Department of Paediatric Urology, Great Ormond Street Children's Hospital, London WC1N 3JH, UK.
Arch Dis Child. 2011 Nov;96(11):1072-6. doi: 10.1136/adc.2009.175034. Epub 2011 Jan 23.
Persistent cloaco is the most severe type of anorectal malformation encountered in children. Patients with cloacal anomalies have a high incidence of associated anomalies most commonly: urinary tract and spinal. Persistent cloaca remains a difficult reconstructive challenge but it is now possible to anatomically correct the defect with surgery in the majority of patients. This review discusses embryology, prenatal diagnosis, neonatal physical and radiological findings. A summary of early management and investigation is provided and the commonest surgical reconstruction techniques are discussed. The main goals of surgical reconstruction are the achievement of bowel and bladder control for the child and normal sexual function in adult life. Although the majority of cloaca patients can achieve faecal and urinary continence with the surgical reconstructive procedures performed today, many require additional/multiple urological procedures to achieve continence, treat bladder dysfunction and to protect renal function. One half of patients will develop renal failure, so regular and life long surveillance is mandatory. Due to the high number of associated gynaecological problems all patients should be assessed by a paediatric gynaecologist at puberty.
持续性泄殖腔畸形是儿童中最严重的肛门直肠畸形类型。患有泄殖腔畸形的患者常伴有多种相关畸形，最常见的是泌尿系统和脊柱畸形。持续性泄殖腔畸形仍然是一个具有挑战性的重建难题，但现在大多数患者都可以通过手术进行解剖学矫正。这篇综述讨论了胚胎发生、产前诊断、新生儿体格检查和影像学发现。本文还总结了早期的处理和检查方法，并讨论了最常见的手术重建技术。手术重建的主要目标是使患儿实现肠道和膀胱控制，并在成年后具有正常的性功能。尽管大多数泄殖腔患者可以通过今天进行的手术重建来实现粪便和尿液的控制，但许多患者需要额外的/多次泌尿科手术来实现控制、治疗膀胱功能障碍和保护肾功能。一半的患者会出现肾衰竭，因此必须进行定期的终身监测。由于存在大量的妇科问题，所有患者都应在青春期由儿科妇科医生进行评估。
