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Wydanie 4/2015
str. 19

Mezenchymalne dysgenezje przedniego odcinka oka

Anterior Segment Mesenchymal Ocular Dysgeneses

Andrzej Sawicki, Justyna Kaproń-Świś, Tomasz Żarnowski

Katedra Okulistyki Klinika Diagnostyki i Mikrochirurgii Jaskry Uniwersytetu Medycznego w Lublinie
Kierownik Kliniki: prof. dr hab. n. med. Tomasz Żarnowski

Summary: Mesenchymal dysgeneses of the cornea and iris include numerous disorders or groups of congenital defects. The aim of this work is to describe relevant abnormalities and review of medical literature. The most frequent abnormality is embryotoxon posterior – observed in 6.8% of patients, which is a component of Alagille syndrome and Axenfeld–Rieger syndrome. The Axenfeld–Rieger syndrome is autosomal dominant disorder or occurs spontaneously, includes Axenfeld anomaly, Rieger anomaly and Rieger syndrome. Axenfeld–Rieger syndrome is characterized by abnormalities in genesis of eyes and by non-ophthalmic symptoms like dental problems or hypertelorism. The next disorder is iris hypoplasia (aniridia) occurring in three clinical forms: AN1, AN2 and AN3. The most severe coexisting pathology of aniridia is Wilms tumor, hence these patients need strict follow up. Abnormal differentiation of neural crest cells, causing inter alia corneal opacity, is called a Peters syndrome. A congenital glaucoma is observed in 50% of these patients. The diagnosis of any of these disorders is the absolute indication for further thorough diagnostics in case of presence of other co-existing defects.

Słowa kluczowe: mezenchymalne dysgenezje przedniego odcinka oka, embryotoxon tylny, zespół Axenfelda–Riegera, anomalia Axenfelda, anomalia Riegera, zespół Riegera, aniridia, anomalia Petersa.

Keywords: anterior segment mesenchymal ocular dysgeneses, embryotoxon posterior, Axenfeld-Rieger syndrome, Axenfeld anomaly, Rieger anomaly, Rieger syndrome, Peters anomaly.

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