Galifer R B, Chavrier Y, Viala J S
Chir Pediatr. 1985;26(2):95-103.
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)
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)