PEDIATRIC

OPHTHALMOLOGY &
RETINOPATHY OF
PREMATURITY
Cabahug, Kurt Raymond Y.
OUTLINE
■ I. Growth & Development of the Eye
■ II. Abnormal Visual Development
■ III. Eyelid Disorders
– Congenital
– Infectious & Inflammatory
– Neoplasms & Noninfectious
– Acquired
■ IV. Orbital Disorders
– Craniosynostosis
– Neoplasms & Ectopic Tissue
OUTLINE
■ V. Lacrimal Apparatus
– NLDO
■ VI. Cornea and Iris
– Megalocornea & Microcornea
– Persistent pupillary membranes
– Axenfeld-Rieger Syndrome
■ VII. External Conditions
■ VIII. Pediatric Glaucoma
– Primary
– Secondary
OUTLINE
■ IX. Child Abuse
■ X. Retinopathy of Prematurity
– Classification
– Diagnosis
– Sequelae and Complications
 I. GROWTH AND DEVELOPMENT
■ Dimensions
– Most growth happens in 1st year of life
PHASE AGE GROWTH AXIAL LENGTH
(YEARS) INCREASE IN MM
1ST 0-2 RAPID 4 MM - 1ST 6 MOS.
2 MM – NEXT 6 MOS
2ND 2-5 SLOW 1MM
3RD 5-13 SLOW 1MM
I. GROWTH AND DEVELOPMENT
■ Neonatal Examination
– External anatomy
– Red reflex
■ 6 months
– Alignment (for strabismus)
– Movements
■ 4 years
– Visual acuity (20/20-20/30)
■ 5-16 years
– VA every 2 years until 16 y.o.
– Color vision at 8-12 y.o.
 I. GROWTH AND DEVELOPMENT
■ Corneal growth
– Rapid in 1st year
– 9.5 -10.5mm in newborns to12mm in adults
– mild corneal clouding: common in prematurity
 Refractive State
• Axial length increases and the cornea & lens flatten.
• Eyes are hyperopic at birth, become more so until 7 y.o.
• Myopic shift until reaching adult dimensions
I. GROWTH AND DEVELOPMENT

■ Orbit and Adnexa

– Orbit volume increases
– Orbital opening – less circular
– Lacrimal fossa - more superficial
– Palpebral fissure length increases rapidly causing the
round infant eye to become its elliptically adult
shape
I. GROWTH AND DEVELOPMENT
■ IOP
– Lower in infants; <21 mm Hg

■ Cornea, Iris, Pupil & Anterior Chamber

– Average central corneal thickness decreases in the 1st
6-12 mos.
– Iris color change at the 1st 6-12 mos.
– PLR at 31 weeks AOG
– Posterior migration of the lens and ciliary body creating
the angle recess
I. GROWTH AND DEVELOPMENT
■ Retina
– poorly developed macula changes over the first 4 years
– retinal vascularization: centrifugal, from the optic disc
at 16 weeks AOG to the temporal ora serrata by 40
weeks AOG
■ Extraocular muscles
– conjugate horizontal gaze - present at birth
– vertical gaze: may not be fully functional until 6 mos.
 Abnormal Growth & Development
■ Agenesis
■ Hypoplasia
■ Hyperplasia
■ Dysgraphia
■ Failure to divide/canalize
■ Persistence of vestigial
structures
II. VISUAL DEVELOPMENT

• blink reflex: w/in a few days of birth

• PLR: 31 weeks AOG
• eye contact & facial expressions by 6-8 weeks
• interest in bright objects at 2-3 mos.
• smooth pursuit asymmetry resolved by 3-4 mos.
• Fix-and-follow visual response to small toys at 3-4
mos.
II. ABNORMAL VISUAL DEVELOPMENT
■ Disconjugate eye movements,
skew deviation & sun setting in
newborns shouldn’t not persist
>4 mos
■ Searching eye movements
■ Lack of response to familiar
faces and objects
■ Nystagmus
■ Staring at bright lights
■ Oculodigital reflex – forceful
rubbing
Classification of Visual Impairment
■ Delayed Visual Maturation (DVM)/ Cortical Inattention
– Failure of normal visual fixation/tracking to develop within
the 1st 3 mos.
■ Pregeniculate Visual Impairment
– visual deficits resulting from pathology anterior to the lateral
geniculate nucleus
■ Retrogeniculate Visual Impairment/Cerebral Visual Impairment
(CVI)
– derived from pathology posterior to the lateral geniculate
nucleus
III. EYELID DISORDERS

■ TELECANTHUS ■ CONGENITAL ENTROPION

■ DYSTOPIA CANTHORUM ■ CONGENITAL ECTROPION
■ CRYPTOPHTHALMOS ■ BLEPHAROPHIMOSIS-PTOSIS-
EPICANTHUS INVERSUS
■ CONGENITAL COLOBOMA OF
THE EYELID ■ CONGENITAL PTOSIS
■ ANKYLOBLEPHARON ■ MARCUS GUNN JAW-
WINKING SYNDROME
III. Congenital Eyelid Disorders

■ Telecanthus
– Greater-than-normal
distance between the
inner canthi
– Primary- interpupillary
distance is normal
– Secondary-
interpupillary distance
greater than normal
III. Congenital Eyelid Disorders

■ Dystopia Canthorum
– lateral displacement of
both inner canthi &
lacrimal puncta
 III. Congenital Eyelid Disorders

■ Cryptophthalmos
– failed differentiation
of eyelid structures
III. Congenital Eyelid Disorders

■ Ankyloblepharon
– fusion of part or all of
the eyelid margins
III. Congenital Eyelid Disorders

■ Congenital Coloboma
– Eyelid clefting or
notching
III. Congenital Eyelid Disorders

■ Distichiasis
– partial/complete
accessory row of
eyelashes out of or
slightly posterior to
the Meibomian gland
orifices
 III. Congenital Eyelid Disorders
■ Blepharophimosis-Ptosis-
Epicanthus Inversus
Syndrome (BPES)
– Blepharophimosis:
narrowing of eye opening
– Ptosis:
eyelid drooping
– Epicanthus Inversus: upward
fold of the skin of the lower
eyelid near the inner corner
of the eye
III. Congenital Eyelid Disorders

■ Marcus Gunn Jaw-Winking

Syndrome
– congenital synkinesis of
the jaw and levator
muscles
– ptotic eyelid elevates
with opening of
mouth/contralateral
jaw movement
III. Infectious/Inflammatory Eyelid
Disorders
■ Chalazion
■ Hordeolum
■ Molluscum
Contagiosum
III. Noninfectious Eyelid Lesions

■ Port-wine Stain
– congenital capillary
malformation
manifesting as a flat
red or pink
cutaneous lesion
III. Noninfectious Eyelid Lesions

■ Congenital
Nevocellular Nevi
III. Acquired Eyelid Conditions
– Excessive blinking
– Trichotillomania
■ pulling out of
one’s hair like
eyebrows and
eyelashes
IV. ORBITAL DISORDERS

■ Craniosynostosis syndromes
– Premature closure of at least 1 cranial
suture
– Syndactyly or brachydactyly
(except Crouzon syndrome)
IV. ORBITAL DISORDERS

■ Crouzon Syndrome
– Most common
autosomal dominant
craniosynostosis
syndrome
– Broad forehead
– Hypertelorism
– Proptosis
IV. ORBITAL DISORDERS

■ Apert syndrome
– Similar facial
features as Crouzon
syndrome
– Extreme syndactyly
(mitten deformity)
 IV. ORBITAL NEOPLASMS
■ 1o malignant neoplasms
– rhabdomyosarcoma
■ Metastatic tumors
– Neuroblastoma
■ Benign tumors (hemangiomas)
– PHACE syndrome
- post. fossa deformity
- hemangioma
-arterial lesion
-cardiac and
-eye anomalies
IV. Ectopic Tissue Masses

■ Dermoid Cyst
– Most common space-
occupying orbital lesion
– Cyst w/ epithelium and
dermal appendages
■ Epidermoid Cyst
– -Cyst w/ epithelium
– NO DERMAL
APPENDAGE
IV. Ectopic Tissue Masses

■ Congenital Cystic
Teratoma
– Tumor w/ tissues
derived from all 3
germinal layers
– Permanent optic
damage
 V. Lacrimal Apparatus

■ NASOLACRIMAL DUCT
OBSTRUCTION (NLDO)
– Most common lacrimal disorder
– 5% of full-term newborns
– Usual cause: thin mucosal
membrane the valve of
Hasner
– Treatment: probing
VI. CORNEA & IRIS

■ CORNEA
– Megalocornea
> 13 mm
– Microcornea
< 9 mm (NB)
< 10 mm (after 2 yrs)
VI. CORNEA & IRIS

■ IRIS
■ Persistent pupillary
membranes
– Most common iris
abnormality
– 95% of NBs
– Rarely of any visual
significance
VI. CORNEA & IRIS

■ IRIS
– Axenfeld-Rieger Syndrome
■ commonest cause of iris (stromal) hypoplasia
■ prominent Schwalbe line
■ iris hypoplasia
■ 50% lifetime risk of glaucoma
VII. EXTERNAL DISEASES
VII. EXTERNAL DISEASES

■ Ophthalmia Neonatorum
– Conjunctivitis in 1st
month of life

Neiserria gonorrhoeae
- First 3-4 days
- Ceftriaxone & saline
irrigation
VII. EXTERNAL DISEASES

Chlamydia trachomatis
■ Around the 1st week
■ Oral erythromycin, 50 mg/kg/day in 4 divided
doses for 14 days
Herpes simplex virus
■ HSV-2
■ Around the 2nd week
 VIII. PEDIATRIC GLAUCOMA
■ PRIMARY CONGENITAL GLAUCOMA
■ Visual acuity worse than 20/50 in
at least 50% of cases
■ Bilateral; Males
■ Onset: within 1st year of life
■ Increased resistance to aqueous
outflow
■ Classic triad:
– 1.Epiphora
– 2.Photophobia
– 3.Blepharospasm
VIII. PEDIATRIC GLAUCOMA

■ JUVENILE OPEN-ANGLE GLAUCOMA

■ Autosomal dominant condition
■ After 4 years of age
■ Different from PCG due to:
– Cornea not enlarged
– Haab striae not present
– Anterior chamber angle appears normal
 IX. Child Abuse

– Shaken baby syndrome

■ When an adult shakes a baby
out of anger/frustration,
because the baby wont stop
crying
■ intraretinal/preretinal/vitreous
hemorrhages w/o external
evidence
X. RETINOPATHY OF
PREMATURITY
X. Retinopathy of Prematurity (ROP)

■ Vasoproliferative retinal disorder unique to

premature infants
– Abnormal growth of retinal blood vessels in a
premature infant due to the complex interaction
between the Vascular Endothelial Growth Factor
(VEGF) and Insulin Growth Factor – I (IGF-I)
ICROP (Int’l. Classification of ROP)
■ Stage,
■ Zone/location,
■ Extent

■ The higher the stage

or the lower the
zone, the worse the
ROP
ZONES
I – around the optic
disc; radius = 2x disc
macula distance
II – ring-shaped;
extends to ora serrata
III – crescent zone of
temporal retina
Acute Stages of ROP
5 STAGES OF ROP
 Stage 1

• DEMARCATION LINE
separates normal vascular
retina & the avascular
peripheral retina
• Immature vascularization,
no ROP
• No need for treatment
Stage 2

• RIDGE
• May have small tufts
of fibrovascular
proliferation
(popcorn)
Stage 3

• RIDGE W/ BLOOD VESSELS

(extraretinal fibrovascular
proliferation)
• Neovascularization from
the ridge into the vitreous
• Ragged appearance
 Stage 4
-SUBTOTAL RETINAL DETACHMENT
-Traction from the scar produced
by bleeding, abnormal vessels
pulls the retina away from the
wall of the eye
4A – extrafoveal detachment
4B – detachment including fovea
Stage 5

• TOTAL RETINAL
DETACHMENT
• Detachments are
generally tractional and
usually funnel shaped
• May have catact,
glaucoma etc.
Plus and Pre-plus disease

■ PLUS disease ■ PRE-PLUS disease

– Marked arteriolar – Less dilation &
tortuosity tortuous
– Venous engorgement – Warning sign
of post. Pole
vasculature
– Vascular shunting
thru the new vessels
– Severe disease
 Risk Factors

■ Gestational age at birth

(inverse)
– < 30 weeks
■ Birth weight (inverse)
– <1500 g
■ Excessive supplemental
oxygen: role not well
understood
Screening & Diagnosis

■ Dilated fundus exam – first exam at 4 weeks

– Those born <30 weeks AOG
– BW <1500G
– Esp. in unstable premature infants
■ Digital retinal photography - highly accurate
■ 1st exam: 4 weeks postnatal age or corrected gestational
age of 30-31 weeks
Proposed Phil. Guidelines for ROP
Screening (PAO)
■ Infants w/ unstable clinical course
■ Premature infants
– <35 wks GA
– BW < 2000g
Proposed Phil. Guidelines for ROP
Screening (PAO)
■ Timing of screening
– 2 weeks post-natal age
– 32 weeks postconceptional age
Proposed Phil. Guidelines for ROP
Screening (PAO)
■ Termination of Screening
– Zone 3 vascularization
– Full retinal vascularization close to ora
serrata
 Treatment
■ Cryotherapy
■ Laser treatment – for any eye with type 1 ROP
■ Bevacizumab
■ Close observation
■ Late-onset retinal detachment
■ Vitrectomy & sclearal buckling – Stage 4/5 ROP w/ reasonable
hope of reattachment
 Sequelae & Complications
• Myopia: most common; may be severe;
amblyopia may result from high myopia or
strabismus
• dragging of macula may cause
pseudostrabismus and appearance of an
exotropia
• •late retinal detachment •cataract,
glaucoma, pthisis bubi
References

■ Vaughan & Asbury’s General Opthalmology

■ AAO 2016-2017 Pediatric Ophthalmogy and Strabismus
■ Classifying Retinopathy of Prematurity; Molinari et al.
■ Risk Factors for Retinopathy of Prematurity in Premature Born Children;
Alajibegovic-Halimic et al.
■ Proposed Philippine Guidelines for Screening and Referral of ROP