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Hereditary Hyaloideoretinal Degeneration and Palatoschisis
A. Th. M. van Balen, MD;
E. L. F. Falger, MD
Arch Ophthalmol. 1970;83(2):152-162.
Abstract
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.
Author Affiliations
Rotterdam, Netherlands; Utrecht, Netherlands
From the Department of Ophthalmology, Faculty of Medicine, Rotterdam (Dr. van Balen), and the Department of Ophthalmology, University of Utrecht, Utrecht (Dr. Falger), Netherlands.
Footnotes
Submitted for publication May 16, 1969.
Reprint requests to Medische Faculteit Rotterdam, Afdeling, Oogheelkunde, Rotterdam 1, Netherlands (Dr. van Balen).
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