[Klippel-Trenaunay-Weber综合征患者的脾脏囊性淋巴管瘤：MRI表现]

[Cystic lymphangioma of the spleen in a patient with Klippel-Trenaunay-Weber syndrome: MRI findings].

作者信息

Montes M, Ciudad M J, Cabeza B, Méndez R

机构信息

Departamento de Diagnóstico por Imagen, Hospital Clínico San Carlos. Madrid. España.

出版信息

Radiologia. 2007 Sep-Oct;49(5):355-7. doi: 10.1016/s0033-8338(07)73793-1.

DOI:10.1016/s0033-8338(07)73793-1

PMID:17910875

Abstract

We present the case of a 22-year-old woman with Klippel-Trenaunay-Weber syndrome who presented with clinical signs and symptoms of gastrointestinal obstruction. Ultrasonography and magnetic resonance imaging showed massive splenomegaly with multiple cysts replacing the normal parenchyma of the spleen. Histologic study after splenectomy confirmed the diagnosis of cystic lymphangioma of the spleen, which is an exceptional manifestation of Klippel-Trenaunay-Weber syndrome.

摘要

我们报告一例22岁患有克-特-韦综合征（Klippel-Trenaunay-Weber syndrome）的女性患者，其出现了胃肠道梗阻的临床体征和症状。超声检查和磁共振成像显示脾脏巨大，多个囊肿取代了脾脏的正常实质。脾切除术后的组织学研究证实为脾脏囊性淋巴管瘤，这是克-特-韦综合征的一种罕见表现。