Evaluation of Paediatric Cataract Dr Sunayana Bhat Consultant

Evaluation of Paediatric Cataract Dr Sunayana Bhat Consultant Paediatric Ophthalmology , Strabismus and Neuro Ophthalmology. Vasan Eye Care , Mangalore Ex faculty , Father Muller Medical College chanyn 9@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 • • • 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 • • 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 Multicolour flecks “ christmas tree ” Diagnosis Fabry disease , diabetes 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 • • Age Visual disability Lateraliy Prognostic indicators BCVA >20/60 Observation BCVA<20/60 Signs of visual compromise Surgery Cataract

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