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 7 weeks

(embryonic nucleus )

Ant / post Y sutures 12-14 weeks

(fetal nucleus )

Quick Revision : Lens Anatomy

Evaluation • 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 defectsAutosomal 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 • RubellaOthers • Cysticercosis • Leprosy• Onchocerciasis • Toxoplasmosis

Chromosomal disorders• Patau's syndrome• Schmid-Fraccaro syndrome • Trisomy 18 (Edward's

syndrome)• Turner's syndromeDisease 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 diseasesToxic 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 anomalies-microspherophakia

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 • 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 Diagnosis Spoke like Fabry disease , diabetes Multicolour flecks “ christmas tree ”

Hypoparathyroidism , myotonic dystrophy

Green “ sunflower ” Wilson disease Lamellar “ oil droplet ” 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 • Age • Visual disability • Lateraliy • Prognostic indicators