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一名新生儿患有肛门闭锁(肛门发育不全)并伴有直肠和乙状结肠闭锁。

Imperforate anus (anal agenesis) with rectal and sigmoid atresias in a newborn.

作者信息

Ein S H

机构信息

Division of General Surgery, Hospital for Sick Children, Toronto, Ontario, Canada, M5G 1X8.

出版信息

Pediatr Surg Int. 1997 Jul;12(5-6):449-51. doi: 10.1007/BF01076966.

DOI:10.1007/BF01076966
PMID:9244126
Abstract

A male baby was born to a healthy mother after an uncomplicated 39-week pregnancy. He had finger and toe syndactyly, right hemidiaphragm eventration, and a high imperforate anus (anal agenesis). The kidneys and chromosomes were normal. A right transverse colostomy was done and 5 months later he underwent a posterior sagittal anorectoplasty (Pena procedure). An intermediate imperforate anus with no urethral fistula and a rectal atresia 2 cm proximal to the distal rectal pouch were found; the distal rectal pouch was resected and the standard Pena procedure completed. Dilatations were initiated 2 weeks postoperatively and continued until the colostomy was closed. Prior to closure of the colostomy, a distal colon loopogram demonstrated an obstruction in the sigmoid; constrast introduced via the rectum outlined the same obstruction separated by 2 cm. A low sigmoid atresia was then repaired. Three weeks later, a contrast loopogram showed a narrow but intact rectosigmoid anastomois, and the colostomy was closed. The anorectal dilatations were discontinued and he remains well at 7 years of age.

摘要

一名男婴在怀孕39周且无并发症的情况下,由一位健康的母亲产下。他患有手指和脚趾并指畸形、右侧半膈膨出以及高位肛门闭锁(肛门发育不全)。肾脏和染色体正常。进行了右侧横结肠造口术,5个月后他接受了后矢状位肛门直肠成形术(佩纳手术)。发现为中度肛门闭锁,无尿道瘘,直肠闭锁位于远端直肠袋近端2厘米处;切除远端直肠袋并完成标准的佩纳手术。术后2周开始进行扩张,并持续至结肠造口关闭。在结肠造口关闭前,远端结肠灌肠造影显示乙状结肠梗阻;经直肠注入造影剂勾勒出同一梗阻,相距2厘米。随后修复低位乙状结肠闭锁。三周后,灌肠造影显示直肠乙状结肠吻合口狭窄但完整,结肠造口关闭。停止肛门直肠扩张,他7岁时情况良好。

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引用本文的文献

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Imperforate anus associated with eventration of diaphragm.肛门闭锁合并膈膨出
J Neonatal Surg. 2013 Jan 1;2(1):14. eCollection 2013 Jan-Mar.
2
Rare association of severe hypoplasia of the abdominal aorta with imperforate anus, colonic atresia, and choledochal cyst.腹主动脉严重发育不全与肛门闭锁、结肠闭锁及胆总管囊肿的罕见关联。
Pediatr Surg Int. 2006 Mar;22(3):289-92. doi: 10.1007/s00383-005-1604-5. Epub 2005 Nov 26.

本文引用的文献

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Congenital abnormalities of the anus and rectum.肛门和直肠的先天性异常。
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Agenesis of the descending colon with imperforate anus. Correlation with modern concepts of the origin of intestinal atresia.降结肠缺如合并肛门闭锁。与现代肠道闭锁起源概念的相关性。
Am J Surg. 1967 Feb;113(2):279-81. doi: 10.1016/0002-9610(67)90237-1.