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一名12岁男孩患戈洛普-沃尔夫冈复合体的病例报告。

A case report of Gollop-Wolfgang complex in 12 years old boy.

作者信息

Elewee Ahmad, Mayo Wafik, Mirali Bashar, Alaktaa Mohammad Ezzat, Hmaidy Osama

机构信息

Department of Orthopedic Surgery, Damascus Hospital, Damascus, Syria.

Faculty of Medicine, Aleppo University, Aleppo, Syria.

出版信息

Int J Surg Case Rep. 2023 May;106:108223. doi: 10.1016/j.ijscr.2023.108223. Epub 2023 Apr 15.

Abstract

INTRODUCTION

The Gollop-Wolfgang Complex (GWC) was initially described by Gollop et al. and is a rare congenital limb anomaly disorder characterized by the association of distal bifid femur and tibial agenesis.

CASE PRESENTATION

This study presents a case of a 12-year-old boy with Gollop-Wolfgang Complex (GWC), a rare congenital limb anomaly disorder characterized by the association of distal bifid femur and tibial agenesis. The patient did not have any VACTERL abnormalities and had a normal level of intelligence. Examination revealed coxa valga in both hips and upper limbs on both sides, a shortened left leg with a palpable bony protuberance and absence of the patella, and a shortened right leg with a palpable fibula lateral to the knee and absent tibia with severe knee varus deformity on both sides. Both feet revealed equinovarus deformity with ectrodactyly. The patient underwent through-knee amputation and was fitted with two prostheses to provide enhanced functional support.

CLINICAL DISCUSSION

The etiology of GWC is still unknown, but errors in the complex genetic control of limb development are believed to be related.

CONCLUSION

Treatment choice depends on the deformity type, with through-knee amputation recommended for cases with observed flexion contracture, bifid femur, and tibial hemimelia, followed by modern prosthesis fitting for optimal outcomes. This case illustrates the efficacy of this surgical management and highlights the need for ongoing follow-up care.

摘要

引言

戈洛普 - 沃尔夫冈复合体(GWC)最初由戈洛普等人描述,是一种罕见的先天性肢体异常疾病,其特征为远端双叉股骨与胫骨发育不全相关联。

病例报告

本研究报告了一例12岁患有戈洛普 - 沃尔夫冈复合体(GWC)的男孩,这是一种罕见的先天性肢体异常疾病,其特征为远端双叉股骨与胫骨发育不全相关联。该患者没有任何VACTERL异常,智力水平正常。检查发现双侧髋关节和上肢均有髋外翻,左腿缩短,有可触及的骨性突起且髌骨缺失,右腿缩短,在膝关节外侧可触及腓骨,胫骨缺失,双侧膝关节有严重内翻畸形。双足均表现为马蹄内翻畸形并伴有缺指(趾)畸形。该患者接受了经膝关节截肢手术,并安装了两个假肢以提供更好的功能支持。

临床讨论

GWC的病因仍然未知,但据信与肢体发育复杂基因控制中的错误有关。

结论

治疗选择取决于畸形类型,对于观察到有屈曲挛缩、双叉股骨和胫骨半侧发育不全的病例,建议行经膝关节截肢手术,随后进行现代假肢安装以获得最佳效果。本病例说明了这种手术治疗的有效性,并强调了持续随访护理的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9e66/10149289/a1af218446bb/gr1.jpg

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