Kapoor Rohit, Gupta Amit, Yadav Partap Singh, Mandelia Ankur
Department of Pediatric Surgery, Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India.
Department of Pediatric Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
J Indian Assoc Pediatr Surg. 2022 Nov-Dec;27(6):707-712. doi: 10.4103/jiaps.jiaps_39_22. Epub 2022 Nov 14.
Posterior cloacal malformations (PCMs) are distinguished from classical cloacal malformations by the posterior location of the common opening in the perineum. We aim to describe our experience of management of these rare and complex malformations.
This study was a retrospective chart review of all patients with PCM who underwent treatment at Kalawati Saran Children's Hospital (KSCH), New Delhi, and Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, between 2013 and 2021. Individual anatomical variations and their impact on the clinical presentation, management, and final outcome (cosmesis and function) were recorded and analyzed.
During the study period, four girls with a median age of 2 (range: 0-5) years presented to us with PCM. Three patients were referred to KSCH as ambiguous genitalia whereas one patient was born at SGPGIMS with an antenatal diagnosis of "urorectal septal defect." On examination, three patients had the urogenital sinus (UGS) opening immediately anterior to the normally located anal opening (2 orifices), whereas one had a single orifice at the normal location of the anus with the UGS opening in the anterior rectal wall. Associated anomalies included: (a) hydrocolpos ( = 3), which was managed by a tube vaginostomy; (b) urethral duplication with dorsal atretic urethra ( = 3); (c) uterine didelphys ( = 1); (d) bilateral grade 5 vesicoureteric reflux ( = 1); and (e) vaginal calculus ( = 1). Total urogenital mobilization (anterior sagittal approach) with feminizing genitoplasty was performed for patients with UGS and normal anus ( = 3). In the patient with a single opening at the normal location of the anus, posterior sagittal ano-recto-urethro-vaginoplasty was performed. At a median follow-up of 24 months, three patients are continent for urine and stool, whereas one patient is yet to be toilet trained.
PCMs are unusual complex malformations that necessitate meticulous clinical examination, detailed diagnostic workup, and multistaged surgical management. Management should be tailored as per each individual patient's anatomy and clinical presentation for an optimal outcome.
后泄殖腔畸形（PCM）与经典泄殖腔畸形的区别在于会阴区共同开口的位置靠后。我们旨在描述我们对这些罕见且复杂畸形的治疗经验。
本研究是一项回顾性病历审查，研究对象为2013年至2021年间在新德里的卡拉瓦蒂·萨兰儿童医院（KSCH）和勒克瑙的桑贾伊·甘地医学科学研究生学院（SGPGIMS）接受治疗的所有PCM患者。记录并分析个体解剖变异及其对临床表现、治疗和最终结局（美容效果和功能）的影响。
在研究期间，4名中位年龄为2岁（范围：0 - 5岁）的女孩因PCM前来就诊。3名患者因生殖器模糊被转诊至KSCH，而1名患者在SGPGIMS出生，产前诊断为“尿直肠隔缺损”。检查发现，3名患者的泌尿生殖窦（UGS）开口紧邻正常位置的肛门开口前方（2个开口），而1名患者在肛门正常位置有一个单一开口，UGS开口于直肠前壁。相关异常包括：（a）阴道积水（n = 3），通过阴道造瘘管治疗；（b）尿道重复畸形伴背侧闭锁尿道（n = 3）；（c）双子宫（n = 1）；（d）双侧5级膀胱输尿管反流（n = 1）；以及（e）阴道结石（n = 1）。对于UGS且肛门正常的患者（n = 3），采用完全泌尿生殖系统游离术（前矢状入路）并进行女性化生殖器成形术。对于肛门正常位置有单一开口的患者，进行后矢状肛门 - 直肠 - 尿道 - 阴道成形术。中位随访24个月时，3名患者大小便自控，而1名患者尚未接受如厕训练。
PCM是不常见的复杂畸形，需要细致的临床检查、详细的诊断评估和多阶段手术治疗。应根据每个患者的解剖结构和临床表现进行个体化治疗，以获得最佳结局。
