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对软骨发育不良(内生软骨瘤病)患者进行多阶段股骨和胫骨延长术的37年随访评估,净增长23.3厘米。

A 37-year follow-up evaluation of multiple-stage femur and tibia lengthening in dyschondroplasia (enchondromatosis) with a net gain of 23.3 centimeters.

作者信息

Urist M R

机构信息

Department of Surgery, University of California Medical Center, Los Angeles.

出版信息

Clin Orthop Relat Res. 1989 May(242):137-57.

PMID:2706845
Abstract

This case report presents the rare combination of circumstances of a child with (a) dyschondroplasia (enchondromatosis) limited to all the major epiphyseal plates of one lower extremity, (b) unusual capacity to cooperate, and (c) parents who refused permission to treat the limb by amputation but allowed the child to participate in a long-term clinical experiment. In dyschondroplasia (Ollier's disease), the long bones grow in diameter but not in length. In this condition, it was possible (by fasciotomy, osteotomy, and interposition of wedge blocks or hemicylindrical bone implants (stabilized by an intramedullary rod) to lengthen the leg 23.3 cm in ten stages, during the period between five and 14 years of age. With the additional procedure of epiphyseal arrest of the contralateral distal end of the femur at age 11, the eventual correction of discrepancy in leg length was 25.6 cm. Intraoperative and postoperative skin temperature recordings, toe plethysmography, and electromyography show that there were no deleterious changes in the circulatory or neurological functions of the extremity.

摘要

本病例报告呈现了一种罕见的情况组合

一名儿童患有(a)仅累及一侧下肢所有主要骨骺板的软骨发育异常(内生软骨瘤病),(b)具有不同寻常的配合能力,以及(c)父母拒绝同意对该肢体进行截肢治疗,但允许孩子参与一项长期临床实验。在软骨发育异常(奥利尔病)中,长骨直径增大但长度不增加。在此情况下,在5至14岁期间,通过筋膜切开术、截骨术以及插入楔形块或半圆柱形骨植入物(由髓内棒固定),分十个阶段将腿部延长了23.3厘米成为可能。在11岁时对股骨对侧远端进行骨骺阻滞的额外手术,最终腿部长度差异的矫正为25.6厘米。术中及术后的皮肤温度记录、趾容积描记法和肌电图显示,该肢体的循环或神经功能没有有害变化。

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