Aniridi ile İlişkili Konjenital Glokomlu Bir Olguda Alt Kapak Epiblefaron Cerrahisi: Olgu Sunumu

Year 2022, Volume: 55 Issue: 2, 143 - 145, 31.08.2022

Derya Doğanay , Yasemin Katircioglu , Gozde Orman

https://doi.org/10.20492/aeahtd.1018216

Abstract

Aniridi, kromozom 11p13 üzerinde bulunan PAX6 genindeki bozukluğa bağlı gelişen kısmı veya tam iris defektidir. Aniridiye konjenital katarakt, konjenital glokom, keratopati veya foveal aplazi gibi oküler anomaliler eşlik edebilir. Epiblefaronun etyopatojenezi tam olarak bilinmemektedir. Bununla beraber epiblefaron etyopatogenezinde konjenital cilt katlanma anomalisi ve alt kapak retraktörlerinin yanlış yapılanması olduğu düşünülmektedir. Karotiko-kavernöz fistül, tiroid oftalmopati, megalo-oftalmi ve konjenital glokom gibi oftalmolojik hastalıklarla birlikteliği bildirilmiştir. Bu çalışmada aniridiye bağlı konjenital glokom ile takipli Doğu-Asyalı dört yaş erkek olguda gelişen epiblefaronun tanı ve tedavisinin sunulması amaçlandı. Özgeçmişinde atrial septal defekt bulunan hastanın her iki gözünde alt kapakta epiblefaron, aniridi, mikrosferofaki ve katarakt mevcuttu. Bu olguya epiblefaron nedeniyle alt kapak retraktörleri ile orbiküler kası hedef alan cerrahi teknik uygulandı.

Keywords

Epiblefaron, konjenital glokom, aniridi, mikrosferofaki, Epiblefaron, konjenital glokom, aniridi, mikrosferofaki

Supporting Institution

yoktur

Project Number

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Thanks

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Lower Lid Epiblepharon Surgery in a Case with Congenital Glaucoma Associated with Aniridia: Case Report

Abstract

Aniridia is complete or partial iris defect that develops due to a defect in the PAX6 gene located on chromosome 11p13. Aniridia may be accompanied by ocular findings such as congenital cataract, congenital glaucoma, keratopathy or foveal aplasia. Epiblepharon is congenital eyelid anomaly with fully unknown etiopathogenesis. However, in the etiology of epiblepharon is thought that congenital skin fold anomaly and wrongly structured lower eyelid retractors. Its association with ophthalmologic diseases such as caroticocavernous fistula, thyroid ophthalmopathy, megalo ophthalmia and congenital glaucoma has also been reported. In this study, we aimed to present the diagnosis and treatment of epiblepharon developed in an East-Asian four-year-old male patient with congenital glaucoma due to aniridia. The patient had a history of atrial septal defect and had epiblepharon, aniridia, microspherophacia and cataract on both eyes. Surgical technique targeting the orbicular muscle with lower eye lid retractors was applied to this case due to epiblepharon.

Keywords

Epiblefaron, konjenital glokom, aniridi, mikrosferofaki, Epiblefaron, konjenital glokom, aniridi, mikrosferofaki

Project Number

yoktur

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