Glaucoma and expulsive hemorrhage mechanisms in the Sturge-Weber syndrome

An eye from an adult with Sturge-Weber syndrome and glaucoma is described histologically following enucleation for massive choroidal hemorrhage. The specimen is noted to contain hemangiomas of the episclera and choroid as well as a partial angle cleavage abnormality. Histologic evidence reviewed herein supports the concept that infantile glaucoma associated with this syndrome may be associated with marked filtration angle cleavage deformities. Some Sturge-Weber patients who develop unilateral glaucoma later as juveniles or adults appear to have milder expressions of angle cleavage abnormality or a forme fruste of this angle disorder. Any surgical procedure for this glaucoma preferably should be one which minimizes or eliminates operative hypotony, thus avoiding a possible expulsive hemorrhage.