Gupta Rahul, Radhakrishnan Jayant
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India.
Emeritus Professor of Surgery and Urology, University of Illinois, Chicago, Illinois, USA.
J Indian Assoc Pediatr Surg. 2024 Jul-Aug;29(4):319-328. doi: 10.4103/jiaps.jiaps_30_24. Epub 2024 Jul 6.
Cloacal malformations are rare and are typically reported in females. There are a few scattered reports in males. It is not clear why they are so rare in males since both sexes negotiate this stage of embryonal development.
The present study aims to share our experience and review all the cases of persistent cloaca and cloacal variants in males reported in the literature.
The male cloaca is defined as a single common channel of varying lengths with separate inlets for the urinary tract (urethra) anteriorly and the rectum posteriorly at its cranial end and with a solitary perineal orifice/opening for external drainage. We also carried out an electronic literature search for cloaca, persistent cloaca, common cloaca, cloacal dysgenesis, cloacal malformation, cloacal membrane agenesis, urorectal malformation sequence, rectourinary perineal fistula, sirenomelia, and caudal regression syndrome.
After eliminating other cloacal anomalies and persistent cloaca in females, we found a total of 22 males with persistent cloaca or cloacal variant reported in the literature. In addition, we are adding two cases we have managed since our previous report.
An effort should be made to search for the presence of the common channel in male patients with a single perineal opening. Recognition of the anomaly, width of the common cloacal channel, location of the rectal pouch with relation to the sacrum or pubis, status of the spine and sacrum, and nature of the anal sphincter are vital pieces of information to successfully manage the anomaly. It would be worthwhile if future reports on the subject also include long-term information about urinary and fecal functions and continence.
泄殖腔畸形较为罕见，通常在女性中报道。男性中仅有少数散在病例报告。目前尚不清楚为何男性病例如此罕见，因为两性都会经历这一胚胎发育阶段。
本研究旨在分享我们的经验，并回顾文献中报道的所有男性持续性泄殖腔及泄殖腔变异病例。
男性泄殖腔定义为一个长度不等的单一共同通道，在其头端前方有尿道（泌尿道）的独立入口，后方有直肠入口，且有一个单独的会阴孔用于外部引流。我们还通过电子文献检索了泄殖腔、持续性泄殖腔、共同泄殖腔、泄殖腔发育不全、泄殖腔畸形、泄殖腔膜发育不全、泌尿直肠畸形序列、直肠泌尿会阴瘘、并腿畸形和尾椎退化综合征等内容。
在排除女性的其他泄殖腔异常和持续性泄殖腔后，我们在文献中发现了总共22例男性持续性泄殖腔或泄殖腔变异病例。此外，自我们上次报告以来，我们又增加了两例我们所诊治的病例。
对于有单个会阴开口的男性患者，应努力寻找是否存在共同通道。识别该畸形、共同泄殖腔通道的宽度、直肠盲袋相对于骶骨或耻骨的位置、脊柱和骶骨的状况以及肛门括约肌的性质，是成功处理该畸形的关键信息。如果未来关于该主题的报告也能包含有关泌尿和排便功能及控便能力的长期信息，将是很有价值的。
