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先天性胫骨纵向缺损

Congenital longitudinal deficiency of the tibia.

作者信息

Schoenecker P L, Capelli A M, Millar E A, Sheen M R, Haher T, Aiona M D, Meyer L C

机构信息

Shriners Hospital for Crippled Children, St. Louis, Missouri 63131.

出版信息

J Bone Joint Surg Am. 1989 Feb;71(2):278-87.

PMID:2918013
Abstract

Fifty-seven patients (seventy-one limbs) who had congenital longitudinal deficiency of the tibia (tibial hemimelia) were retrospectively categorized according to radiographic type (Types 1 through 4, as described by Jones et al.). At an average follow-up of nine years, fifty-six of fifty-seven patients walked independently. An ablative surgical procedure was performed on sixty-one of the seventy-one lower extremities. According to the classification of Jones et al., fifty-four limbs had a Type-1 (a or b) or Type-2 deficiency. In twenty-two of these extremities, disarticulation of the knee was performed; in twenty-five, a Syme amputation; and in one, a Chopart amputation. The ipsilateral foot was retained in six extremities that had a severe Type-1 or Type-2 deficiency. Medial transfer of the fibula (the Brown procedure) generally yielded less than satisfactory results; in ten of fourteen extremities, one or more additional operations were needed. Seventeen extremities were classified as having a Type-3 or Type-4 deficiency; Syme amputation was done in nine and Chopart amputation, in four. Despite satisfactory reconstruction of the ankle, a Syme amputation was necessary in most extremities that had a Type-4 deficiency because a major leg-length discrepancy was projected. In four limbs that had a Type-3 or Type-4 deficiency, the foot was retained.

摘要

对57例(71条肢体)先天性胫骨纵向缺损(半侧胫骨发育不全)患者,根据影像学类型(Jones等人描述的1至4型)进行回顾性分类。平均随访9年时,57例患者中有56例可独立行走。71条下肢中的61条进行了截肢手术。根据Jones等人的分类,54条肢体为1型(a或b)或2型缺损。在其中22条肢体上进行了膝关节离断术;25条进行了Syme截肢术;1条进行了Chopart截肢术。6条患有严重1型或2型缺损的肢体保留了同侧足部。腓骨内侧移位术(Brown手术)的效果通常不尽人意;14条肢体中有10条需要进行一次或多次额外手术。17条肢体被归类为3型或4型缺损;9条进行了Syme截肢术,4条进行了Chopart截肢术。尽管踝关节重建效果良好,但大多数4型缺损的肢体仍需进行Syme截肢术,因为预计会出现较大的下肢长度差异。4条3型或4型缺损的肢体保留了足部。

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