Acute postnatal cataract formation in Smith-Lemli-Opitz syndrome.
We report on two patients with severe Smith-Lemli-Opitz syndrome who acutely developed postnatal cataracts. These postnatal cataracts appear to be due to a different pathological mechanism and have a different clinical course than the typical congenital cataracts that occur in SLOS. The rapid development of these cataracts suggests that dysfunction or rupture of the lens capsule leading to acute osmotic shifts contributes to their formation. In addition, cataract formation in these patients was associated with conjunctival injection, fibrotic irido-lenticular adhesions, and capsular fibrosis. These findings are suggestive an inflammatory component that is consistent with impaired competence of the lens capsule.
Goodwin
Halima
H
Heritable Disorders Branch, National Institute of Child Health and Human Development, NIH, DHHS, Bethesda, Maryland 20892, USA.
Brooks
Brian P
BP
Porter
Forbes D
FD
D002386:Cataract / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D007223:Infant; D019082:Smith-Lemli-Opitz Syndrome / Q000175:diagnosis*