Evaluation of Paediatric Cataract
Dr Sunayana Bhat Consultant Paediatric Ophthalmology ,Strabismus and Neuro Ophthalmology.Vasan Eye Care , Mangalore Ex faculty , Father Muller Medical College [email protected]
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