.: OKULISTYKA :.

Wydanie 4/2013
str. 12

Nerwiakowłókniak towarzyszący małooczu z torbielą szczelinową oczodołu – opis przypadku

Neurofibroma Coexisting with Microphthalmia and Colobomatous Cyst of the Orbit – Case Report

Joanna Jędrzejczak-Młodziejewska, Krystyna Kanigowska, Anna Rogowska, Wojciech Hautz

Klinika Okulistyki „Instytutu – Pomnik Centrum Zdrowia Dziecka” w Warszawie
P.o. kierownika: dr n. med. Wojciech Hautz

Summary: Material and methods: A child presented at birth with left exophthalmia. Ocular examination showed correct right eye and microphthalmia with iris and choroid coloboma in the left eye. Computer tomography in the 2nd week of life showed two-chamber cyst with consequent orbit enlargement. The deformed eye had 14x13x14 mm with retinal calcification (2x2.5 mm). Because of the enlargement of the cyst in the 6th month of life, an aspiration has been performed. After the examination of the aspired fluid it has been classified as residual cerebrospinal fluid. Progressing exophthalmia gave reasons to the subsequent aspiration in the 8th month of life. Because of the lack of response to the treatment it has been decided to perform a neuro-ophthalmic surgery. At the age of 14 month the enucleation of the left eye, drainage of the cyst with orbit revision and preservation of the orbit apex and residual cyst walls with sutures and tissue adhesive had been carried out. Unexpectedly, histopathological examination of the cyst walls showed neurofibroma. The patient stays under ophthalmologic and oncological supervision. At present the patient is 2 years old and the cosmetic effect of treatment is satisfactory.
Results: Histopathological examination showed neurofibroma in the cyst walls.
Conclusions: In case of microphthalmia and colobomatous cyst of the orbit a neurofibroma may also occur.

Słowa kluczowe: torbiel szczelinowa, małoocze, nerwiakowłókniak.

Keywords: colobomatous cyst of the orbit, microphthalmia, neurofibroma.

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