PDF(Pubmed)
Abstract：
Introduction Acrorenal syndrome is autosomal recessive inherited disorder commonly associated with congenital renal disorders and ipsilateral hand/foot anomalies. The hand and foot deformities corrections are challenging to achieve a good functional and cosmetic result. We described a case of acrorenal syndrome with suppressed radial elements and absent thumb in whom we were able to reconstruct the hand. Case Report A 5-year-old girl presented to us with right renal agenesis and right hand/foot deformity. The hand showed a cleft hand with central deficiency, index finger hypoplastic, and syndactylyzed to middle finger, absent thumb. The hand was nonfunctional because of absent thumb. To improve the functions, it was decided to proceed with thumb reconstruction. The middle finger was pollicized to regain tripod grip and thereby the functions was enhanced. Discussion The cleft hand belongs to \"failure of finger ray induction group\" in classification by \"International Federation of Societies for Surgery of the Hand (IFSSH).\" Absence of thumb and first web space makes it a strong indication for surgical reconstruction. In our case, thumb was addressed by pollicization of middle finger and we were able to provide a good tripod grip.
摘要：
简介肩肾综合征是常染色体隐性遗传性疾病，通常与先天性肾脏疾病和同侧手/足异常有关。手部和足部畸形矫正对于实现良好的功能和美容效果是具有挑战性的。我们描述了一例肢端肾综合征，其桡骨成分受到抑制，拇指缺失，我们能够重建手。病例报告一名5岁女孩向我们介绍了右肾发育不全和右手/足畸形。手显示有中央缺陷的裂口手，食指发育不良,与中指同步，拇指缺席。由于拇指不存在，手没有功能。为了改善功能，决定继续拇指重建。对中指进行抛光以恢复三脚架的抓地力，从而增强了功能。讨论裂口手在“国际手外科协会联合会（IFSSH）的分类中属于“指线诱导失败组”。“没有拇指和第一网空间使其成为手术重建的有力指征。在我们的案例中,拇指通过中指的礼貌处理，我们能够提供良好的三脚架抓地力。
