Congenital pupillary-iris-lens membrane with goniodysgenesis (a new entity).
We encountered two clinically similar but genetically unrelated cases of a disorder characterized by an unusual white pupillary-iris-lens membrane with extension to a prominent Schwalbe's line, the membranes were vascularized and their appearance changed over time. In both cases the abnormality was unilateral. Anomalous chamber angles were seen on gonioscopy. The clinical appearance is similar to a combination of iridogoniodysgenesis and pupillary membrane. However, our two cases are unique and fit into neither category. We postulate that this is a new entity consisting of an anomalous chamber angle and iris-lens membrane resulting from a localized iris (pupillary membrane), infarct with secondary neovascularization, during formation of the chamber angle.
D000867:Anterior Chamber / Q000002:abnormalities; D005128:Eye Diseases / Q000151:congenital / Q000473:pathology / Q000503:physiopathology; D005260:Female; D005451:Fluorescein Angiography; D006068:Gonioscopy; D006801:Humans; D007231:Infant, Newborn; D007498:Iris / Q000473:pathology / Q000648:ultrastructure; D007499:Iris Diseases / Q000151:congenital* / Q000473:pathology / Q000503:physiopathology; D007908:Lens, Crystalline / Q000002:abnormalities; D008297:Male; D008854:Microscopy, Electron; D011680:Pupil