先天性静脉畸形骨肥大综合征合并膀胱血管瘤一例报告并文献复习  

Klippel-Trenaunay syndrome associated with bladder hemangioma： a case report and literature analysis

作　　者：张震宇[1] 李汉忠[1] 张玉石[1] 毛全宗[1] 牛吉瑞[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院泌尿外科,100730

出　　处：《中华泌尿外科杂志》2011年第11期775-777,共3页Chinese Journal of Urology

摘　　要：目的提高对先天性静脉畸形骨肥大综合征合并膀胱血管瘤患者的诊治水平。方法患者，女，23岁。主因间断无痛性全程肉眼血尿1个月，发现膀胱肿物20d于2010年11月人院。外院膀胱活检病理提示血管瘤病。既往：左下肢血管瘤23年，分别于3个月、3岁、13岁时行左下肢血管瘤切除术。查体见左下肢膝关节以下明显增粗并长于右侧，左小腿及足踝部软组织增生。B超检查提示膀胱左前壁实性占位，范围4．1cm×3．0cm×2．5cm。膀胱镜检可见膀胱顶部略偏左侧黏膜隆起并可见曲张静脉血管，范围3．0cm×5．0cm。结果患者诊断为膀胱血管瘤，先天性静脉畸形骨肥大综合征。行膀胱部分切除术。术后病理为膀胱肌间血管瘤。术后肉眼血尿消失。术后3个月复查B超，膀胱未见异常。结论先天性静脉畸形骨肥大综合征可能合并膀胱血管瘤，膀胱部分切除术可以作为治疗方法之一。Objective To discuss the treatment of a rare case of Klippel-Trenaunay syndrome associated with bladder hemangioma and conduct a brief literature review. Methods A 23-year-old female manifested episodes of painless hematuria for 1 month. A biopsy by cystoscopy diagnosed angiomatosis. The patient had a history of left lower limb hemangioma for 23 years, and underwent 3 surgical operations at 3 months old, 3 years old, and 13 years old. The left leg was longer than the right leg. There was some soft tissue hypertrophy in the left lower extremity. Ultrasonography showed a bladder mass. Cystoscopy revealed a 3 cm × 5 cm sized hemangiomatous on the left lateral and anterior wall of the bladder. Results The patient was diagnosed with bladder hemangioma and Klippel-Trenaunay syndrome. Partial cystectomy was performed. The pathological diagnosis was bladder intramuscular hemangioma. Conclusion Klippel-Trenaunay syndrome associated with bladder hemangioma can be treated by partial cystectomy.

关 键 词：膀胱肿瘤 血管瘤 KLIPPEL-TRENAUNAY-WEBER综合征 诊断 外科手术 

分 类 号：R737.14[医药卫生—肿瘤]