Cataract

Retinopathy

Child hood glaucoma

Iris abnormalities

Ocular tumors

Visual dysfunction(amblyopia)
Childhood cataract
Disorders of the Pediatric lens includes
 cataract,
 abnormalities in size and shape,
 location and development
Pediatric cataracts - congenital type are responsible
for nearly 10% of all visual loss in children world
wide
 Ped. cataract
The successful Mx of Pediatric cataracts fall under five
headings
a. cataract morphology and severity
b. timing of surgical intervention
c. optical correction
d. acuity measurement and amblyopia therapy
e. management of complications
 Pediatric cataracts
 Morphology – cataracts can involve entire lens or
part of the lens structure . The location in the lens
and morphology can give Info. about
onset,aetiology,laterality,and prognosis
a. Anterior polar cataracts= are common appearing
as small white dots in the center of the anterior
lens capsule
- thought to be remnants of persistent tunica
vasculosa lentis
- Not visually significant and rarely require surgery
- Anisometropia more common ,so careful
refraction is need
- Usually associated with corneal guttata and
astigmatism
Cont…
b. Nuclear cataracts –opacities that involve
the center of the lens
- bilateral or unilateral, inherited or sporadic , can progress
- if unilateral associated with microcornea and high risk of
aphakic glaucoma after extraction
c. Lamellar cataracts -involve cortex discrete, round shape
and larger in diameter than nuclear
- always bilateral ,usually acquired but can be inherited
- normal size and corneal diameter
- good visual prognosis after surgery
Cont…
d. Posteroir lenticonus/lentiglobus –caused by relative
thinning of central or paracentral posterior capsule
• “oil droplet “appearance on red reflex
• almost always unilateral and equal size of eye
• acquired type and progress to cortex
e. Persistent fetal vasculature – failure of the fetal
hyaliod vascular complex to regress
- Congenital ,almost always unilateral
-clinically due to retrolental membrane of varying size
and density attached to the posterior lens surface
f. Posterior subcapsular cataracts – are acquired,
bilateral and progressive
- occur due to steroids, uvietis, external beam
radiation
Timing of surgical intervention
Visually significant cataracts should be removed with in
the critical period of visual system development to
avoid vision loss.
IOL are becoming a common method of optical
rehabilitation but most Ophthalmologist do lensectomy
and contact lens correction in infants born with dense
congenital cataract.
Reoperation rate is very high
IOL power calculation
Formula => IOL power = A+2.5Al-0.9k
- where, A=constant
AL=axial length (mm)
K= average k-readings (in D)
GOAL = undercorrction
about 20% for infants and 10% in toddlers
The higher the IOL power, the more under correction
is needed
Optical correction
If lensectomy – decision is made to fitting of contact lenses
If IOL is to be implanted , a decision has to be made to the
power of the lens
 keratometry and biometry should be carried out under
anesthesia preoperatively
Infants less than 4 months will be 8-9.00D undercorrected
i.e. take in to account post operative refraction
Children over 5yrs are left with 1.00D for small amount of
axial elongation
After 8yrs treated like adults
Acuity measurement and amblyopia therapy
Children with unilateral congenital cataracts need
long term “aggressive” occlusion therapy
Good out come depends on compliance with
occlusion and optical correction
If bilateral – part time occlusion and usually if early
surgery done VA is 6/18or better
Management of complications
Aphakic glaucoma – less in IOL implant
Pupil block glaucoma – as a result of postoperative papillary
membrane formation or vitreous body prolapsed to AC
Mx = vitrectomy and peripheral iridoectomy
Strabismus ( ET>XT)
Nystagmus – affects about 50% of children and associated with
deprivational amblyopia, late surgery, and poor acuity
PCO- YAG laser under anesthesia
Amblyopia – most common
Retinal D.,macular edema ,endophthalmities are rare
Ped cataract
A child with cataract may have one or all of

these presenting signs and features :

Loose of red reflex

Nystagmus(wobbly eyes)

Strabismus

Impaired visual behavior

What is Leukocoria?
 LEUKOCORIA
Means white pupil . Cataract &retinoblastoma-most
possible causes of Luekocoria
Differential diagnosis :
congenital cataract
congenital glaucoma
Retinopathy of prematurity
Retinoblastoma
Retinal detachment
Coat’s disease
Retinopathy of prematurity
A proliferative retinopathy that develops in
premature infants due to incomplete vasculogenesis
of the retina at the time of birth. Is a biphasic
blinding disease

Phase I - delayed retinal vascular growth after

premature birth
Phase II - follows when phase I-induced hypoxia
releases factors to stimulate new blood vessel growth
Retinopathy of prematurity
Normal retinal vascular development altered and abnormal
Neovascularization occurs
Infants born prematurely and exposed to oxygen therapy , e.g.

or respiratory distress
Oxidative insult to immature retinal vessels

Risk factors: low birth weight(< 2.5Kg)

and <7mon birth

Artificial oxygen
 Key Clinical features of ROP
Avascular retina
Stage 1 = demarcation line between normal and avascular
retina.
Stage 2 = elevated ridge between normal and avascular
retina.
 Stage 3= extraretinal fibrovascular proliferation at the
ridge between normal and avascular retina.
Stage 4=contraction of fibrovascular proliferation exerts
traction on the retina
Stage 5= total retinal detachment
 ROP
Cryotherapy – main stay of treatment but technically

more difficult
Retinal laser photocoagulation – prevent VEGF factors

The ultimate prevention of ROP would be prevention of

premature birth itself

ROP
Paediatric glaucoma
 primary Congenital glaucoma

- Occurs sporadically ( infrequent ,periodic ,random )or

inherited as autosomal recessive

- In almost all cases untreated ,progressive and leads to

blindness
- Blindness = 2-15% of cases

Disease of surgery / cannot be treated medically

 Signs and Sxs:
Hx = Epiphoria, photophobia, blepharospasm
 Clouding and megalocornea
 corneal edema
 Haab striae
 Long axial length- common feature
Corneal opacification – irreversible
Myopic Fundus – irreversible
Optic nerve cupping – reversible but high cupping ( CD
ratio –high)
 Primary congenital glaucoma
Buphthalmos and pseudoproptosis–
“OX” eye appearance
The hallmark of all forms of glaucoma in infants and young
children is ocular enlargement
Spontaneous lens dislocation
Treatment
Types of surgery :
 Goniotomy, , trabeculotomy, trabeculoectomy ,
cycloablation
Secondary paediatric glaucoma's
 2nd ry to ocular structural abnormalities

Or associated with systemic conditions

 Aniridia – associated with glaucoma, cataract,

foveal hypoplasia, and nystagmus

- is sporadic or inherited
Axenfeld syndrome- Cxed by anteroirly displaced
schwalbe’s line with iridocorneal adhesions , slit like
pupils
Reiger anomaly – iris hypoplasia ,and atrophy
,ectropion uvea
Peters anomaly – central corneal opacification with
iridocorneal adhesions to the edges of the leukoma
Secondary to systemic disease
Sturge –Weber syndrome- also named nevus flammeus

or port-wine stain – features include port-wine stain of

the face or upper eye lid , intracranial calcifications,
and glaucoma
Iris abnormalities
 Dyscoria – abnormality of the shape of the pupil
and is congenital malformation
Aniridia –panocular ,bilateral disorder ranges from
total absence to mild iris hypoplasia
 Typical features = nystagmus ,foveal hypoplasia, VA less

than 5/60 ,photophobia, corneal opacification with

pannus
Iris abnormalities …
Iris coloboma = typical if occur in inferonasal
quadrant
Pupil shaped like light bulb, keyhole or inverted tear
drop
Brushfield spots
Heterochromia iridis
Persistent pupillary membrane –most common dev’tal
abnormality of iris ( about 90% of newborns)
Abnormalities of size, shape or
location of pupil
Microcoria – indicate an absence or malformation of

dilator pupillae
Macrocoria –congenitally dilated and fixed pupils

with normal appearing iris

- iris sphincter trauma, pharmacologic mydriasis, and

acquired neurological disease
 Cont…

 Polycoria

 Congenital iris ectropion = of the posterior pigment

epithelium on to anterior surface of the iris
• Iris trasillumination –result from the absence of
pigmentation in the posterior epithelial layers
lacrimal drainage system
Developmental problems
Atresia of puncta or canaliculi – failure of canalization
- Sxs : accumulation and over flow of tears
Dacrocele – unusual condition that occurs when a distal
blockage usually membranoues causes distenstion of
the sac
- Appearance with bluish swelling just below and nasal
to the medial canthus
- Mx = digital massage

- Nasolacrimal duct obstruction; clinical features include:

Epiphoria , a sticky muciod or mucopurulent material,

failure of fluorsecin dye to appear in the nose
- DDx : of CNLDO include:- blepharitis with dry crusting on

eyelid margins, congenital dacrocele, congenital glaucoma

 Retinoblastoma
The most common type of malignant tumor of

childhood and one of the most common pediatric

solid tumor
Inheritable =Loss of tumor suppressor gene RB1

(chromosome 13)
 Detected after birth usually around 8 months

and rarely seen after 7yrs

 Retinoblastoma
Incidence : 1: 14,000- 1:20,000live births

Typically diagnosed in the first year of life in

familial and bilateral cases

Most common initial presenting sign –

Luekocoria described by the parents as glow,

glint or cat’s eye appearance
 Retinoblastoma
The most important feature that allows
differentiation of retinoblastoma from these other causes
of leukocoria is the presence of calcification
25 % cases present with ET/ XT and less common
presenting ophthalmic features are; vitreous Hge,
ocular or periocular inflamations, gluacoma,
proptosis,
Is a nueroblsatic tumor ,biologically similar to
neuroblastoma, and meduloblastoma
Diagnosis
Based on ophthalmic appearance
Evaluation : imaging of head and orbits
Mx= enucleation of eyes with unilateral RB to avoid
systemic chemotherapy
Avoid unnecessary manipulation of the globe
Primary chemotherapy -> local therapy most
commonly used vision sparing technique
Metastatic tumor of the eye
Nueroblastoma
 one of the most common childhood cancer . most
common source of metastasis : adrenal gland and
sympathetic cervical ganglion chain
It develops from the tissues that form the sympathetic
nervous system (which controls body functions, such as
heart rate and blood pressure, digestion and levels of
certain hormones
20% show orbital involvement
 clinical manifestations :
- unilateral proptosis
- lid ecchymosis lid swelling, ocular
motility disorders,
Other systemic signs: - abdomina fullness, pain, venous
obstruction and edema, hypertension,
Mean age of diagnosis with orbital nueroblastoma = 2yrs
Metastatic tumors
Rhabdomyosarcoma - the most common
primary malignant tumor of the orbit
- average age of onset 5-7yrs
- 5 % incidence
More aggressive ,carries poorer prognosis
Ophthalmic features = proptosis ( 80- 90%)
= globe displacement
= blepharoptosis
= conjunctiva and lid swelling, pain
(10%) palpable mass
Diagnosis
MRI / CT scan = show irregular, well

circumscribed mass
Tx= radiation and chemotherapy