反向克莱佩尔-特劳纳综合征。
INVERSE KLIPPEL-TRENAUNAY SYNDROME.
机构信息
Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Centro Hospitalar Universitário de Lisboa Central, Lisboa, Portugal.
出版信息
Rev Paul Pediatr. 2020 Dec 18;38:e2020091. doi: 10.1590/1984-0462/2020/38/2020091. eCollection 2020.
OBJECTIVE
To report a rare case of inverse Kipplel-Trenaunay.
CASE DESCRIPTION
A 16-year-old girl with a grayish-depressed plaque on her left thigh. Angioresonance showed a vascular malformation affecting the skin and subcutaneous tissue.
COMMENTS
Inverse Klippel-Trenaunay is a Klippel-Trenaunay syndrome variation in which there are capillary and venous malformations associated to hypotrophy or shortening of the affected limb. Modifications on the limb's length or width result from alterations in bones, muscles, or subcutaneous tissues. It has few described cases. Further clinical and molecular studies must be performed for a proper understanding.
目的
报告一例罕见的逆 Kipplel-Trenaunay 病例。
病例描述
一名 16 岁女孩，其左大腿有一块灰蓝色凹陷斑块。血管回声显示，一处血管畸形影响皮肤和皮下组织。
讨论
逆 Klippel-Trenaunay 是 Klippel-Trenaunay 综合征的一种变异，其特征是毛细血管和静脉畸形伴受累肢体萎缩或缩短。肢体长度或宽度的改变是由于骨骼、肌肉或皮下组织的改变引起的。这种病例很少见，需要进一步进行临床和分子研究以进行准确的理解。
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