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完全性单侧下肢重复畸形伴同侧肾缺如。

Complete unilateral leg duplication with ipsilateral renal agenesis.

作者信息

Lilje Christian, Finger Leron J, Ascuitto Robert J

机构信息

Department of Pediatrics, Tulane University Health Sciences Center, New Orleans, LA, USA.

出版信息

Acta Paediatr. 2007 Mar;96(3):464-5. doi: 10.1111/j.1651-2227.2007.00127.x.

Abstract

UNLABELLED

A case of a 5-month-old infant with complete duplication of the right leg and ipsilateral renal agenesis is presented. There was also a duplicated scrotum, posterior urethral valves with hydronephrosis, abnormal pelvic bones, and a ventricular septal defect. Complete caudal duplication is exceedingly rare. Associated defects are common. Several aetiological theories have been proposed. Among them, an underlying polytopic (acro-renal) developmental field defect has been suggested. It best explains the specific findings in our patient. However, the aetiology of caudal duplication syndromes may be multifactorial. In the light of twinning theories, associated duplication of hindgut derivates should be suspected in similar cases. Further research is needed. The motor-skeletal functional outcome after leg duplication surgery is mostly favourable.

CONCLUSION

A case of complete unilateral leg duplication with ipsilateral renal agenesis is presented. It appears to support the theory of a polytopic developmental field defect.

摘要

未标注

本文报告了一例5个月大的婴儿,其右腿完全重复且同侧肾缺如。此外,还有阴囊重复、后尿道瓣膜伴肾积水、骨盆骨骼异常以及室间隔缺损。完全性尾端重复极为罕见。相关缺陷很常见。已经提出了几种病因学理论。其中,一种潜在的多部位(肢-肾)发育场缺陷被提出。这最能解释我们患者的具体发现。然而,尾端重复综合征的病因可能是多因素的。根据双胎理论,在类似病例中应怀疑后肠衍生物的相关重复。需要进一步研究。腿部重复手术后的运动-骨骼功能结果大多良好。

结论

本文报告了一例完全性单侧腿部重复伴同侧肾缺如的病例。这似乎支持多部位发育场缺陷理论。

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