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两名兄弟姐妹患亚当斯-奥利弗综合征及先天性皮肤发育不全

Adams-Oliver syndrome and isolated aplasia cutis congenita in two siblings.

作者信息

Rajabian Mohammad Hossain, Aghaei Shahin

机构信息

Department of Plastic Surgery, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Dermatol Online J. 2006 Oct 31;12(6):17.

Abstract

Adams-Oliver syndrome is a rare congenital anomaly complex characterized by aplasia cutis congenita (ACC) and terminal transverse limb defects. We present a 9-year-old girl with a large, congenital scalp defect on her vertex, without underlying bone defect. Brachydactyly and syndactylia of her toes were also evident. Her 13-year-older brother had an 8 x 5 cm scalp defect without any limb defects (isolated ACC).

摘要

亚当斯-奥利弗综合征是一种罕见的先天性异常综合征,其特征为先天性皮肤发育不全(ACC)和四肢末端横向缺损。我们报告一名9岁女孩,其头顶有一个巨大的先天性头皮缺损,下方无骨质缺损。她的脚趾还存在短指畸形并指畸形。她13岁的哥哥有一个8×5厘米的头皮缺损,无任何肢体缺损(孤立性ACC)。

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