CHARGE ASSOCIATION WITH CONGENITAL GLAUCOMA DUE TO MALDEVELOPMENT OF THE ANTERIOR-CHAMBER ANGLE

Purpose: We examined the clinicopathologic features of a male infant w ith the CHARGE association and bilateral congenital glaucoma. Methods: Trabeculectomy specimens were obtained from the anterior chamber angl e and examined by light and electron microscopy. Results: Histopatholo gic examination of the trabeculectomy specimens showed immature develo pment of the trabecular meshwork that was covered by the ciliary muscl es. There were few intertrabecular spaces because the meshwork was alm ost completely filled with cells and extracellular substances. Deposit ion of a granular and/or homogeneous substance was observed in the sub endothelial area of Schlemm's canal. Conclusions: Our patient exhibite d features typical of the CHARGE association, but also had congenital glaucoma. We hypothesize that these clinical findings are all mediated by a neurocristopathic mechanism. Our findings suggest that the CHARG E association may predispose to anterior chamber angle maldevelopment, which can lead to congenital glaucoma.