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Reis-Bücklers'角膜失養症-家族病例治療報告

Surgical Treatment of Reis-Bücklers' Corneal Dystrophy-Familial Cases Report

摘要


Reis-Bücklers'角膜失養症(Reis-Bücklers' corneal dystrophy)是一種顯性遺傳的角膜失養症,病患通常從小會有復發性角膜糜爛(recurrent corneal erosion)的症狀,成年後則視力逐漸減退,病灶主要在Bowman's層(Bowman's layer)附近,有不正常的膠原纖維(collagen fiber)堆積。 本篇提出一個家族的三個病例,一個針對復發隨角膜糜爛施以角膜上皮清創術(corneal epithelial debridement),配戴軟式眼鏡和人工淚水藥物治療;一個施以表淺性角膜切除術(superficial keratectomy)來治療;另一個則施以全層角膜移植術(penetrating keratoplasty)來治療,並將取下的角膜作組織切片檢查。

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並列摘要


Reis-Bücklers' dystrophy is an autosomal dominant inherited corneal disease and is characterized by an irregular, semiopaque accummulation of fibrous connective tissue in the area of Bowman’s membrane. Symptomatically, the recurrent corneal erosion occurred early in life and the vision decreased progressively. Different kinds of surgical methods, such as corneal epitheiial debridement, superficial keratectomy and penetrating keratoplasty, are chosed to treat 3 patients with Reis-Bücklers' dystrophy according to their clinical appearances. The excised corneal tissue was confirmed by histopathological examination. The postoperative follow-up period ranged from 14 months to 39 months. All the surgically treated eyes had improved vision and cessation of the recurrent erosions.

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