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[哈勒曼-施特雷夫综合征一例的自发性双侧晶状体吸收]

[Spontaneous bilateral lens resorption in a case of Hallermann-Streiff syndrome].

作者信息

Soriano J M, Funk J

机构信息

Universitäts-Augenklinik Freiburg.

出版信息

Klin Monbl Augenheilkd. 1991 Sep;199(3):195-8. doi: 10.1055/s-2008-1046071.

Abstract

The Hallermann-Streiff-syndrome was first described in 1948 by Hallermann and in 1950 by Streiff. The most common features are dyscephalia, cataract, microphthalmia, dental anomalies, hypotrichosis, cutaneous atrophy, and nanism. Anomalies of the eye include cataract, microphthalmia, nystagm, strabism, blue sclera, fundus anomalies and combined anomalies of all segments of the eye. The frequency of cataract is about 90%. Spontaneous resorption of the lens is described in about 8%. We present a five year old girl showing the typical dyscephalia of the Hallermann-Streiff-syndrome (bird face). Her lenses were replaced by opaque membranes. These membranes presumably were remnants of the posterior lens capsule.

摘要

哈勒曼-施特雷夫综合征于1948年由哈勒曼首次描述,1950年由施特雷夫描述。最常见的特征是头颅异常、白内障、小眼症、牙齿异常、毛发稀少、皮肤萎缩和身材矮小。眼部异常包括白内障、小眼症、眼球震颤、斜视、蓝色巩膜、眼底异常以及眼部各节段的联合异常。白内障的发生率约为90%。约8%的病例有晶状体自发吸收的描述。我们报告一名5岁女孩,表现出哈勒曼-施特雷夫综合征典型的头颅异常(鸟脸)。她的晶状体被不透明膜替代。这些膜可能是晶状体后囊的残余物。

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