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[肛门直肠畸形]

[Anorectal malformations].

作者信息

Cretolle C, Rousseau V, Lottmann H, Irtan S, Lortat-Jacob S, Alova I, Michel J L, Aigrain Y, Podevin G, Lehur P A, Sarnacki S

机构信息

Service de Chirurgie pédiatrique, CRMR Malformations ano-rectales et pelviennes rares (MAREP), Hôpital Necker Enfants Malades, Université Paris Descartes, 149 rue de Sèvres, 75015 Paris, France.

出版信息

Arch Pediatr. 2013 Sep;20 Suppl 1:S19-27. doi: 10.1016/S0929-693X(13)71405-2.

Abstract

Anorectal malformations (ARM) are the result of an abnormal development of the terminal part of the digestive tract interesting anus and/or rectum that occur early between the sixth and tenth week of embryonic development. They carry a malformation spectrum of severity depending on the level of disruption of the anorectal canal and of the associated caudal malformations (sacrum and spine). ARM are associated in over half the cases with other malformations that can be integrated in some cases in known syndromes. If surgical treatment to restore anatomy as normal as possible is indispensable, post-operative care is essential for these patients whose defecation mechanisms are altered, to reach if not continence, at least a socially acceptable cleanliness.

摘要

肛门直肠畸形(ARM)是消化道末端涉及肛门和/或直肠的异常发育的结果,这种异常发育发生在胚胎发育的第六至第十周之间的早期阶段。根据肛管中断的程度以及相关的尾部畸形(骶骨和脊柱),它们具有不同严重程度的畸形谱。超过半数的ARM病例与其他畸形相关,在某些情况下,这些畸形可纳入已知的综合征中。如果进行手术治疗以尽可能恢复正常解剖结构是必不可少的,那么术后护理对于这些排便机制改变的患者至关重要,以使其即使不能达到控便,至少达到社会可接受的清洁程度。

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