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肛门闭锁、肠旋转不良和先天性巨结肠病:一种罕见的关联。

Imperforate anus, malrotation and Hirschsprung's disease: a rare association.

作者信息

Poenaru D, Uroz-Tristán J, Leclerc S, Murphy S, Bensoussan A L

机构信息

Hôpital Sainte-Justine, Montreal, Quebec, Canada.

出版信息

Eur J Pediatr Surg. 1995 Jun;5(3):187-9. doi: 10.1055/s-2008-1066202.

DOI:10.1055/s-2008-1066202
PMID:7547811
Abstract

The association of imperforate anus with anomalies of rectal innervation is very rare. Moreover, malrotation only rarely accompanies these malformations. We report 2 patients with this unusual triple association and its impact on their management. Retrospectively both patients presented at birth with the same radiological picture of distended distal colon and right-sided small bowel location. We discuss the importance of a systematic search for associated intestinal malformations in cases of imperforate anus in order to avoid serious complications and to choose the optimal operative strategy.

摘要

肛门闭锁与直肠神经支配异常的关联非常罕见。此外,肠旋转不良仅很少伴随这些畸形。我们报告了2例这种不寻常的三联征病例及其对治疗的影响。回顾性分析显示,两名患者出生时均表现出相同的影像学表现,即远端结肠扩张和小肠位于右侧。我们讨论了在肛门闭锁病例中系统检查相关肠道畸形的重要性,以避免严重并发症并选择最佳手术策略。

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