[Glaucoma with Axenfeld-Rieger syndrome in three generations. Aqueous humor dynamics]

A 21-year-old woman was diagnosed as having glaucoma associated with Axenfeld-Rieger syndrome. Her family was examined and 3 additional patients with Axenfeld-Rieger syndrome were found in three generations. All of them had glaucoma with various types of onset. Aqueous humor dynamics were studied in 5 eyes of 3 patients using fluorophotometry. The basic secretion of aqueous humor in 5 eyes was 4.18 +/- 1.13 microliter/min. Averaged uveoscleral outflow in 3 eyes with low transtrabecular outflow facility was 61% of total aqueous outflow. It appeared that uveoscleral outflow was increased to compensate for the impaired transtrabecular outflow route.