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克-特综合征的多样表现及治疗选择:单中心10年经验

Diverse manifestations and management options in Klippel-Trenaunay syndrome: a single centre 10-year experience.

作者信息

Sreekar Harinatha, Dawre Sandeep, Petkar Kiran S, Shetty Rahul B, Lamba Shashank, Naik Sukriya, Gupta Ashish K

机构信息

Plastic and Reconstructive Surgery, Christian Medical College Vellore, India.

出版信息

J Plast Surg Hand Surg. 2013 Sep;47(4):303-7. doi: 10.3109/2000656X.2013.766201. Epub 2013 May 28.

Abstract

Klippel-Trenaunay syndrome is a well-known conglomeration of capillary malformations, bony or soft tissue hypertrophy, and abnormal deep or superficial veins. Although it generally presents with grossly enlarged limbs, it can present with more serious features like haematuria, haematochezia, and seizures. This retrospective study included patients admitted with the diagnosis of Klippel-Trenaunay syndrome in this institute from 2001-2010. The patients' demographic data, clinical features, associated findings, and treatments given were tabulated. A total of 19 patients were included in the study. Two patients presented with haematocezia and had to undergo bowel resection. Five presented with bleeding and ulceration. Debulking surgery was done in three of them. Patients also presented with abdominal distension, jaundice, seizures, and haematuria. Although the common presentation of varicose veins was treated with sclerotherapy, the treatment was tailored to each patient. Klippel-Trenaunay syndrome is a multifaceted disorder which can manifest in a number of different ways. These features may be missed by an unwary plastic surgeon treating them only for the limb hypertrophy.

摘要

克-特综合征是一种众所周知的毛细血管畸形、骨或软组织肥大以及深浅静脉异常的集合体。虽然它通常表现为肢体明显增粗,但也可能表现出更严重的特征,如血尿、便血和癫痫发作。这项回顾性研究纳入了2001年至2010年在本机构确诊为克-特综合征的患者。将患者的人口统计学数据、临床特征、相关发现及接受的治疗进行列表整理。该研究共纳入19例患者。两名患者出现便血,不得不接受肠道切除术。五名患者出现出血和溃疡,其中三名接受了减容手术。患者还出现腹胀、黄疸、癫痫发作和血尿。虽然对于常见的静脉曲张表现采用硬化疗法进行治疗,但治疗是根据每位患者的情况量身定制的。克-特综合征是一种多方面的疾病,可表现为多种不同形式。这些特征可能会被仅因肢体肥大而治疗的粗心整形外科医生所忽视。

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