ABSTRACT
In this retrospective study, we investigated vascular abnormalities in sarcoidosis using fluorescein gonioangiography (FGA) to detect angle neovascularization (ANV), fundus fluorescein angiography (FFA), and pathological specimens from the aspects of microangiopathy. In 57 sarcoidosis patients, clinical data was reviewed by dividing the cases into three groups (Group I: histologically diagnosed; Group II: positive bilateral hilar lymphadenopathy (BHL); Group III: negative BHL). The FFA, FGA, and pathological examination data in the autopsy eyes and trabeculectomy specimens were investigated. FGA and FFA detected ANV (91%) and nodule-associated abnormalities (87%), respectively. No intraocular pressure (IOP) elevation was observed after continuous topical betamethasone, except in the steroid responder group. Maximum IOP had significant correlation with nodules in the angle (p = 0.02696) and visual field defect (p = 0.0151). Granulomas adjacent to blood vessels, including the Schlemm's canal, and thickening of the retinal blood vessel wall caused occlusion of those vessels. Photocoagulation was required for retinal tears (14%) and the retinal blood vessel occlusion (7%). Suppression of IOP elevation via continuous topical betamethasone may be important to avoid irreversible outflow-route changes and optic-nerve damage, and the concept of microangiopathy in ocular sarcoidosis may be important for understanding the proper treatment of serious complications.
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: 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.
