OVERGROWTH MANAGEMENT IN KLIPPEL-TRENAUNAY-WEBER AND PROTEUS SYNDROMES

被引：24

作者：

GUIDERA, KJ

BRINKER, MR

KOUSSEFF, BG

HELAL, AA

PUGH, LI

GANEY, TM

OGDEN, JA

机构：

[1] Shriners Hospital for Crippled Children, Tampa Unit, Tampa, FL

[2] The University of South Florida, Tampa, FL

[3] Tulane University School of Medicine, New Orleans, LA

来源：

JOURNAL OF PEDIATRIC ORTHOPAEDICS | 1993年 / 13卷 / 04期

关键词：

ASYMMETRIC OVERGROWTH; JUDICIOUS SURGERY; KLIPPEL-TRENAUNAY-WEBER SYNDROME; PROTEUS SYNDROME; SYSTEMIC MANIFESTATIONS;

D O I：

10.1097/01241398-199307000-00009

中图分类号：

R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学（修复外科学）];

学科分类号：

摘要：

Twenty-eight patients with limb overgrowth and the diagnosis of Klippel-Trenaunay-Weber or Proteus syndromes were evaluated retrospectively. These disorders are part of the phakomatosis spectrum of syndromes. The orthopedic problems consisted of asymmetric limb overgrowth, localized gigantism, angular deformities, scoliosis, vascular malformations, and skin anomalies. Systemic abnormalities are common and deserve full evaluation before treatment. Surgical treatment consisted of epiphysiodesis, osteotomies, debulking procedures, and amputation. Mixed results were obtained with surgery, and conservative or supportive treatment should be the primary mode of orthopedic care.

引用

页码：459 / 466

页数：8

共 38 条

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