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[泄殖腔畸形的外科治疗原则。附3例报告]

[Principles of the surgical treatment of cloacal malformation. Apropos of 3 cases].

作者信息

Galifer R B, Chavrier Y, Viala J S

出版信息

Chir Pediatr. 1985;26(2):95-103.

PMID:4042264
Abstract

The persistent cloaca is one of the most complex and challenging developmental malformation. It is a rare anomaly occuring only in the female newborn and represented by the association of a urogenital sinus with an anorectal malformation. We report here three cases seen and recently treated in Montpellier (France) in the unit of visceral pediatric surgery (Pr J.G. Pous) between 1981 and 1983. A precise knowledge of normal embryology is very helpful in order to understand these complex anatomical situations. Every case is probably unique, but some main principles of management can be stressed. Neonatal loop colostomy is probably safer in most cases and can allow temporizing until the child is 6 months to 1 year of age. Interim management is often needed: intermittent catheterisation of the bladder and/or urine-filled vagina via the uro-genital sinus, avoiding urinary diversions, except possible temporary supra-pubic cystostomy. Definitive reconstruction is often a long and complex procedure, strictly dependent on absolutely thorough radio-endoscopic pre-operative investigations. It is vital to know exactly the length of the cloacal canal and of the urethra, because surgical reconstruction will vary according to the level at which the various structures converge. Cloacas represent a wide anatomic spectrum: as the severity of the defect increases, there is urogenital sinus with anterior anus ("Near cloacal malformation"), low and high confluence of all three systems. The goal of surgery is to perform in a single operation, the separation of the different conduits and to preserve all the sphincteric mechanisms for urinary-anal continence and genital functions.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

泄殖腔存留是最复杂且具挑战性的发育畸形之一。它是一种仅见于女性新生儿的罕见异常,表现为泌尿生殖窦与肛门直肠畸形并存。我们在此报告1981年至1983年间在法国蒙彼利埃小儿内脏外科(Pr J.G. Pous)诊治的三例病例。准确了解正常胚胎学对于理解这些复杂的解剖情况非常有帮助。每个病例可能都是独特的,但一些主要的处理原则可以强调。在大多数情况下,新生儿袢式结肠造口术可能更安全,并且可以暂行处理直至患儿6个月至1岁。通常需要进行临时处理:通过泌尿生殖窦对膀胱和/或充满尿液的阴道进行间歇性插管,避免尿流改道,除非可能需要临时耻骨上膀胱造瘘。确定性重建往往是一个漫长而复杂的过程,严格依赖于术前绝对彻底的放射内镜检查。确切了解泄殖腔管和尿道的长度至关重要,因为手术重建将根据各结构汇合的水平而有所不同。泄殖腔代表了广泛的解剖范围:随着缺陷严重程度的增加,存在泌尿生殖窦与前位肛门(“近泄殖腔畸形”),以及所有三个系统的低位和高位汇合。手术的目标是在一次手术中分离不同管道,并保留所有用于泌尿-肛门节制和生殖功能的括约肌机制。(摘要截取自250字)

相似文献

1
[Principles of the surgical treatment of cloacal malformation. Apropos of 3 cases].[泄殖腔畸形的外科治疗原则。附3例报告]
Chir Pediatr. 1985;26(2):95-103.
2
Posterior cloaca--further experience and guidelines for the treatment of an unusual anorectal malformation.后肛——一种不常见的肛门直肠畸形的治疗经验及指南。
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[Total mobilization of the urogenital sinus in the treatment of cloaca].[全动员泌尿生殖窦在泄殖腔治疗中的应用]
An Esp Pediatr. 2001 Dec;55(6):573-5.
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"Perineal canal": a rare anorectal malformation of variable complexity.会阴管:一种复杂程度各异的罕见肛肠畸形。
Eur J Pediatr Surg. 2008 Dec;18(6):392-4. doi: 10.1055/s-2008-1038909. Epub 2008 Nov 14.
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Management of cloacal anomalies: report on three cases.泄殖腔畸形的治疗:三例报告
N Z Med J. 1989 Sep 27;102(876):503-4.
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Reoperations in anorectal malformations.肛门直肠畸形的再次手术
J Pediatr Surg. 2007 Feb;42(2):318-25. doi: 10.1016/j.jpedsurg.2006.10.034.
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Further experience in reconstructive surgery for cloacal anomalies.
J Pediatr Surg. 1982 Dec;17(6):695-717. doi: 10.1016/s0022-3468(82)80434-x.
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[Exstrophy of the cloaca: report of two cases. Review of embryology and management (author's transl)].
An Esp Pediatr. 1980 Oct;13(10):881-8.
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Surgical reconstruction of cloacal malformation can alter bladder function: a comparative study with anorectal anomalies.泄殖腔畸形的手术重建可改变膀胱功能:与肛门直肠畸形的比较研究。
J Urol. 2004 Dec;172(6 Pt 1):2377-81; discussion 2381. doi: 10.1097/01.ju.0000145201.94571.67.

引用本文的文献

1
Congenital rectovaginal fistula with anorectal agenesis: A rare anorectal malformation.先天性直肠阴道瘘合并肛门直肠闭锁:一种罕见的肛门直肠畸形。
Int J Pediatr Adolesc Med. 2017 Dec;4(4):138-140. doi: 10.1016/j.ijpam.2017.08.003. Epub 2017 Dec 16.
2
Urogenital sinus, rectovaginal fistula, and an anterior stenosed anus--another cloacal variant.泌尿生殖窦、直肠阴道瘘和肛门前方狭窄——另一种泄殖腔变异。
Pediatr Surg Int. 2004 Jul;20(7):556-8. doi: 10.1007/s00383-004-1216-5. Epub 2004 Jul 3.