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Familial exudative vitreoretinopathy mimicking persistent hyperplastic primary vitreous
A Chang-Godinich1, E A Paysse, D K Coats
1Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, Texas Children's Hospital, Houston 77030, USA.
American Journal of Ophthalmology
|April 28, 1999
Summary
Familial exudative vitreoretinopathy can mimic persistent hyperplastic primary vitreous in infants, even with asymmetric eye involvement. Prompt treatment is crucial as fellow eye involvement can progress rapidly.
Area of Science:
- Ophthalmology
- Genetics
Background:
- Familial exudative vitreoretinopathy (FEVR) is a genetic disorder affecting retinal vascular development.
- Persistent hyperplastic primary vitreous (PHPV) is a congenital condition causing visual impairment.
Observation:
- A 6-day-old infant presented with unilateral microphthalmia and a retrolental plaque, initially diagnosed as PHPV.
- The fellow eye later showed peripheral retinal vascular changes and a fibrovascular ridge, indicative of FEVR.
Findings:
- The affected right eye was unsalvageable.
- The left eye developed exudative retinal detachment, which resolved with photocoagulation and cryotherapy.
- Treatment led to regression of vascular changes in the left eye.
Implications:
- Neonatal FEVR can present with highly asymmetric ocular involvement, mimicking PHPV.
- Early diagnosis and intervention are critical for managing FEVR and preventing rapid progression in the fellow eye.