Histopathological Study of a Case with Glaucoma Due to Sturge-Weber Syndrome

Abstract

Purpose:To investigate the cause of the secondary glaucoma in a case of Sturge-Weber syndrome by histopathology. Case:A 10-year-old boy with Sturge-Weber syndrome and glaucoma in the right eye was studied. Trabeculectomy was performed because of uncontrolled intraocular pressure, and the trabeculectomy specimen was examined histologically by both light and electron microscopy.Results: Histological examination of the trabeculectomy specimen showed that the ciliary muscle was dislocated anteriorly, and the Schlemm canal was not present. The spaces in the juxtacanalicular connective tissue (JCT) were replaced by vascular structures and connective tissue. There were two kinds of vascular structures: in one, the endothelium was surrounded by pericytes; and in the other, the endothelium was not surrounded by pericytes. Conclusions

Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome

“…The spaces in the juxtacanalicular connective tissue were replaced by vascular structures and connective tissue. 10 Most likely, veins draining aqueous from the canal of Schlemm are part of an intrascleral or episcleral hemangioma. The canal of Schlemm itself may be part of the hemangioma.…”

Developmental and childhood glaucoma

“…If the glaucoma occurs in infancy, an isolated trabeculodysgenesis type of angle anomaly usually is assumed, described in one case as due to abnormalities in the canal of Schlemm and juxtacanalicular tissue. 203 Some claim that this is sometimes responsive to goniotomy, 204,205 with more than one procedure frequently required. Medical therapy effectively controls the glaucoma in but a third of pediatric cases.…”