Pediatric Eye Diseases

o peripheral retina  retinopathy of prematurity

o follow up eye exam @ 6 mos: ocular fixation and eye movement
o 4 yrs up: visual acuity and following target (MORE reliable)
o Cycloplegia is paralysis of the ciliary muscle of the eye  curvature of the lens can no
longer be adjusted to focus on nearby objects  resulting in a loss of accommodation.
 Refraction testing: 1% cyclopentolate + Atropine to relax ciliary muscles and lens
 80% hyperopic N in ages 2-6
o 3D Vision important in fine motor functioning
o error of refraction  most common eye problem in childhood (ciliary muscle inc. AP diameter of
lens = accommodation process; cyclopentolate drops, atropine - gold standard
o physiologic hyperopia +4 in 1 y/o is OK but in 7 y/o is pathologic
o myopia is NEVER normal in any age group

I. CONGENITAL OCULAR ABNORMALITIES

CONGENITAL PTOSIS
o Levator muscle dystrophy: replaced with fibrous tissue **LID LAG ON DOWNGAZE
o Congenital Horner’s Sydrome / Cong. CN3 Palsy: inappropriate eye movements  amblyopia
**DROOPING EYELIDS
o How to assess severity?
1. look at levator function: move eyes up/look up  eyelids expected to move up, measure
in mm the movement of eyelids going up (N: 15 mm)
2. measure interpalpebral fissure ht between upper and lower eyelid margin
Mild 10-12mm LEVATOR RESECTION
Moderate 6-10mm
Severe 0-5mm, FRONTALIS SLING
poor levator function

3. look at margin reflex (N: 0 which is corneal light reflection); if upper eyelid covers corneal
reflection, already a (-). Fascia lata used in frontalis sling starting 4 y/o. If eyelid covers
the pupil  IMMEDIATE surgery d/t to potential amblyopia.

CORNEAL DEFECTS
o Corneal Opacities:
 If 2 to forceps injury at delivery: spontaneously resolves. **HAAB’s STRIA (tears in
Descement’s Membrane)
 Congenital Glaucoma: dysgenesis of angle structures. **EPIPHORA (tear overflow d/t
nasolacrimal obstruction)
 Endothelial Dystrophy: Endothelial Cells unable to pump out aqueous humor. (Review)
cornea should be DRY. (+) water  edema  corneal opacity
 Hurler’s Syndrome aka Gargoylism  mucopolysacccharidosis - glycosaminoglycans
 deficient a-iduronidase = buildup of dermatan sulfate and heparin sulfate
 AR
 Clouding of the cornea and retinal degeneration may occur within the first year of
life, leading to blindness
 musculoskeletal alterations: short stature, dysostosis multiplex, thoracic-lumbar
kyphosis
 facial facies: large head with bulging frontal bones, depressed nasal bridge with
broad nasal tip and anteverted nostrils, full cheeks and enlarged lips
 cardiomyopathy and valvular abnormalities
 neurosensorial hearing loss
 enlarged tonsils and adenoids, and nasal secretion
 Developmental delay
  Peters Syndrome  Ant. Segment Abnormality
 a cornea that is cloudy (opaque) and causes blurred vision, clouding of lenses
(cataracts); bilateral
 prominent forehead; small, malformed ears; narrow eyes; a long area between
the nose and mouth (philtrum); and a pronounced double curve of the upper lip
(Cupid's bow). The neck may also be broad and webbed. A cleft lip with or
without a cleft palate is common.
o Megalocornea
 horizontal diameter (limbus-limbus, temporal-nasal) N is 10-11.5mm at birth. > 12 mm is
megalocornea.
 Ddx is Cong. Glaucoma.
 d/t high IOP
 (-) hx of trauma or ocular hypertension
 x-linked recessive  more common in males
o Microcornea:
 <10mm d/t arrest in corneal development; if with gen decrease in eyeball =
microphthalmos (w/ defective function); nanophthalmos (small eyeball with N function)
o Anophthalmos: NO eye d/t RAX mutation  (-) optic vessicle

LENS ABNORMALITIES
o Coloboma: a portion is absent / incomplete lens, embryonic fissure fails to close
 AD
 iris coloboma always loc INFERIORLY  “KEYHOLE” appearance
o Subluxation
 partial lens displacement superiorly and temporally, bilateral and symmetric
 Ectopia Lentis  MARFAN’S SYNDROME, by age 5.
o Cong. Cataract
 white pupil = leukucoria
 ddx: retinoblastoma (most common malignant intraocular tumor in children);
prematurtity retinopathy stage 5 w/ retinal detach
 Galactosemia: gal-1-phosphate-uridyltransferase def.
 Philippines: most common etio is Infectious  Rubella in 1st trimester
 Congenital Rubella Syndrome: cataract + CoAo + sensorineural hearing loss
 Surgery: LENSECTOMY @ 4 mos, as early as 2-3 mos
 Lens implantation @ 1 y/o inserted inside capsule. Abnormal zonule fiber function (ie
Marfan’s) can cause lens to drop. Visual cortex maturity ages 7-10. Growth of eyeball can
reach until 18, case to case basis.

OPTIC NERVE HYPOPLASIA

o optic nerve head is small, non-progressive congenital disease
o DOUBLE RING SIGN  outer ring is the N junction between sclera and lamina cribrosa; inner
ring is abnormal extension of retina and retina pigment epithelium overlying lamina cribrosa
 Scleral canal (is of N size): orifice where optic disc passes to connect to eyeball
 Optic disc: optic nerve head is the point of exit for ganglion cell axons leaving the eye.
Because there are no rods or cones overlying the optic disc, it corresponds to a small
blind spot in each eye, pinkish red.
 In ONH = OD is pale and smaller than normal
 Optic cup: devoid of neural tissue, yellowish
o midline brain abnormalities (Septo-Optic Dysplasia of de Morsier) = **PITUITARY DYSFXN

CONGENITAL NASOLACRIMAL DUCT OBSTRUCTION

o tear stagnation within nasolacrimal system  overflow (Epiphora)
o before birth or within 1st month
 o resolves spontaneously; at 1 y/o tearing still present  do PROBING AND IRRIGATION. Beyond
3 y/o  Dacrocystorhinostomy
o punctal massage at medial aspect of lacrimal sac
o How to test?
 (1) put flourescin dye, wait for 5 mins, if dye still present = (+) obstruction
 (2) dacroscintigraphy

II. POSTNATAL EYE PROBLEMS

OPHTHALMIA NEONATORUM / Conjunctivitis of the Newborn

A. Chemical
o silver nitrate drops - prophylaxis vs neonatal infections prior to antibiotics, 1st - 2nd day

B. Chlamydia
o most common infectious cause
o 5th - 14th day of life
o Conjunctival scrapings under Giemsta = (+) inclusion bodies
o systemic and topical erythromycin, treat parents

C. BACTERIAL
o Gonococcal
 mucopurulent discharge
 presents earlier than Chlamydia and more virulent
 penetrates thru intact epithelium (vs Chlamydia NO corneal epithelium
penetrance when intact)
 Pen G, IM Ceftriaxone, treat parents

D. VIRAL
o Herpes Simplex: giant cells + viral inclusions
o resolves spontaneously; OR give antivirals

RETINOPATHY OF PREMATURITY
o prev. Retro (‘at the back’) lental (‘lens’) Fibroplasia (‘fibroplastic membrane’) before the advent
of Indirect Ophthalmoscopy, already stage 5 ROP aka total retinal detachment  retina attaches
to pos part of lens  visualized as an opaque membrane
o Retinal Revascularization: centrifugal approach from optic nerve (ref. point)
 reach nasal osa serrate @ 8 mos AOG, temporal @ 9 mos AOG
 complete revascularization = 9 mos
 avascular vessels in retina  VEGF  new b.v. grow into vitreous humor with
fibrovascular membrane formation  regresses, contracts  pulling of retina and
vitreous humor = retinal detachment
o Location
1. Zone 1: Posterior Pole, 2x macula-disc distance
2. Zone 2: from zone 1 to the most periphery of nasal osa serrata
3. Zone 3: remaining temporal crescent
o Staging
I. Demarcation line separating vascular from avascular retina, NO volume
II. Ridges, +/- small tufts or fibrovascular proliferation WITH volume, “humps”
III. Ridge with new blood vessel formation, extraretinal fibrovascular proliferation
IV. Subtotal retinal detachment
V. Total retinal detachment
 PLUS disease = + vascular shunting  enlarged vessels and tortuous arteries in
posterior pole / zone 1
o Aggressive Posterior ROP
 posterior location with Plus Disease with rapid progression to stage 5
 o Screening: BW < 1500g, AOG < 32 weeks // Philippines 34-35 weeks, 2000g
o Treat within 72H. Threshold is Stage 3 ROP, 5H contiguous / 8H cumulative in either zones 1 or
2.
o **NEW Threshold: Early treatment!
 Type 1 ROP, do retinal ablation. (REMEMBER)
a) Zone 1, with plus disease
b) Zone 1, stage 3 w/o plus
c) Zone 2, stage 2/3 with plus
 Type 2 ROP, treat only of progresses to Type 1.
o Management: Retinal photocoagulation (laser) to burn the avascular area. Vitrectomy if Stages 4
or 5. Anti-VEGF.

CONGENITAL GLAUCOMA
o Triad: epiphora, photophobia, blepharospasm
o other signs and symptoms: increased corneal diameter > 11.5mm, increased IOP > 10-20 mmHg
o Buphthalmus  neonate sclera less rigid, easily expands and cornea grows bigger
o Surgical management
 Goniotomy: establish a N anterior chamber angle
 Trabeculotomy: shunting procedure to move aqueous humor from ant. chamber to
subconjunctival space

RETINOBLASTOMA
o 90% diagnosed before 3 y.o.
o Differential of a “white pupil” or leukocoria.
o Heritable type: bilateral + multifocal.
 loss of function of RB1 gene on chromosome 13q14, encodes RB protein (tumor
suppressor)  role in cell cyle and apoptosis
o Mutated type: unilateral.
o Knudson’s “two-hit” model of oncogenesis.
o Imaging studies NOT diagnostic, biopsy CONTRAINDICATED. Diagnosis is made thru Indirect
Ophthalmoscopy with Slit Lamp Evaluation.
o “Trilateral Retinoblastoma” = hereditary retinoblastoma + pineal tumor
o Presentation: leukocoria and strabismus (misalignment)
o Treatment:
1. ENUCLEATION: large tumor
2. GLOBE SALVAGE: small tumor and detected early

III. STRABISMUS
o 4% of children
o Sherington’s Law: Agonist-Antagonist of 1 eye. Agonist contracting, antagonist relaxing.
(abnormal in Duane Syndrome)
o Hering’s Law: muscles of 2 eyes / yolk muscles. Both should simultaneously contract together.
o Congenital or hereditary in most cases.
o HIRSCHBERG TEST (Learn this!)
 shine light center of nose bridge and observe light corneal reflection
 Normal: light reflection at pupil center
 1mm deviation = 7 degrees x 2 (diopter value)
 Lend edge: 150; Iris center: 300; Limbus edge: 450
Eyes UP and RIGHT R Superior Rectus + L Inferior Oblique
UP and LEFT L Superior Rectus + R Inferior Oblique
LEFT L Lateral Rectus + R Medial Rectus
RIGHT R Lateral Rectus + L Medial Rectus
DOWN and RIGHT R Inferior Rectus + L Superior Oblique
DOWN and LEFT L Inferior Rectus + R Superior Oblique
 o COVER-UNCOVER TEST
 cover unaffected eye (fixating eye) or Normal eye
 If abnormal eye / deviating eye / uncovered eye has good vision = fixate and move
centrally
 After removal of cover, eye holds fixation  Alternating Esotropia (vision is same in both
eyes)
 After removal of cover, eye position doesn’t fixate and instead goes returns to its prior
position  AMBLYOPIA (brain prefers unaffected eye)
o Beyond 10 years old, strabismus is difficult to treat.
o Surgical Procedures:
 Resection  shortening to strengthen muscle
 Recession  weakening, cut or move entire muscle to a more posterior location
 Faden Procedure  posterior fixation w/o actually cutting the muscle, just suturing

IV. ESOTROPIA
o most common strabismus

A. Infantile Esotropia
 normal in < 6 months old
 “Comitant”: in all positions of gaze, measurement of deviations is the same or equal
 large deviation > 40mm diopters
 assoc. with Overacting Inferior Oblique and Dissociated Vertical Deviation (doesn’t
follow Hering’s Law)
 NO refractive error (differentiates from Accommodative Type)
 Surgical treatment: Bimedial Rectus Recession/Resection  so lateral rectus pulls
eye outward

B. Non-Accommodative / Acquired
 only diff from IE is manifests > 2 y/o

C. Accommodative
 overactive convergence response with insufficient fusion divergence: px has hyperopia ie
+6, eyes converge to accommodate but the brain has fusion divergence power to counter
however of insufficiency there is not enough brain signal to move ocular muscle