Fibrous congenital iris membranes with pupillary distortion

In 1986 Cibis and associates described 2 children with a new type of congenital pupillary-iris-lens membrane with goniodysgenesis that was unilateral, sporadic, and progressive. These membranes were different from the common congenital pupillary strands that extend from 1 portion of the iris collarette to another or from the iris collarette to a focal opacity on the anterior lens surface. They also differed from the stationary congenital hypertrophic pupillary membranes that partially occlude the pupil, originating from multiple sites on the iris collarette, but not attaching directly to the lens.

The present report is an account of 7 additional infants with congenital iris membranes, similar to those reported by Cibis and associates, which caused pupillary distortion and were variably associated with adhesions to the lens, goniodysgenesis, and progressive occlusion or seclusion of the pupil. Six of the 7 patients required surgery to open their pupils for visual purposes or to abort angle closure glaucoma. A remarkable finding was that the lenses in the area of the newly created pupils were clear, allowing an unobstructed view of normal fundi.

This type of fibrous congenital iris membrane is important to recognize because of its impact on vision and its tendency to progress toward pupillary occlusion. Timely surgical intervention can abort this progressive course and allow vision to be preserved.