Samuk Inbal, Bischoff Andrea, Hall Jennifer, Levitt Marc, Peña Alberto
Department of Pediatric and Adolescent Surgery, Schneider Children's Medical center, affiliated to Sackler faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel; Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA.
Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA.
J Pediatr Surg. 2016 Oct;51(10):1592-6. doi: 10.1016/j.jpedsurg.2016.06.001. Epub 2016 Jun 14.
Rectobladder neck fistula is the highest and most complex anorectal malformation in boys and the only one that requires an abdominal approach, open or laparoscopic, for repair. The aim of this study was to describe the unique characteristics of rectobladder neck fistulas that warrant special attention and to describe the associated anatomic variants in the genitourinary tract.
The database of a tertiary medical center was retrospectively reviewed for all patients treated for rectobladder neck fistula, by our team in 1980-2011. Data on surgical history, associated and functional defects, treatment and outcome were collected by chart review.
The study group included 111 patients. The most common anatomic urologic defect was a single kidney in 37 patients (33.3%) and the most common functional urologic defect was vesicoureteral reflux in 40 patients (36%), including 11/37 patients with a single kidney (29.7%). Of the 40 patients who underwent cystoscopy, 16 (40%) had a higher than normal location of the verumontanum. Follow-up ranged from 2 to 290months (median 59). Urinary continence was achieved in 40 of the 61 patients (65.5%) for whom data were available, and fecal continence was achieved in 9 of the 69 patients (13%) for whom data were available. A sacral ratio of 0.4 or less was associated with lower rates of urinary control (23%) and fecal control (0%), relative to higher ratios. Twenty stomas (18%) were found to be located too distally, limiting the availability of the bowel for a pull through.
Rectobladder neck fistula carries a poor prognosis for bowel control and is associated with a high rate of urinary malformations that require long-term care. Pediatric surgeons need to be aware of these complications in order to provide proper treatment and parental counseling. Intra-vesical verumontanum is found in a surprisingly high percentage of patients. The combination of a single kidney with vesicoureteral reflux is common and should be closely followed to avoid renal deterioration. Special attention should be given to colostomy construction to avoid complications and unnecessary procedures. A sacral ratio of 0.4 or less is an indicator of poor fecal and urinary control.
直肠膀胱颈瘘是男孩中最高位且最复杂的肛门直肠畸形，也是唯一需要采用腹部入路（开放或腹腔镜）进行修复的畸形。本研究的目的是描述直肠膀胱颈瘘值得特别关注的独特特征，并描述泌尿生殖道相关的解剖变异。
回顾性分析1980年至2011年期间我们团队治疗的所有直肠膀胱颈瘘患者的三级医疗中心数据库。通过病历审查收集手术史、相关及功能缺陷、治疗及结果的数据。
研究组包括111例患者。最常见的泌尿系统解剖缺陷是37例（33.3%）患者为单肾，最常见的泌尿系统功能缺陷是40例（36%）患者存在膀胱输尿管反流，其中37例单肾患者中有11例（29.7%）存在膀胱输尿管反流。在40例行膀胱镜检查的患者中，16例（40%）精阜位置高于正常。随访时间为2至290个月（中位时间59个月）。61例有数据的患者中，40例（65.5%）实现了尿失禁控制，69例有数据的患者中，9例（13%）实现了大便失禁控制。相对于较高的骶骨比例，骶骨比例为0.4或更低与尿失禁控制率（23%）和大便失禁控制率（0%）较低相关。发现20个造口（18%）位置过于靠下，限制了用于拖出术的肠管的可用性。
直肠膀胱颈瘘在肠道控制方面预后较差，且与需要长期护理的泌尿系统畸形的高发生率相关。小儿外科医生需要了解这些并发症，以便提供适当的治疗和家长咨询。在相当高比例的患者中发现膀胱内精阜。单肾合并膀胱输尿管反流很常见，应密切随访以避免肾功能恶化。应特别注意结肠造口的构建，以避免并发症和不必要的手术。骶骨比例为0.4或更低是大便和尿失禁控制不佳的指标。
