Hereditary Hyaloideoretinal Degeneration and Palatoschisis

Examination of three large pedigrees gives additional evidence for the existence and irregular dominant hereditary syndrome of retinal degeneration and palatoschisis. The symptoms have a mutual variable expression. Formes frustes of retinal degeneration and palatoschisis have been found. We have also taken account of the fact that the retinal degeneration is apparent only after a certain age (15 years?). The retinal degeneration in this syndrome can be of different kinds but is probably always combined with vitreal degeneration and degeneration of other parts of the eye (lens, cornea). Surgical treatment of the retinal detachment in this syndrome is extremely unsuccessful. Prophylactic treatment is obligatory and has to be done early because presenile cataract is a frequently occurring associated symptom.