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先天性束带综合征。病理生理学与治疗

Congenital constriction band syndrome. Pathophysiology and treatment.

作者信息

Light T R, Ogden J A

机构信息

Dept. of Orthopaedic Surgery, Loyola University School of Medicine, Maywood, Illinois 60153.

出版信息

Yale J Biol Med. 1993 May-Jun;66(3):143-55.

PMID:8209551
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2588858/
Abstract

The clinical manifestations of 88 children with congenital constriction band syndrome involvement of the hand were reviewed. Seventy-five of these children had evidence of digital or limb amputations, with 235 upper limb amputations and 138 lower limb amputations. In the hand, digital amputations were most common in the index, middle, and ring fingers, whereas in the foot, amputations of the hallux were most often noted. Band indentation was often present at multiple levels. Proximal bands may be associated with neural compression. Syndactyly was invariably associated with a proximal interdigital sinus or cleft and was frequently associated with distal amputation. Examination of a 27-week gestation stillborn specimen having manifestations of congenital constriction band syndrome demonstrated the intrauterine biologic response to band constriction. The variable clinical manifestations of congenital constriction band syndrome can best be explained as the response of the growing, embryologically defined limb to intrauterine deformation or band-induced compression and ischemia.

摘要

回顾了88例先天性束带综合征累及手部的患儿的临床表现。其中75例患儿有手指或肢体截肢的证据,上肢截肢235例,下肢截肢138例。在手部,手指截肢最常见于示指、中指和环指,而在足部,拇趾截肢最为常见。束带压痕常出现在多个层面。近端束带可能与神经受压有关。并指总是与近端指间窦或裂隙相关,且常与远端截肢相关。对一例有先天性束带综合征表现的孕27周死产标本进行检查,显示了子宫内对束带压迫的生物学反应。先天性束带综合征的各种临床表现最好解释为胚胎学上定义的正在生长的肢体对子宫内变形或束带引起的压迫和缺血的反应。

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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c3d/2588858/e089dd7fc97e/yjbm00045-0009-b.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c3d/2588858/976315e70e29/yjbm00045-0011-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c3d/2588858/f25bb42725d7/yjbm00045-0011-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c3d/2588858/b54f283409d1/yjbm00045-0012-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c3d/2588858/b3c6390057a0/yjbm00045-0013-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c3d/2588858/407b4c23fe83/yjbm00045-0013-b.jpg
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