P-pediatric Flashcards

1
Q

infant w photophobia + epiphora + blepharospasm
description
ddx
eval
mgmt

A

enlarged and cloudy cornea

-congenital glaucoma / sturgey weber
-STUMPED (sclerocornea, trauma, ulcers, metabolic, peters, PPMD, dermoid)
-Congenital hereditary endothelial dystrophy
-congenital hereditary stromal dystrophy

-hx:
–sx: onset (old photos), progression
–RFs: family hx, eye injuries (forceps), eye infections (maternal infections during pregnancy)

-exam: EUA likely needed
–ocular VS (nystagmus, strab as sign of poor visual function)
–specials for congenital glaucoma: K edema / pachymetry, K diameter >12mm, Haab striae, high iris insertion, CDR, CRx to determine myopic shift 2/2 progressive axial elongation
–specials for STUMPED: Vogt striae, scars, epi defects
–specials for sturgy-weber: choroidal hemangiomas, port-wine stains

Mgmt for congenital glaucoma
-IOP lowering drops and diamox, EXCEPT brimonidine given risk of CNS depression / bradycardia / hypotension / apnea
-surgery: goniotomy preferable but if no view, then trabeculotomy
-counsel
–complications: poor VA -> strab, amblyopia; K scarring
–needs lifelong management; no cure but can preserve useful vision

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2
Q

6F p/w white spot on eye, intermittent h/o blurred vision but no eye pain

description
ddx
eval
mgmt

A

early band keratopathy nasally and temporally

-juvenile idiopathic arthritis
-chronic inflammation (uvietis, sarcoidosis)
-high Ca (trauma, hypercalcemia / vit D tox -> GI problems)
-high PO4 / low K (hyperparathyroidism, renal disease)
-silicone oil (RD)

-hx:
–symptoms: onset, progression (pain, vision loss, bothersome)
–RFs: any of the above diseases

-exam:
–ocular VS
–confirm band K: deposits in interpalpebral zone start at limbal regions of 3 and 9, within Bowman’s layer
–special evals: KPs, cell, posterior synchiae, cataract, vitritis, RD
–look for systemic manifestations: arthritis, palpate thyroid
–testing: ESR, CRP, RF, ANA, Ca, PO4, ACE, lysozyme, chest CT

Management:
–refer to rheum
–tx any active inflammation (steroid, MTX, atropine for uveitis)
–if band K is vision limiting, then EDTA chelation
–f/u can range from 3mo to 12mo, depending on type (arthritic vs. systemic disease), ANA, age at onset and duration of disease
–counsel regarding glaucoma, cataracts, CME
–counsel regarding side effects of steroids (mood, osteoporosis, weight gain) and Mtx (bone marrow suppression, liver/GI/kidney tox)

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3
Q

10-day old infant

description
ddx
eval
mgmt

A

mucopurulent discharge

bacterial conjunctivitis
–gonorrhea: 3-4 days, most purulent, usually vaginal birth
–chlamydia: 1 wk
viral conjunctivitis (herpetic): 2wks
chemical conjunctivitis (silver nitrate): 1st 24hrs, self resolves by day 2

hx:
–sx: onset, progression, severity
–RFs: maternal hx, prolonged rupture of membrane, ocular ppx at birth

exam:
– ocular VS
– lid, adnexa, conj, K lacerations / ulcerations
– culture discharge: gram stain
—gram-, intracellular diplococci on thayer martin (chocolate agar) = gonorrhea
–antibody test (ELISA or fluorescent direct) = chlamydia

Mgmt:
- gonorrhea: irrigate
–if no K involvement: IM CTX x1 dose
–if yes K involvement: IV CTX x3 days
- chlamydia: PO erythromycin x 2wks, neonatologist referral because chlamydial infections can have systemic co-morbidities (PNA, GI)
- cipro gtts for any epi breaks
- prognosis is good w proper and timely treatment
- counsel
–risk of K scarring and perf
–counsel mom regarding STDs and the importance of notifying partners to halt spread

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4
Q

description
ddx
eval

A

peripheral non-perfusion with demarcation ridge and normal vessels

-ROP
-FEVR: inherited retinal disease, bilateral but asymmetric
-IP (unlikely male because usually lethal): avascular -> neovascular -> RD, splashed paint, abnormal teeth, seizures and delay
-Norrie Disease (predominantly male): b/l and symmetric infantile RD -> blindness, iris atrophy, cataracts and RD. progressive hearing loss + cognitive issues

hx: gestational age, birth weight, clinical course, FHx of retinal diseases

exam:
-ocular VS (VA at this age is usually just blinks to light)
-specialty eye: NVI

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5
Q

Mgmt of rop

A

Screen if <1500g or <30wks or rocky clinical course (supplemental O2, GI complications)
Start screen at later of 4-6wks after birth or 33wks gestational age

stages of ROP
1: demarcation line without height
2: demarcation ridge
3: fibrovascular proliferation
4: subtotal RD
5: total RD
plus disease: enlarged and tortuous posterior pole veins (severe vascular shunting)

zones of ROP
I: 2x distance from nerve to fovea, centered on nerve
II: between zone I and nasal ora serrata
III: temporal crescent

per Cryo-ROP: laser/cryo for threshold disease = 5 contiguous or 8 total hrs of stage 3 plus disease in zones I or II

per Early Treatment for ROP: treat pre-threshold disease within 72hrs:
- in zone I: plus disease or stage 3 w/o plus disease
- in zone II: stage 2 plus

Can also give anti-VEGF

monitor weekly initially
counsel: permanent vision loss, refractive error, amblyopia, strab, RD

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6
Q

description
ddx
eval

A

unilateral leukocoria

retinoblastoma
ROP
Coat’s disease (boys)
Norrie disease (bilateral, predominantly male)
Eale’s disease (bilateral, venous occlusion and periblibitis)
FEVR (picture; bilateral but asymmetric)
toxocariasis (uveitis)
persistent hyperplastic primary vitreous = persistent fetal vasculature (microphthalmic, cataracts, similar to picture, off ON)
cataract
leukemia (white centered heme)

hx:
-onset (any prior normal exams), progression
-sxs: red eye w irritation
-RFs: FHx, consanguinity, maternal infection, premature, trauma, pets

exam:
-ocular VS (strabismus, nystagmus)
-specialty eye:
–K diameter
NV: iris, hyphema, retina
–uveitis: AC cell, pseudohypopyon, posterior synechiae, vitreous seeding
–cataract
–scleral depression for mass in retina / choroid, large areas of telangiectasias or exudation c/w Coat’s / RB / FEVR
-testing:
–A-scan and B-scan to characterize the mass
–MRI to look for nerve / extraorbital / pineal involvement (not CT 2/2 risk of radiation)

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7
Q

mgmt of RB

A
  • refer to oculo-oncology, genetic counseling (1/3 inherited)
  • if small: cryo and PRP
  • if unilateral and advanced w low visual potential: enuc
  • if b/l large tumors: chemoreduction + cryo/PRP
  • if refractory vitreous seeding: intravitreal / intra-arterial melphalan
  • if extraocular: chemo + surgical reduction
  • f/u q3mo to monitor recurrence / involvement of fellow eye
  • counsel:
    –fatal if untreated, 95+% survival rate if treated
    if germline mutation, then avoid radiation given risk of secondary malignancy
    life long surveillance needed for secondary tumors like osteosarcoma
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8
Q

8mo girl
description
ddx
eval

A

large angle esotropia

-intermittent esotropia
-congenital esotropia
-accommodative / refractive esotropia
-sensory esotropia
-CN6 palsy / Duane’s syndrome
-negative angle kappa (2/2 macula dragging in ROP / FEVR / toxo)

hx: onset (old photos), constant/intermittent, fixation preference, progression, FHx
exam: ocular VS esp preferential looking for amblyopia, ocular motility for nerve palsy, ocular alignment (cover uncover, alternate cover), stereopsis, lid fissure for Duane’s, CRx for accomodative ET (usually >+2.00D), cataract/RB/other pathology for sensory ET

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9
Q

strab exams

-cover-uncover
1. no movement
2. covered eye flicks immediately in on uncover
3. covered eye flicks in seconds after uncover

-alternate cover

-Hirschberg

-Krimsky

A

-is there heterotropia? Which eye is preferred?
1. no tropia / preferred fixating eye
2. exophoria
3. intermittent extropia

  • elicits tropia + phoria
  • estimate of deviation based on light reflex: 30PD @ pupil, 60PD @ iris, 90PD @ limbus
  • requires no patient cooperation
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10
Q

Duane’s syndrome
-pathophys
-presentation
-types
- workup
-goal of surgery
-counsel

A

pathophys: no CN6 nucleus -> LR innervated by aberrant branch of CN3 = non-progressive

retraction +/- upshoot / downshoot on adduction + palpebral fissure narrowing

1: abduction deficit = ET
2: adduction deficit = XT
3: abduction and adduction deficits = ET/XT/ortho

none needed for duane (if suspicious for other etiologies, then CT/MRI brain for muscle restriction or compressive lesion or intracranial HTN)

expand field of binocular vision: MR and/or LR recession +/- transposition of SR/IR

prognosis good with surgical correction if no amblyopia; counsel regarding risks of strab surgery (sebsequent misalignment)

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11
Q

mgmt of congenital ET

A

-if accomodative (>+2D), full correction. If straight in distance and ET at near, 2.5D executive bifocal
-if amblyopia, patch (start w 2hrs/day while engaged)
-if congenital, b/l MR recession
-counsel re goals of treatment: cosmetic and to preserve stereopsis. Warn about risk of subsequent alignment issues

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12
Q

VA test based on age
1. <2yo
2. 2-5yo
3. >5yo

A
  1. preferential looking (prefers stripes vs homogenous card) / Teller acuity (grates)
  2. Allen cards (pictures)
  3. Snellen chart
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13
Q

angle kappa

A

-angle between visual axis and pupillary axis.
-Patient has manifest strabismus on hirschberg / Krimsky but no misalignment on cover-uncover testing
-Can be positive (XT) or negative (ET) due to macular dragging.

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14
Q

2yo, intermittent

description
ddx
eval

A

large angle exotropia

-intermittent exotropia
-congenital exotropia
-sensory exotropia
-nerve palsy / duane
-positive angle kappa (macula dragging 2/2 ROP/FEVR/toxo)
-convergence insufficiency (>10D difference between near and distance)

hx: onset (old photos), constant/intermittent, which eye, progression, FHx, prematurity
symptoms: blinking, eye rubbing to suggest diplopia, eye strain
exam: ocular VS esp preferential looking for amblyopia, ocular motility for nerve palsy, ocular alignment (cover uncover, alternate cover), stereopsis, lid fissure for Duane’s, CRx/cataract/RB/ON anomalies/other pathology for sensory XT

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15
Q

mgmt of intermittent alternating XT

A

-orthoptic treatment
-if high myope: glasses
-if amblyopia: patch good eye 2hrs/day (if cannot tolerate patching, then atropine drops)
-if poor control: b/l LR recession or unilateral recess/resect
-counsel: condition is progressive but no need for surgery if control is good. Good prognosis: most develop good stereopsis and no amblyopia

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16
Q

description
ddx
eval

A

bilateral media opacities

congenital cataracts (TORCH)
acquired childhood cataracts (diabetes, galactosemia, trauma, alport, lowe’s)
RB / FEVR / PHPV
ROP w total RD
Norrie or Coat’s in boys / IP in girls
Eale’s disease

hx: onset (old photos), progression
RFs: FHx (RB, cataracts, FEVR), prematurity, maternal diseases, metabolic diseases
eye exam: ocular VS (VA -> strab, nystagmus), EUA for ?cataracts, posterior exam
systemic exam: splashed paint
testing: US if no view to the back, TORCH titers, test urine for galactose (galactosemia), amino acids (Lowe’s), RBC galactokinase (alport)

17
Q

mgmt of congenital cataracts

A

-if NVS, observe
-if VS, CE + capsulotomy + anterior vitrectomy given high pco in children (aspiration only since soft, do NOT implant IOL) w aphakic contact lens or glasses
–unilateral: remove <6wks old
–bilateral: remove <10wks old

-refer to pediatrician for mgmt of systemic stigmata
–assess for TORCH infections (toxo, other - syphilis, HIV, rubella, CMV, herpes)
–screen for metabolic diseases (galactosemia, alport, Lowe, diabetes)

-counsel good prognosis if removed early but still risk of amblyopia requiring patching
-counsel need for IOL later
-counsel risk of glaucoma

18
Q

description
ddx
eval

A

vascular lesion of upper eyelid

capillary hemangioma / PHACES
lymphangioma
port-wine stain
pre-septal or orbital cellulitis
neuroblastoma (picture, onset more acute)
rhabdomyosarcoma (later onset, more acute)
encephalocele (present at birth and enlarges w crying)

hx: onset, progression, worsens w valsalva / URI / tears, sxs (head turns? obstructed vision -> strab, nystagmus)
RFs: FHx
Exam: ocular VS, alignment, CRx (expect significant flattening of area under lesion requiring positive cyl correction), lid warmth or tenderness
systemic: fever, syndromic features
testing: orbital imaging if orbital signs (RAPD, motility restriction, proptosis)

19
Q

mgmt of capillary hemangioma

A
  • glasses for astigmatism
  • patching for amblyopia
  • if progressive amblyopia despite patching / dysfiguring:
    – superficial lesions: topical timolol or laser (older tx was intralesional steroid w risk of retrograde CRAO)
    – deep lesions: PO propranolol (first rule out PHACES, administer under supervision from pediatrician 2/2 bradycardia)
  • if severe amblypia and increasing lesion size, consider surgical excision
    -eval for:
    –PHACES: posterior fossa, hemangiomas, arterial, cardiac, eye (morning glory), sternal
    Kassabach Merritt: hemangioma + thrombocytopenia (consumptive coagulopathy) and hemolytic anemia
  • natural course: progress over first year, then gradually resolves over first 4 years
  • good prognosis if no amblyopia
20
Q

young child

description
ddx
eval

A

subluxation of the lens upward and outward

Trauma
Marfan: ST subluxation
Ehler Danlos: IN subluxation
Homocystinuria: IN subluxation
Weill-Marchesani: microspherophakia -> anterior dislocation through the pupil

hx: onset, duration, progression, triggering event, FHx, PMHx
exam:
-eyes: ocular VS, ?capsule intact, ?other ocular signs of trauma, ?blue sclera
-systemic
–Marfan: tall & slender, arachnodactyly, heart problems
–Ehler Danlos: elastic skin
–Homocystinuria: mental retardation and light-colored hair, thrombotic events under anesthesia
–Weill-Marchesani: short stature w stubby fingers

21
Q

mgmt of lens subluxation in child

A
  • refraction to maximize vision
  • usually the result of trauma so fix other sequelae such as capsule violation w lensectomy w aphakic contact lens, RD w PPV
  • if vision is good (no diplopia), then can observe

-if systemic disease is suspected, then refer to pediatrician for workup:
–Marfan: cardiac echo for cardiac valve involvement; warrants abx ppx for surgery if cardiac defects to prevent endocarditis
–Homocystinuria: sodium nitroprusside testing - HIGHER RISK OF THROMBOEMBOLIC EVENTS DURING GENERAL ANESTHESIA

-counsel
–if trauma: risk of other sequelae such as RD
–if metabolic: genetic testing
–prognosis good

22
Q

Child presents w epiphora

ddx
eval

A

nasal lacrimal duct obstruction
punctal / canalicular atresia
congenital glaucoma
foreign body

hx: onset, progression, intermittent vs. constant, ?photophobia, ?blepharospasm, ?trauma, FHx of congenital glaucoma?
exam: ocular VS, tear lake, puncta, medial canthus warmth/pain/discharge/exudate w pressure, CRx, K abrasion, K edema / diameter / haab striae, Angle anatomy, evert lids to look for FB
testing: dye disappearance test (5min)

23
Q

mgmt of pediatric NLDO

A

Most likely blocked valve of Hasner, 90% open by age 1

<1yo:
-no mucopurulent discharge: conservative w warm compresses and crigler massage QID
-mild mucopurulent discharge: abx gtts
-dacryocystitis: admit for IV abx and monitoring of orbital cellulitis

> 1yo:
-probing successful 70% of the time
-failed probing x2: DCR

-anisometropia can be present in patients w unilateral NLDO, so glasses
-counsel good prognosis and educate regarding signs of dacryocystitis

24
Q

description (ignore anisocoria)
ddx
eval
mgmt

A

apparent esotropia but normal, centered K light reflex and flat nasal bridge

pseudostrabismus (can be due to epicanthus, not here, which also resolves w maturation)
esotropia

hx: onset, variability, progression, FHx, birth hx, ?worse w near focus / tiredness
exam: ocular VS (preferential looking / asymmetric objection to occlusion of either eye), ocular alignment (cover-uncover / alternate cover if baby able to participate; if not then Hirschberg or Krimsky), stereopsis, CRx

-counsel regarding the condition, that there is no real eye turn and the appearance will improve as the face and nasal bridge grows. But monitor for changes
-f/u annually

25
Q

description
ddx
eval
mgmt

A

left sided ptosis + iris heterochromia + anisocoria with left < right

-congenital Horner (has iris heterochromia because sympathetic tone is needed for iris darkening)
-acquired Horner (no iris heterochromia if acquired after first few months of life)
-physiologic anisocoria
-pharm anisocoria
-CN3
-Adie’s tonic pupil

hx: onset (old photos), progression, birth hx (forceps), meds (scopalamine, recent eye exam)
exam: ocular VS, pupil size in light and dark, dilation lag in Horners
testing:
- For anisocoria: 1% apraclonidine to reverse anisocoria in Horners; 0.1% pilo to r/o Adie, 1% pilo to r/o pharmacologic

-Unless clearly documented congenital horner’s, MRI/MRA w/wo contrast sympathetic pathways to look for compressive lesions (neuroblastoma in peds)
-counsel regarding r/o imaging as a precaution but likely congenital and can be observed

26
Q

description
ddx
eval
mgmt

A

right sided ptosis

congenital ptosis: levator fibrosis -> lid elevated during downgaze, limited upgaze function, low/no lid crease
Horner’s (congenital or acquired)
CN3
traumatic
Marcus Gunn jaw winking
MG

hx: onset (old photos), progression, variability, FHx of congenital ptosis, trauma, birth hx, eye turn
exam: ocular VS (?amblyopia, ?EOM, ?anisocoria), ?iris heterochromia, ocular alignment, CRx, e/o trauma. Lid function and position. Pigmentary retinopathy for Kearns-Sayer.

Mgmt:
- if mild w vision good and no amblyopia: observe
- if severe +/- amblyopia: frontalis sling + patching
- if astigmatism: glasses
- counsel about amblyopia but usually good prognosis (VA and cosmesis) w ptosis correction and amblyopia mgmt

27
Q

5mo

description
ddx
eval
mgmt

A

reddish skin unilatearlly + K diameter asymmetry

Sturge Weber
hemangioma
periocular contact dermatitis
preseptal cellulitis

hx: onset, progression, epiphora/photophobia/blepharospasm, developmental delay, seizures
exam: ocular VS (VA, IOP), signs of glc (increased K diameter, K edema / pachymetry, Haab striae, CRx), gonio, iris heterochromia, choroidal hemangioma
testing:
–Ascan for AL
–MRI to look for leptomeningeal angiomatosis (which is diagnostic for SWS)

manage glc:
- gtts and oral acetazolamide for IOP control (but avoid brimonidine in infants due to CNS depression, bradycardia)
- goniotomy if good view, otherwise trabeculotomy or tube
- glasses for longer axial length

manage choroidal hemangioma:
- if vision limiting, irradiate
- patching for amblyopia

manage systemic:
- neurology for seizures
- peds for developmental delay

counsel:
- both glaucoma and choroidal tumors can cause permanent vision loss
- treatment of SWS glaucoma is particularly difficult due to elevated episcleral pressure and would require surgery
- not genetic dx

28
Q

description
ddx
eval
mgmt

A

ptosis, epicanthus inversus, telecanthus

blepharophimosis syndrome
isolated belpharophimosis
ptosis
facial dysmorphism (crouzon syndrome)

hx: onset, progression, FHx, growth hx
exam: ocular VS (VA, IOP), ocular alignment, CRx, cleft/high palate, cupped ears

mgmt:
-genetic counseling: AD, FOXL2 on chr 3
-endocrine: type 1 a/w premature ovarian failure
-glasses
-patching
-delay surgery as can improve w age; discuss multi-stage nature of surgery - canthal surgeries first, then ptosis surgery
-if evidence of deprivational amblyopia, however, offer single stage combined surgery

29
Q

pulsates w light

ddx
exam
testing
mgmt

A

Morning glory

Moyamoya (circle of willis stenosis) - developmental delay
Basal encephalocele (looks like nasal polyps)
PHACES

exam: central glial tuft *morning glory flower must have a stem, straight papillary vessels, contractile tissue, peripapillary pigments
vs. coloboma/staphylomas, which have no tuft. Coloboma have no pigments. Staphylomas have normal vessels.

needs MRI/MRA

no tx -> optimize vision
prognosis varies 20/20-CF

30
Q

ddx
eval
mgmt

A

-aniridia (isolated or associated w systemic syndromes - WAGR [wilm’s tumor, aniridia, genitourinary, retardation] or Gillespie [ataxia and intellectual disability])
-trauma
-ICE
-Axenfeld-Rieger: spectrum from anteriorly displaced SL (posterior embryotoxin) -> +prominent iris processes (axenfeld anomaly) -> TM abnormalities and glaucoma (axenfeld syndrome) -> iris stromal hypoplasia (rieger anomaly) -> dental or skeletal abnormalities (rieger syndrome) ->

hx: onset, progression, photophobia, GU defect, ataxia, intellectual development, FHx
exam: ocular VS, nystagmus / strab, K pannus / limbal stem cell deficiency -> opacification; K diameter / haab striae, gonioscopy, cataract, foveal / ON hypoplasia.
testing:
–imaging for Wilm’s tumor
PAX6 (though can also be sporadic)

mgmt:
-isolated iris coloboma: symptomatic (tinted glasses, CL, surgery) and amblyopia management only
-sydnromic: refer to peds and genetics

31
Q

DVD
IOOA

A

fog -> affected eye drifts up

lateral gaze -> adducted eye shoots up

32
Q

PHPV
features
mgmt and counselling

A

fibrovascular stalk, cataract, RD, glaucoma, microphthalmos

PPV to relieve traction
lesectomy for media opacity
patching to minimize amblyopia

2nd MCC of leukocoria (after RB), 90% unilateral
CF vision but can improve with early intervention

33
Q

mgmt of NF1

A

C: observe
A: observe
L: observe
B: observe
O: observe unless dysfiguring or threatening chiasm, then chemo/rad -> surgical debulking if NLP
R: observe
N: can excise if dysfiguring but will recur

decreases life expectancy by ~15yrs

34
Q

pigmentary retinopathy, cataracts, microphthalmia, and/or glaucoma -> dx?

A

congenital rubella

35
Q

labs for neonatal herpes
counsel

A

serology and corneal swab -> viral culture and PCR
risk for fatal encephalitis and disseminated disease w developmental delay -> treat early and aggressively to minimize sequae such as scarring and astigmatism / amblyopia

36
Q

Features of neuroblastoma

testing

mgmt

A

ecchymosis and proptosis and Horner’s and opsoclonus (paraneoplastic syndrome)

Tumors in
neck -> SVC
chest -> breathing problems
abd -> distention and GI issues
spine -> paraplegia and urinary/bowel control issues

MRI/MRA w/wo contrast
urine catecholamines (not serum)

co-manage w onc: +/- surgery, chemo, radiation, bone marrow transplant
Counsel: younger (<18mo) presentation carries better prognosis

37
Q

ddx for retinal coloboma

A

idiopathic

CHARGE (coloboma, heart, atresia choanae, retardation, genitourinary, ears): genetic testing (CHD7), co-manage w peds, monitor for strab / amblyopia / CNV / RD -> low vision services; visual prognosis variable depending on location of defects
Gorlin-Goltz
trisomy 13 or 18

38
Q

spontaneous hyphema in a child w iris nodules

dx
test
mgmt

A

juvenile xanthogranuloma
skin nodule biopsy for confirmation
manage hyphema and IOP
iris nodules can be managed with steroids, radiation or excision

good prognosis