diplopia description ddx eval mgmt

Answer 46

CT coronal scan showing fracture of inferior orbital wall with **hemo-sinus** and likely muscle entrapment orbital fracture w entrapment orbital fracture w/o entrapment -hx: mode of injury, onset, duration, progression of sx, associated sx (VA, pain, diplopia, flashes), last meal -exam: ocular VS, **enophthalmos**, forced ductions if restricted EOM (looking for restriction or activation of oculo-cardiac reflexes - decreased HR, nausea), full exam for other signs of trauma -NPO -> urgent repair in kids (24-48hrs) 2/2 risk of greenstick fractures that impinge muscle; **10-14 days for adults to wait for swelling to go down (repair indicated if bothersome diplopia, >50% of floor, enophthalmos >2mm)** -> forced ductions + abx in OR -> plate if large fracture -f/u 1wk to ensure healing and check EOM -counsel risk of infection due to surgery/plate, diplopia, strab, enophthalmos, need for additional surgeries

Question 47

description ddx eval dx and mgmt

Answer 47

-coronal MRI showing hyperintense region in ST left orbit with extension into the bone - primary tumor (lymphoma, lacrimal gland tumor) - **met** - **vascular anomaly** - inflammatory (IOIS, collagen vascular) -hx: onset, duration, progression, associated sx (VA, pain, diplopia), triggers/risk factors (h/o cancer, smoking) - exam: ocular VS, color vision, hertel, VF, **systemic symptoms (night sweats, weight loss)** - testing: **CBC (r/o lymphoma),** c-ANCA (r/o wegener's), full body scan / PET after consultation w heme/onc given age -primary tumor based on age and slow progression; lymphoma most common in 60+yo. - biopsy via lateral orbitotomy -> path w special stains for lymphoproliferative malignancies -if lymphoma: local external beam radiation usually curative for lesions confined to the orbit; onc referral for systemic workup (CBC, pet/CT, MRI, bone marrow biopsy) +/- chemo -good prognosis if local -if systemic, ok prognosis if responsive to chemo

Question 48

optic atrophy eval ddx mgmt for hereditary

Answer 48

hx: onset, progression, sxs (pain, unilateral vs. bilateral [dominant] vs. sequential over wks [Leber] VA decrease, steady decline [dominant] vs. can improve [leber], ataxia/**intellectual disability [Recessive]**), RFs (**nutrition, GI issues, substance**, medications, FHx) Exam - VA, IOP, P, color plate - DFE: disc pallor DDx - Toxic optic atrophy (alcohol, ethambutol, isoniazid, plaquenil, lead, thallium) - Metabolic = hereditary optic neuropathy - Glaucoma - Compressive **- h/o papilledema, NAION, optic neuritis** Management - RNFL (temporal thinning for hereditary OA), HVF - CBC, B1, B12, folate, lead, thallium - MRI - idebenone for Leber; **B12/C/lutein/CoenzymeQ10 for AD** - stop smoking - severe vision loss -> vision services - Leber is tranmitted via mitochondria

Question 49

Myasthenia Gravis eval ddx for ptosis mgmt

Answer 49

History -Onset, progression / variability -sxs: dyspnea, dysphagia, dysarthria, all worse at night -RFs: none. But there are aggravating factors: **stress (infection, pregnancy, extreme temp)**, thyroid, thymoma Exam -ocular VS -lid: position, levator function, **orbic function**, upgaze fatigue, cogan lid twitch (prolonged downgaze -> lid twitches in primary gaze) -eval anisocoria to r/o Horner's -eval EOM (upgaze fatigue) and alignment (variable strab) Tests: ice (ice x 5min -> temporarily improves ptosis due to slowed acetylcholinesterase inhibitors), rest / sleep, tensilon (acetylcholinesterase inhibitor; have atropine ready for side effects), anti-MuSK, anti-AchR (r/o thymoma FP w CT chest in adults because all kids have thymus), single fiber EMG DDx - MG - 3rd or horners - Aponeurotic - Traumatic - Mechanical Management - Mestinon = pyradostigmine = acetylcholinesterase inhibitor -> parasym side effect - **if uncontrolled, Steroids, IMT** - **if strab: Patching, prism** - if thymoma: Thymectomy Education - If develops systemic sx, need to go to ED. **2/3 of ocular MG become generalized MG**. - Side effect of treatment - counsel associations w thyroid disease and thymoma

Question 50

description eval ddx mgmt

Answer 50

(**Segmental** or diffuse) disc edema, contralateral eye disc at risk hx: onset, progression, sxs (sudden painless loss of vision; r/o GCA w scalp tenderness, jaw claudication, malaise, weight loss), RFs (cardiovascular disease, DM, **OSA**, amiodarone, viagra) exam: -ocular VS -nerve exam (colors, VF, CDR, heme, swelling, pallor) testing: -RNFL, HVF (altitudinal), **FA (normal choroidal filling, mildly delayed disc filling)** -CBC, ESR/CRP DDx: - NAION - AAION - Optic neuritis - CRVO - Papillitis (DM) Management - Avoid antihypertensive at sleep - Treat underlying dz Education - 40% can show improvement, 40% stable, 20% worse. - Fellow eye at risk 15% **at 5 years** **- f/u 2 months, if worse, then MRI**

Question 51

mgmt of orbital hemangioma

Answer 51

if asymptomatic: observe otherwise (proptosis, diplopia, compressive neuropathy): orbitotomy -> complete excision monitor w ON exam, Hertel, HVF

Question 52

Important to rule out what entity in amaurosis fugax

Answer 52

GCA

Question 53

Counselling for adie's

Answer 53

-idiopathic -improves or recovers over months