Evaluation of Paediatric
Cataract
Dr Sunayana Bhat
Consultant
Paediatric Ophthalmology ,Strabismus and Neuro
Ophthalmology.
Vasan Eye Care , Mangalore
Ex faculty , Father Muller Medical College
chanyn9@gmail.com
Epidemiological Data
• True prevalence of congenital cataracts is probably 2.5 3 per 10,000 live births
• Although some sort of cataract is present in 1 in 250
newborns
• Accounts for 10% of all visual loss in children worldwide
Quick Revision : Lens Embryology
Thickened ectodermal layer : lens plate 28 th day
Invaginates : lens vescicle
5 weeks
post epithelium elongation
(embryonic nucleus )
7 weeks
Ant / post Y sutures
(fetal nucleus )
12-14 weeks
Quick Revision : Lens Anatomy
Evaluation
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History
General physical exam
Ocular exam
Laboratory investigations
Screening
History
• Laterality
• Present since birth /acquired
congenital and unilateral cataract : usually not
associated with metabolic disease
• H/O trauma : Postnatal
• Noticed due to ….
- Leucocoria …
- Diminished vision ….
- Squinting ….
- Nystagmus ….
History
• Family history : Trace atleast
3 generations
Mode of inheritance
Disorders
Autosomal dominant
1,2,16,17 gene defects
Autosomal recesive
X linked
Nance Horan syndrome
Chromosomal
Trisomy 21,18/Turner
syndrome/Cri du chat syndrome
/translocations
Birth History
• Antenatal : TORCH infections
Drug intake
• Natal : Prematurity
Birth asphyxia
Neonatal hypoglycaemia
• Postnatal : Systemic diseases
Chromosomal disorders
Etiology
Congenital infections
• Congenital herpes simplex
• Congenital syphilis
• Cytomegalic inclusion disease
• Rubella
Others
• Cysticercosis
• Leprosy
• Onchocerciasis
• Toxoplasmosis
Chromosomal disorders
• Patau's syndrome
• Schmid-Fraccaro syndrome
• Trisomy 18 (Edward's
syndrome)
• Turner's syndrome
Disease of the skin and mucous
membranes
• Atopic dermatitis
• Basal-cell nevus syndrome
• Ichthyosis
• Pemphigus
Etiology
Metabolic and nutrition diseases
• Aminoaciduria(Lowe's
syndrome
• Diabetes mellitus
• Fabry's disease
• Galactosemia / Galactosemic
Cataract
• Homocystinuria
• Hypervitaminosis D
• Hyperparathyroidism
• Hypothyroidism
Other multisystem disorders
• Alport's syndrome
• Conradi's syndrome
• Myotonic dystrophy
• Mucopolysaccharidoses
• Wilson's disease
• Infectious diseases
Toxic substances introduced
systemically
• Corticosteroids
• Haloperidol
• Miotics
• Triparanol
Physical Evaluation
• Physician / Paediatrician evaluation
Ophthalmic Evaluation
Visual Significance : Concept of “ functional cataract ”
• Assessment of red reflex
( before and after dilatation )
• Visual improvement after
dilation
Ophthalmic Evaluation
Poor Prognostic Indicators
Ocular alignment and motility
Strabismus
Manifest latent nystagmus
Anterior segment
HCD, dysgenesis- anirdia,corneal
dystrophy,anterior cleavage
syndromes ,lens anomaliesmicrospherophakia
Posterior segment
Choroideremia , RP , PHPV, vitreo
retinal degenerations – Wagner’s
Ophthalmic Evaluation – EUA
• Axial length for prognosis / IOL calculation
• Keratometry
• Morphological evaluation of cataract with slit lamp
under EUA whenever possible
• B scan to assess posterior segment
Zonular Cataract
• Opacification of lens material
between clear nucleus
and cortex
• Intrauterine insult
• Bilateral
• Progressive
Nuclear cataract
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Opacification of nucleus
Bilateral
Dense
Associated with AS dysgenesis
Anterior Polar Cataract
•Dense, usually circular and
well-defined opacity on the
anterior pole of the lens
•Usually symmetric and
bilateral but doesn't have to be
•May protrude slightly into the
anterior chamber like a small
pyramid
Posterior Lenticonus
•Dense, usually circular and
well-defined opacity on
the posterior pole of the lens
•Usually symmetric; may be
misdiagnosed as posterior
subcapsular cataract
•May protrude into the lens like a
pyramid
Cerulean Cataract
•Small, bluish punctate opacities
of the peripheral cortex
(anterior, posterior or both)
•Often associated with
other cataract types
•May exhibit sectoral
distribution but tends to
be symmetric OU
Coronary Cataract
•Single or multiple
finger- or bowling pin-shaped
opacities that ring the
peripheral cortex
•Often associated with
cerulean cataracts
Mittendorf Dot
•Mittendorf's dot is an embryological
remnant of the hyaloid artery that is
attached to the posterior surface of the
lens
• appears as a small punctate opacity
•As shown in the cross-section diagram
above, the lens may also have a
corkscrew "tail" of hyaloid artery
remnant attached to it.
Epicapsular stars
•Small light brown or tan
dots or star-shaped deposits
on the anterior capsule
• single or multiple
• unilateral or bilateral
•Are remnants of the tunica
vasculosa lentis
Sutural Cataract
•Dense opacity that "gloves"
the Y-suture
•May involve the anterior
or posterior Y-suture or both;
however, the anterior is the
most common
Pulverulent Cataract
•Hollow sphere of
punctate opacities involving
the fetal nucleus
•Subtle "bull's eye" may be
noted with ophthalmoscope
retro-illumination
•Usually bilateral
Total Cataract
• Often indicate natural progression from other cataracts
Others …
Morphology
Spoke like
Diagnosis
Fabry disease , diabetes
Multicolour flecks “
christmas tree ”
Hypoparathyroidism ,
myotonic dystrophy
Green “ sunflower ”
Lamellar “ oil droplet ”
Wilson disease
Galactosemia,
hypoglycaemia
Laboratory Evaluation
• Urine
- reducing substance
- amino acids
- microscopy
- protein
• RBC galactokinase
• Antibody titres – TORCH , VDRL
• Serum
- calcium, phosphorus, alkaline phosphatase,
ceruloplasmin
Management decisions
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Age
Visual disability
Lateraliy
Prognostic indicators
BCVA >20/60
Observation
BCVA<20/60
Signs of visual
compromise
Surgery
Cataract
Thank You