Dominant Exudative Vitreoretinopathy and other Vascular Developmental Disorders of the Peripheral Retina
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Monographs in Ophthalmology 5
Dr W. JUNK PUBLISHERS THE HAGUE/BOSTON/LONDON
C .E . van NOUHUYS
Dominant Exudative Vitreoretinopathy and other Vascular Developmental Disorders of the Peripheral Retina
Dr W. JUNK PUBLISHE RS THE HAGUE/ BOSTON/ LONDON
Distribu tors:
for the United States and Canada Kluwer Boston Inc. 190 Old Derby Street Hingham, MA 02043 USA for all other countries Kluwer Academic Publishing Group Distribution Center P.O. Box 322 3300 AH Dordrecht The Netherlands
ISBN-13: 978-94-009-8023·5 e-ISBN-13: 978-94-009-8021·1 DOl: 10.1007/978-94-009-8021·1
Copyright © 1982 Dr W. Junk bv Publishers, The Hague, The Netherlands. Softcover reprint of the hardcover 1st edition 1982
All rights reserved. No part of this publication may be reproduced stored in a retrival system, or transmitted in any form or by any means, mechanical, photocopying, recording, or otherwise, without the prior permission of the publishers. Dr W. Junk bv Publishers, P.O. Box 13713, 2501 ES The Hague, The Netherlands.
PREFACE
Dominant exudative vitreoretinopathy (DEVR) is an eye disease which has only recently received wider attention. In 1969 Criswick and Schepens used the designation "familial exudative vitreoretinopathy" to describe a syndrome they observed in six patients belonging to two families. The condition was characterized by several symptoms involving the vitreous and retina, e.g. "posterior vitreous detachment, organized vitreous membranes, heterotopia of the macula, retinal neovascularizations, subretinal and intraretinal exudates, and localized retinal detachment". The clinical features impressed the authors as strongly reminiscent of retrolental fibroplasia, but none of the patients had a record of premature birth or postnatal oxygen administration. In 1971 Cow and Oliver described the same syndrome in several members of one family. They considered their findings to be compatible with autosomal dominant transmission. Canny and Oliver (I976) were the first to demonstrate the fluorescein-angiographic changes of DEVR in four members of the abovementioned family. The most striking finding was "abrupt cessation of the capillary network in a scaloped edge near the equator". Fluorescein was seen to leak from the retinal vessels localized in this marginal zone, and in some eyes from massive fibrovascular lesions as well. Similar fluoresceinangiographic changes have been described in recent years in other reports on families with DEVR (Nijhuis et aI., 1979; Slusher and Hutton, 1979; Dudgeon, 1979; Ober et a1., 1980; Laqua, 1980). In 1979 I commenced a clinical study of this still little-known condition at the Nijmegen University Institute of Ophthalmology (The Netherlands). This venture was prompted by the fact that two families with DEVR had been known for some time at this institute. The study led to identification of the disease in seven more families. The findings obtained in this total of nine families are presented and discussed in this thesis. Part I briefly reviews the normal development and anatomy of the re
