Congenital pupillary-iris-lens membrane with goniodysgenesis.
BACKGROUND
A unilateral congenital pupil-iris-lens membrane with goniodysgenesis syndrome, not benign tunica vasculosa lentis, was first described by Cibis et al. One of three cases developed angle closure. Robb described catastrophic vision loss from angle closure in one of his seven cases.


METHODS
We did a retrospective review of previously unreported cases of pupil-iris-lens membrane with goniodysgenesis seen in our practices.


RESULTS
We report the clinical spectrum of a further nine cases, three of which needed surgery for angle closure, two of which needed surgery for clearing the visual axis.


CONCLUSIONS
Congenital pupil-iris-lens membrane with goniodysgenesis is a unilateral membrane clearly differentiated from benign persistent tunica vasculosa lentis tissue. The membrane represents ectopic iris on the lens with abnormal iris stroma and chamber angle from aberrant induction, migration, or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. Surgery may be needed to open the visual axis even when glaucoma is not present and may prevent angle closure.
Department of Ophthalmology, University of Kansas Medical Center, KS, USA.
Gerhard W
GW
Cibis
David S
DS
Walton
D000869:Anterior Eye Segment / Q000002:abnormalities*; D005124:Eye Abnormalities / Q000209:etiology* / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007429:Intraocular Pressure; D007499:Iris Diseases / Q000151:congenital* / Q000601:surgery; D007905:Lens Diseases / Q000151:congenital* / Q000601:surgery; D008566:Membranes / Q000473:pathology; D011681:Pupil Disorders / Q000151:congenital* / Q000601:surgery; D012189:Retrospective Studies; D013577:Syndrome; D014786:Vision Disorders / Q000209:etiology / Q000601:surgery; D014792:Visual Acuity