June 05, 2009
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Congenital cataracts require immediate attention

An anxious young mother brings her newborn daughter to you for an evaluation. At birth, the pediatrician noted a poor red reflex of the right eye and has referred the patient to you. You note a healthy-appearing, 9-pound, full-term infant who is now 2 weeks old. Initial examination of her right eye shows a congenital cataract with significant blockage of the visual axis.

Congenital cataract in a newborn.
Congenital cataract in a newborn.

Congenital cataracts are lens opacities that are present at birth. The incidence in the U.S. is approximately two to six per 10,000 births, which means that nearly all ophthalmologists will encounter patients with congenital cataracts in their career.

Unilateral congenital cataracts tend to have different causes than bilateral ones, with the unilateral more often sporadic and not inherited. Unilateral congenital cataracts can sometimes be traumatic or associated with rubella, persistent hyperplastic primary vitreous or posterior pole tumors. Bilateral congenital cataracts are often inherited and typically associated with systemic diseases such as trisomy 21, trisomy 18, myotonic dystrophy, TORCH infections, prematurity and hypoglycemia. Particularly with bilateral congenital cataracts, these babies require a full systemic workup with the pediatrician and geneticist.

These tiny patients often need surgical treatment urgently in order to prevent dense amblyopia. Because the treatment merely begins with surgery, but lasts many years via rehabilitation and amblyopia therapy, a team approach with a pediatric ophthalmologist is recommended.