Wydanie 1/2013
str. 43

Garbiak rogówki u noworodka – opis przypadku

Staphyloma in a Newborn – Case Report

Danuta Węgrzynowska1,2, Dorota Tomaszkiewicz-Mondry1, Magdalena Pilas-Pomykalska1,2, Piotr Grzybowski2, Anna Groblewska1

1 Klinika Okulistyki Instytutu „Centrum Zdrowia Matki Polki” w Łodzi
P.o. kierownika: dr n. med. Dorota Tomaszkiewicz-Mondry
2 Centrum Okulistyki „Oko Dziecka” Sp. z o.o.
Kierownik: dr Danuta Węgrzynowska


Summary: Corneal Staphyloma is severe, rare, mostly unilateral, sporadic congenital eye anomaly. It is characterized by staphylomatous, opaque cornea with superficial neovascularization, destruction of Bowman's layer, and absence of Descemet's layer as well as endothelium. This complex malformation should be distinguished from primary congenital glaucoma, scleorcornea, Peters anomaly and posterior keratoconus. We present a case of corneal staphyloma in a newborn female. Shortly after birth, a massive enlargement of the left eye was observed in an otherwise healthy child. The clinical, US, CT, MRI and histopathological features of the case are discussed. Because of the cosmetically unacceptable appearance and risk of perforation of the globe, the involved eye was enucleated. The morphological investigations of the eye disclosed staphylomatous cornea with superficial neovascularization, destruction of Bowman's layer, absence of Descemet's layer and endothelium, absence of angle structures and anterior chamber. Our case may reflect corneal staphyloma anterior chamber agenesia-microphakia syndrom syndrome (CSAMS). Medical treatment is oriented at improving the cosmetic appearance. Current treatment of choice is evisceration of the globe with primary scleral implant.

Słowa kluczowe: wrodzone anomalie gałki ocznej, garbiak rogówki.

Keywords: congenital eye abnormalities, corneal staphyloma.


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