The ocular pathology of Marfan's syndrome, including a clinicopathological correlation and an explanation of ectopia lentis.

Abstract

Because of numerous and striking ocular abnormalities, Marfan's syndrome has always interested ophthalmologists. In spite of the clinical interest and the shortened life span of many of these patients, few histopathologic studies of the Marfan eye have been reported. Theobald, 1 in 1941, presented the first histologic report, and Reeh and Lehman, 2 in 1954, the second. Other less detailed studies were reported by Fischl, 3 Bergstrand, 4 Moses, 5 and Tobin. 6 Recently, Burian and Allen 7 described in detail the histopathology of the chamber angle of the previously reported cases of Theobald, Reeh and Lehman, and de Buen and Velazquez. They demonstrated unusual abnormalities of the chamber angle indicating an atavistic anomaly. These included prominent pectinate ligament fibers, anomalies of the outflow channel system, underdevelopment of the circular ciliary muscle, disarrangement of uveal trabecular fibers connecting with the ciliary muscles, and passage of longitudinal muscle fibers internal to