Familial exudative vitreoretinopathy and persistent hyperplastic primary vitreous Mieko Nishimura 1 , Toshiko Yamana 2 1Koushinkai Med Corp 2Dept of Ophthalmol, Fac of Med, Kyushu Univ pp.576-580
Published Date 1988/5/15
DOI https://doi.org/10.11477/mf.1410210394
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Familial exudative vitreoretinopathy (FEVR) was associated with falciform retinal fold in 18 eyes, 15 cases, and with advanced form of anterior hyperplastic primary vitreous (PHPV) in 1 eye, 1 case. The falciform retinal fold was located in the temporal fundus in 16 eyes (89%).

The origin of the fold was either neovascular type (12 eyes) or primary vitreous type (6 eyes). In neovascular type, a fibrovascular membrane extended into the temporal equator or retrolental space attached to the fold. In 9 eyes, strand of persistent primary vitreous was also attached to the fold.

In primary vitreous type, strand of persist-ent primary vitreous was attached to the top of the fold. Fibrovascular membrane was either absent orinconspicuous. In the present series, there was no instance of optic disc anomaly nor generalized malformation except congenital heart disease in 3 cases.

We re-examined earlier cases diagnosed or suspected as PHPV. Out of 53 eyes with posterior PHPV, or falciform retinal fold, we could detect 43 eyes (81%) with FEVR. Out of 27 eyes with ante-rior PHPV, we could detect 3 eyes (11%) with FEVR.

The findings show that FEVR frequently under-lies apparent anterior PHPV, retrolental fibro-plasia and falciform retinal fold in full-term infants. Posterior PHPV is a misnomer for falcifor-m retinal fold due to FEVR.

Rinsho Ganka (Jpn J Clin Ophthalmol) 42(5) : 577-580, 1988

Copyright © 1988, Igaku-Shoin Ltd. All rights reserved.


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