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Peds Flashcards

1
Q

Ddx for leukocoria

A

RETINOBLASTOMA until proven otherwise

PREDICT:

P = PFV (persistent fetal vasculature)/cataract
R = RETINOBLASTOMA* or ROP
E = Endophthalmitis
D = Dysplasia of retina -- FEVR
I = Incontinentia Pigmenti (IP)
C = Coats disease
T = Toxocariasis
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2
Q

Ddx for fundus photo showing peripheral non-perfusion, demarcation ridge, and normal vessels

A

ROP
FEVR
Incontinentia pigmenti
Norrie disease

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3
Q

What are the stages of ROP

A 
DR. FIT
Stage 1 = Demarcation line
Stage 2 = Ridge
Stage 3 = Fibrovascular (neovascularization)
Stage 4 = Incomplete RD
Stage 5 = Total RD
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4
Q

Ddx for bilateral mucopurulent discharge from the eyes in a 10 day old infant.

A

Bacterial conjunctivitis (gonococcal)
Chlamydial conjunctivitis
Herpetic conjunctivitis
Chemical conjunctivitis

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5
Q

What is the treatment for neonatal conjunctivitis, specifically gonococcal conjunctivitis?

A

IV or IM ceftriaxone 50 mg/kg daily for 1 week or IV penicillin G

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6
Q

What is the treatment for Chlamydia neonatal conjunctivitis

A

Oral erythromycin 50 mg/kg per day for 10-14 days
Topical irrigation.
Involve pediatrician as well given respiratory and GI involvement

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7
Q

Ddx for 6 year old girl with early band keratopathy nasally and temporally and intermittent history of blurred vision but no eye pain

A

JIA (Juvenile Idiopathic Arthritis)
Silicone oil
Chronic retinal detachment
Trauma with subsequent phthisical eye
Metabolic – hypercalcemia: hyperparathyroidism, renal failure, vit D toxicity
Inflammatory conditions: sarcoidosis, gout
Malignancy

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8
Q

Ddx for young child with photophobia, epiphora and cloudy enlarged cornea.

A

Congenital glaucoma
STUMPED (sclero-cornea, trauma, ulcer, metabolic, peters/ppmd, endothelial (CHED), dermoid)
CHED (Congenital Hereditary Endothelial Dystrophy)
CHSD (Congenital Hereditary Stromal Dystrophy)
Encephalofacial angiomatosis (Sturge-Weber)

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9
Q

Ddx for external photo showing 8 month old with large angle esostropia.

A 
Congenital esotropia
Accommodative esotropia
Sensory esotropia
6th nerve palsy
Duane retraction syndrome
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10
Q

History/work up/physical exam/eval for a large angle esotropia?

A

Complete ophthalmic history:
Onset of eye turn, intermittent or constant, one eye or both eyes turning in? Family hx of strabismus? Review old photos to help determine if fixation preference.

  1. check vision, fix and follow - preferential looking cards to see if any amblyopia or fixation preference
  2. check stereopsis with Lang card
  3. check ocular alignment with cover uncover and alternate cover testing
  4. check ocular motility to make sure no limitation of abduction
  5. retinoscopy for cycloplegic refraction
    Full eye exam - slit lamp exam and dilated fundus exam looking for ocular pathology such as cataracts and retinoblastoma that can cause a sensory strabismus.
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11
Q

Ddx for 2 year old girl who’s mom notes intermittently wandering eye. External photo showing large angle exotropia.

A 
Intermittent exotropia
Congenital exotropia 
Sensory exotropia
3rd nerve palsy
Duane retraction syndrome type II or III
Pseudoexotropia with positive angle kappa (ROP leading to macular dragging)
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12
Q

Ddx for young child - external photograph showing bilateral media opacities

A 
Congenital cataracts
Trauma causing cataracts
PFV
Retinoblastoma/ROP with tractional RD
Meduloepithelioma
Norrie disease (boys), incontinentia pigmenti (girls)
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13
Q

Work up/physical exam for bilateral congenital cataracts

A

Complete ophthalmic history: Family history of congenital cataracts or retinoblastoma? Trauma history? Prenatal and birth history - any TORCH infections? Growth and development?

Complete ophthalmic exam of both eyes

  1. check vision fix and follow
  2. check pupils
  3. check for motility and alignment
  4. any nystagmus?
  5. slit lamp exam to see if cataract present, dfe to rule out retinoblastoma or rop/RD, PFV, meduloepithelioma or other retinal causes of leukocoria. consider ultrasound if no view.

Work up:
labs: cbc, blood sugar, TORCH titers, syphilis/VDRL, urine analysis for reducing substances/galactosemia
Refer child to pediatrician for systemic disease/stigmata of TORCH infections

Management/treatment:
If visually significant (critical size is greater/equal to 3mm), then cataract extraction recommended early at age 6-8 weeks (bilateral cataracts) or 4-6 weeks age (unilateral cataract) to decrease chance of amblyopia. aspiration since lens is soft, posterior capsulotomy and anterior vitrectomy given will develop pco otherwise. for most children would not implant IOL, especially under 6 months of age. will need aphakic CL or spectacles and frequent follow up and treatment of any amblyopia / strabismus if develops

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14
Q

Ddx for 6 month old child with vascular lesion of upper eyelid.

A

Capillary hemangioma
Lymphangioma
Port wine stain
Preseptal or orbital cellulitis

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15
Q

History/work up/eval and treatment for capillary hemangioma.

A

Inquire about age of onset, progression, changes in lesion size or appearance with crying or URI, evidence of ocular misalignments. check vital signs for fever, feel lesion to assess if warmth or tenderness.
Assess vision, pupils for APD, IOP, check motility and alignment.
Perform slit lamp exam and DFE
Retinoscopy for cycloplegic refraction
If concern for orbital involvement (limitation of motility, proptosis, apd - optic neuropathy) get orbital neuro-imaging.
Indications for treatment include risk of amblyopia, optic nerve compression, exposure keratopathy, severe cosmetic defect, infection and necrosis.
If no amblyopia can observe, correct any refractive error/astigmatism.
If amblyopia present and mechanical ptosis, then treatment with oral propranolol, topical timolol can be considered. (risks of propranolol - bradycardia),
If amblyopia is severe and lesion is increasing in size, would consider surgical excision.

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16
Q

Natural history of capillary hemangioma?

A

Initial enlargement and spontaneous resolution. 40% of lesions completely involute by age 4, 80% by age 8.

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17
Q

Ddx for young child with chronic tearing and external photograph showing left sided epiphora, positive dye disappearance test and normal corneas.

A

NLDO - congenital or acquired
Congenital glaucoma
Canalicular/punctal atresia
Foreign body causing reflex tearing

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18
Q

What is the treatment for NLDO?

What is patient education/cause?

A

If under age 12 months and epiphora present since birth, conservative therapy with nasolacrimal sac massage 4 times per day. prescribe ophthalmic antibiotic ointment /drops if mucopurulent drainage is present. Defer NLD probing until after age 1 year provided dacryocystitis does not develop

Cause: imperforate valve of Hasner - usually resolves spontaneously in 90% by 1 year of age.
Education: prognosis is good with probing of NLD. Parents should look for signs of infection such as warmth, swelling and tenderness over lacrimal sac or purulent discharge from the puncta.

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19
Q

What are the characteristics of congenital motor nystagmus?

A 
CONGENITALS
C: Convergence dampens
O: Oscillopsia absent
N: Near vision good
G: Gaze uniplanar (horizontal nystagmus in all positions of gaze)
E: Equal in amplitude and frequency in both eyes
N: Null point present
I: Inversion of OKN response
T: Turning of head present (head turn)
A: Abolishes in sleep
L: Latent nystagmus can be present
S: Strabismus may be present
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20
Q

What is triad of findings in spasmus nutans?

A
  1. Fine, rapid, asymmetric eye movements (shimmering)
  2. Head nodding
  3. Torticollis
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21
Q

What is the work up of spasmus nutans?

A

Diagnosis of exclusion.
Careful exam of pupils checking for APD, optic nerve
Neuroimaging is necessary to rule out tumor (chiasmal or parasellar tumor), optic pathway glioma
Spasmus nutans is benign and disappears by age 5

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22
Q

What are the associated eye disorders of Down Syndrome?

A 
Refractive errors (hyperopia commonly)
Strabismus
Nystagmus
Amblyopia
Lid abnormalities (upward slanting palpebral fissures, epicanthal folds, blepharitis, chalazia, NLDO)
Keratoconus
Iris brushfield spots
Cataracts
Glaucoma
23
Q

Ddx for 7 month old with right eye turning in and poor red reflex. DFE shows vitreous hemorrhage and intraretinal hemorrhages in both eyes.
Also – what is work up?

A

Non-accidental trauma (NAT)
Trauma
ROP
*Ask about pregnancy and birth history, pre-maturity, supplemental oxygen? Any recent history of trauma?Living situation/home situation, baby difficult/crying?

Work up:
Radiology studies (CT/Xray) to evaluate for other traumatic injuries.
MUST REPORT CASE to child protective services
Monitor for amblyopia, may need PPV for non-clearing VH.
peds consult for tx of any other injuries

24
Q

What is the systemic syndrome associated with capillary hemangioma?

A

Kassabach-Merritt syndrome:
Consumptive coagulopathy with platelet trapping
Causes thrombocytopenia and cardiac failure
Mortality rate of 30%

25
Q

What is the etiology of bilateral congenital cataracts?

A

Idiopathic
Hereditary (usually AD)
Metabolic
Assoc with ocular disorder (Lowe’s, Alports)
TORCH intrauterine infection (Toxoplasmosis, Other viruses, Rubella, CMV, Herpesvirus)
Maternal drug ingestion or malnutrition
Trauma

26
Q

Explain Zone 1, Zone 2, Zone 3 in regards to ROP – what does this mean?

A

Zone 1 = posterior pole area enclosed by circle around the optic disc with radius equal to twice distance from disc to macula (diameter of 60 degrees)

Zone 2 = area between Zone 1 and a circle centered on disc and tangent to nasal ora serrata

Zone 3 = remaining temporal crescent

27
Q

What is plus disease?

A

At least 2 quadrants of shunted blood –> produces engorged tortuous vessels in posterior pole.

  • Arteriolar tortuosity and venular dilation
  • Poor prognostic sign.
28
Q

What is threshold disease?

A

Treatment is indicated for threshold disease

At least 5 contiguous or 8 non-contiguous clock hours of stage 3 ROP in zone 1 or zone 2 with plus disease.

*Threshold disease represents the level at which 50% will go blind without treatment

29
Q

What types of ROP require treatment?

Within what time frame?

A

Surgical treatment is currently recommended for the following (defined as “type 1” ROP): based on ETROP (Early Treatment of ROP):

Zone I: with plus (any stage ROP)
Zone I: without plus, and stage 3 ROP
Zone II: with plus, stage 2 or 3 ROP

Eyes meeting these criteria should be treated as soon as possible with laser photocoagulation to peripheral avascular retina, at least within 72 hours.

30
Q

What are potential complications of ROP?

A

High myopia, strabismus, amblyopia, macular dragging, nystagmus, glaucoma, cataracts, KCN, band keratopathy, retinal detachment, phthisis bulbi

31
Q

What are the levels of anisometropia that require correction to prevent possible amblyopia?

A

Myopic anisometropia: greater than or equal to 3 D

Hyperopic anisometropia: greater than or equal to 1 D

Astigmatic anisometropia: greater than or equal to 1.5D

32
Q

What is the rule of thumb for follow up after starting patching therapy for amblyopia?

A

No more than 1 week per year of age before re-examination.

Example: 2 years old – see back in at least 2 weeks

33
Q

What is the ddx for 4 year old boy with droopy eyelid?

A

Congenital ptosis:

  1. Myogenic (most common)
  2. Neurogenic (congenital CN3 palsy or Horner’s)
  3. Aponeurotic (birth trauma - rarely)
34
Q

What are characteristic findings of congenital myogenic ptosis?

What is the treatment?

A

Poor levator function
Loss of lid crease
Eyelid lag, sometimes lagophthalmos
Amblyopia

Treatment: Frontalis suspension with fascia lata/silicone rods

35
Q

What is the work up for retinoblastoma?

A
  • Neuroimaging - MRI to evaluate for extraocular extension and trilateral retinoblastoma (bilateral RB plus pineal blastoma or parasellar mass).
  • Oncology consultation for systemic work up including bone scan, bone marrow aspirate, lumbar puncture for cytology.
36
Q

What are the genetics for retinoblastoma?

A

RB mapped to chromosome 13q14 – must have gene mutation present on both chromosomes (2 hits)

RB is 94% sporadic and 6% familial (autosomal dominant with 80% penetrance – therefore only 40% manifest a tumor)

Most cases are sporadic and unilateral/unifocal
Bilateral cases are familial

37
Q

2 year old has RB. What is the chance of a sibling having RB?

A

If 1 affected child then chance is 1%

If familial, then risk of another child having RB is 40%

38
Q

What are the three characteristic histopathology findings of RB?

A
  • Homer-Wright rosette: nuclei surrounding a tangle of neural filaments without a lumen
  • Flexner-Wintersteiner rosette: single row of columnar cells in a ring around a central lumen
  • Fleurette: appears as a bouquet
39
Q

What are the treatment options for RB?

A
  1. Enucleation: performed on all blind painful eyes
  2. Photocoagulation/cryo: occasionally on eyes with 1 or a few small tumors that dont involve optic n. or macula
  3. External beam radiation: performed on salvageable eyes with vitreous seeding or large tumor or eyes with multifocal tumors
  4. Brachytherapy is performed on salvageable eyes with single medium sized tumor (that doesn’t involve optic n/macula)
  5. Chemoreduction – systemic chemo combined with local therapy used in globe salvaging management. Chemo delivered by catheters in ophthalmic artery or carotid artery.
40
Q

What is the prognosis of RB?

A

Generally good. 90-95% survival rate and 3% regress spontaneously.

Prognosis is poor for:

  • optic nerve or uveal invasion
  • extraocular extension
  • multifocal tumors
  • delay in diagnosis

RB is fatal within 4 years if left untreated

***When metastases occur –> commonly to CNS along optic nerve and 50% to bone.

41
Q

What is the Reese-Ellsworth classification system?

A

Reese-Ellsworth predicts the visual prognosis (NOT SURVIVAL) in eyes undergoing radiation treatment.

Related to size of tumor, if macular or juxtapapillary location, or with subretinal or vitreous seeding
Worst (group E): extensive retinoblastoma occupying > 50% of the globe.

42
Q

What is the ddx for globe displacement in a child?

A

Orbital cellulitis
Orbital mass

*Orbital tumors in kids are usually benign (90%)

Most common benign tumors in kids:

  • capillary hemangioma
  • dermoid cyst
  • lymphangioma

Less common benign:

  • neurofibroma
  • meningioma
  • inflammatory (IOI)

Ddx for malignant tumors in kids:

  • RHABDOMYOSARCOMA
  • NEUROBLASTOMA
  • teratoma
  • optic nerve glioma
  • histiocytic tumors
  • granulocytic sarcoma
  • Burkitt’s lymphoma
43
Q

What are orbital imaging features of rhabdomyosarcoma?

A

Diagnosis: CT, MRI, histopathologic evaluation

On MRI:

  • round or irregular (initially circumscribed later diffuse)
  • Shows enhancement with gadolinium
  • may show bone destruction
44
Q

How do you treat a patient with rhabodmyosarcoma?

A

Emergent diagnostic biopsy with immunohistochemical staining (try to remove as much of the tumor as possible without damaging vital structures).

Pediatric oncology consult for systemic work up: imaging, bone marrow biopsy, lumbar puncture.

Treatment is with combination of surgery, radiation, and chemotherapy – depending on location and extent of the tumor

45
Q

What is the prognosis for rhabdomyosarcoma?

A

Prognosis depends on the TYPE and extent of the malignancy.

Embryonal = everyone (most common)
Alveolar = Awful - worst prognosis, 2nd most common, inferior orbit, often arises in extremeties in adolescence
Pleomorphic = Please. Best prognosis, least common, occurs in adults
Botryoid = subtype of Embryonal

***With chemo and radiation, 3 year survival is 90%
For localized orbital tumors survival is 95% but if there is invasion of adjacent structures, survival is 60%

46
Q

What is the ddx for temporally dragged optic disc?

A 
Toxocariasis
FEVR
ROP
Incontinentia pigmenti
X-Linked retinoschisis
(adults: Tractional RD, PVR, ERM)
47
Q

What are the 3 clinical presentations of Toxocariasis?

A
  1. Chronic endophthalmitis: 2-9 years of age. granulomatous posterior uveitis with vitreous exudate and cyclitic membrane– often leads to destruction of the globe
  2. Localized granuloma: 6-14 years. Granuloma in the macular or peripapillary area, no inflammation, strabismus, non-progressive
  3. Peripheral granuloma: 6-40 years. Granuloma in periphery with fibrotic strand often to disc, strabismus, non-progressive.
48
Q

What is the causative organ in toxocariasis and what is its lifecycle

A

Infection due to second stage larval form of common roundworm toxocara canis or toxocara cati.
Nematode lives in dogs and cats, the mature adult releases eggs that are passed in dog/cat stool.
Contact with infected fecal material leads to human infection - more common in children. Local inflammatory reactions lead to encapsulation of worm.

49
Q

What is treatment for toxocariasis?

A

Antiparasitc therapy is unproven.
Treatment is largely based on REDUCING INFLAMMATION with steroids. With inflammation, start dilating drops.
25% of cases, vitreoretinal surgery to treat TRD / VH, ERM.

50
Q

What is the cause of Duane’s retraction syndrome (and the types?)

What is the treatment for Duane’s?

A

Congenital agenesis or hypoplasia of abducens nerve (CN6) and abnormal innervation of the lateral rectus muscle by a branch of CN3

Type 1: abDuction deficit (esotropia)
Type 2: aDDuction deficit (exotropia)
Type 3: abduction and adduction deficit

Tx for Duanes: correct refractive error, treat amblyopia.
Indications for surgery: deviation in primary position, head turn, significant upshoot or down-shoot

51
Q

What are the types of accommodative esotropia?

A
  1. Refractive accommodative esotropia:
    - moderate hyperopia
    - normal AC/A ratio of less than 5
    - similar esotropia at distance and near
  2. Non-refractive accommodative ET:
    - high AC/A ratio (greater than 5)
    - can be hyperopic or myopic
    - greater esotropia measured at near (usually 10 PD more eso at near than distance)
  3. Mixed mechanism
  4. Decompensated accommodative
52
Q

How do you measure AC/A ratio?

A

Perform a complete ophthalmic exam (duh!)
Cycloplegic refraction
Alignment/motility
Strabismus measurements at distance AND near
Suspect high AC/A ratio if Esotropia at near is 10 PD more than distance ET measured.

AC/A ratio = change in esotropia deviation / change in lens power

AC/A = (No Lens ET - With Lens ET) / Dioptric power lens used

Measure ET at near without any lens
Then measure ET at near with +3D loose lens over both eyes

Example:
you measure 20 PD ET at near without lens
you hold up +3D loose lens over both eyes and patient is orthotropic at near now.
AC/A = 20 (No Lens) - 0 (With Lens) / 3 = 20/3 = close to 7 which is greater than 5 –> high AC/A ratio!

53
Q

What is treatment of refractive accommodative esotropia?

A
  1. Provide full cycloplegic refraction hyperopic correction in spectacles
  2. Treat amblyopia with patching therapy (follow up in no less than 1 week per year of age of patient ie 3 year old RV in 3 weeks)
  3. Once vision in amblyopic eye is stabilized/normalized consider surgery if any esotropia >10 prism diopters is not correctable with spectacles.
54
Q

What is ddx for congenital ptosis?
What are the characteristics of congenital myogenic ptosis?
What is treatment?

A

Ddx: congenital ptosis can be myogenic (most common), neurogenic (congenital 3rd nerve palsy, Horner’s syndrome), aponeurotic (rare - birth trauma)

Characteristics of congenital myogenic ptosis:

  1. poor levator function
  2. loss of lid crease
  3. Eyelid lag
  4. Lagopthalmos (sometimes)
  5. rarely amblyopia

Treatment: treat amblyopia if present, provide full cycloplegic refraction (can cause astigmatism), then surgical repair (surgical repair usually requires frontalis suspension with silicone rods or fascia lata).

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