Histopathological study of a case with glaucoma due to Sturge-Weber syndrome

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: Developmental abnormalities of the Schlemm canal and the JCT may have caused the glaucoma. These observations suggest that the developmental abnormalities of both the mesoderm and the neural crest might be involved in the pathogenesis of the glaucoma in cases of Sturge-Weber syndrome.