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Wydanie 1/2006

Sierpowate odwarstwienie siatkówki

Congenital Falciform Retinal Detachment

Ewa Wójcik

Klinika Okulistyczna Wojskowego Instytutu Medycznego Centralnego Szpitala Klinicznego Wojskowej Akademii Medycznej w Warszawie Kierownik: prof. dr hab. n. med. Andrzej Stankiewicz

Summary: This article concerns less known clinical entity described as congenital falciform retinal detachment. Described case shows falciform fold running in vitreous body, built from double layer retina supplied by branches of central retinal artery. The essence of the clinical entity is in disorder of forming retinal structure. Incoming fold is not folded regularly built retina, but tissue coming from vitreous body fetal artery sheath. There is not any fluid between fold and retinal pigment epithelium. In most cases patients still have stable (more or less) visual acuity (it depends on geographical course of fold as well as coexisting anomalies in the eyeball).
Apposition of the retina by operational trial has no chance for success. However, there are cases when surgical intervention is justified. In Norrie's disease, where AVR coexists with CNS injury and organ of hearing, sometimes vitreous hemorrhage occurs. Surgical intervention allows eyeball preservation, unfortunately without visual function improvement – in case of treatment cessation it results in neovascularization (iris, irido-corneal angle, vitreoretinal traction creation), posterior adhesions arise, anterior chamber shallows, retina’s being totally detached and in consequence eyeball undergoes atrophy.

Keywords: congenital falciform fold, retinal dysplasia, Norrie's disease.

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