2020–2021 BCSC Basic and Clinical Science Course™
6 Pediatric Ophthalmology and Strabismus
Part II: Pediatric Ophthalmology
Chapter 23: Childhood Cataracts and Other Pediatric Lens Disorders
Dislocated Lenses in Children
Patients with Weill-Marchesani syndrome can be thought of as clinical opposites of patients with Marfan syndrome in that the former are characteristically short, with brachydactyly and short limbs. Inheritance can be autosomal dominant or recessive. Mutations in the ADAMTS10 gene have been identified in patients with this disorder. The lenses are microspherophakic. With time, the lenses may dislocate into the anterior chamber and cause pupillary block glaucoma. For this reason, prophylactic laser peripheral iridectomy or lensectomy may be indicated.
Figure 23-11 Homocystinuria. The lens may dislocate into the anterior chamber and cause acute pupillary block glaucoma.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.