Pediatric Surgery Department, Faculty of Medicine, Ain Shams University, Egypt.
Radiodiagnosis Department, Faculty of Medicine, Ain Shams University, Egypt.
J Pediatr Urol. 2021 Jun;17(3):410.e1-410.e7. doi: 10.1016/j.jpurol.2021.01.014. Epub 2021 Jan 20.
'Persistent Cloaca' refers to one of the major groups of anorectal anomalies in the female when a single perineal orifice is located at the normal site of the urethra draining both urogenital and alimentary tracts. However, 'posterior cloaca' is a new term developed by Pena to describe a unique defect in females in which a common urogenital sinus is deviated posteriorly to open into normally located anorectum (type A) or in the perineum slightly anterior to the anus (type B).
The study included seven girls diagnosed as posterior cloaca. Their age at presentation ranged from 1 to 108 months (median 12 months). The main presentation was abnormal external genitalia, while two cases underwent colostomy (±vaginostomy) at birth. Surgical reconstruction varied according to the degree of deviation from normal anatomy. Partial urogenital sinus mobilization (PUM) was used to correct minor forms; while at the other end of the spectrum (absent urinary bladder), continent urinary diversion was performed. Due to the high incidence of renal and urological anomalies, regular follow up at paediatric nephrology/urology clinics was advised for affected cases.
All seven cases had a common urogenital confluence characteristically deviated posteriorly. The degree of backward deviation of the common urogenital orifice was variable ranging from mild to severe posterior displacement. In six cases (85%), the common urogenital orifice was shifted backwards in the perineum approaching the anal verge (type B). In one case, the common urogenital orifice opened internally into the anorectum (type A). Major urinary tract anomalies were quite common (5 of 7 cases; 71%): absent urinary bladder (2 cases); single kidney (2 cases); crossed ectopic kidney (1 case); hydroureteronephrosis (2 cases).
Posterior cloaca is a rare anomaly in the female essentially affecting the lower urogenital tract with a high incidence of associated renal anomalies. Management should be tailored according to the degree of developmental defect.
“永存性肛门直肠闭锁”是女性肛门直肠畸形的主要类型之一，表现为单一的会阴或肛门口位于正常的尿道位置，同时尿液和粪便可经同一开口排出。然而，Pena 提出了一个新的术语“后肛门直肠闭锁”来描述女性的一种独特的畸形，即共同的泌尿生殖窦向后偏离，开口于正常位置的肛门直肠（A型）或肛门稍前方的会阴（B 型）。
本研究纳入了 7 例诊断为后肛门直肠闭锁的女性患者。其就诊时的年龄为 1~108 个月（中位数 12 个月）。主要表现为外生殖器异常，其中 2 例患儿在出生时即行结肠造口术（±阴道造口术）。手术重建的方式取决于偏离正常解剖结构的程度。采用部分泌尿生殖窦游离术（PUM）来矫正轻微的畸形；而对于存在膀胱缺如的严重病例，则行可控性尿流改道术。由于肾和泌尿系统畸形的发生率较高，建议对受累病例进行儿科肾病/泌尿外科的定期随访。
7 例患者均存在共同的泌尿生殖窦向后明显偏离。共同泌尿生殖孔向后移位的程度不一，从轻度到严重的后向移位。在 6 例（85%）患者中，共同泌尿生殖孔向后移位至会阴，接近肛门边缘（B 型）。在 1 例患者中，共同泌尿生殖孔开口于内部的肛门直肠（A 型）。大多数患者存在明显的泌尿道畸形（7 例中的 5 例；71%）：膀胱缺如（2 例）；孤立肾（2 例）；交叉异位肾（1 例）；肾盂积水（2 例）。
后肛门直肠闭锁是一种罕见的女性畸形，主要影响下泌尿生殖道，且常合并肾畸形。应根据发育缺陷的程度进行个体化治疗。
