Guidera K J, Brinker M R, Kousseff B G, Helal A A, Pugh L I, Ganey T M, Ogden J A
Shriners Hospital for Crippled Children, Tampa Unit, FL 33612-9499.
J Pediatr Orthop. 1993 Jul-Aug;13(4):459-66. doi: 10.1097/01241398-199307000-00009.
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.
对28例肢体过度生长且诊断为克-特-韦综合征或变形综合征的患者进行了回顾性评估。这些疾病属于综合征性错构瘤谱系的一部分。骨科问题包括肢体不对称过度生长、局部巨大症、角状畸形、脊柱侧弯、血管畸形和皮肤异常。全身异常很常见，在治疗前值得进行全面评估。手术治疗包括骨骺阻滞术、截骨术、减容手术和截肢术。手术取得了混合结果，保守或支持性治疗应作为骨科护理的主要方式。
