Vilanova-Sanchez Alejandra, Reck Carlos A, McCracken Kate A, Lane Victoria A, Gasior Alessandra C, Wood Richard J, Levitt Marc A, Hewitt Geri D
Pediatric Surgery, Center for Colorectal and Pelvic reconstruction, Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH 43205, USA.
Pediatric Surgery, Center for Colorectal and Pelvic reconstruction, Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH 43205, USA.
J Pediatr Surg. 2018 Apr;53(4):698-703. doi: 10.1016/j.jpedsurg.2017.07.012. Epub 2017 Jul 24.
BACKGROUND/AIM: Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities. We outline our collaborative evaluation process and findings as well as subsequent repair and outcomes.
MATERIAL/METHODS: A single site retrospective chart review from May 2014 to May 2016 was performed. Female patients with a history of prior ARM repair who required subsequent reoperative surgical repair with perineoplasty were included. The decision for reoperation was made collaboratively after a multidisciplinary evaluation by colorectal surgery, urology, and gynecology which included examination under anesthesia (EUA) with cystoscopy, vaginoscopy, rectal examination, and electrical stimulation of anal sphincters. The type of original malformation, indication for reoperative perineoplasty, findings leading to additional procedures performed at time of perineoplasty, postoperative complications, and the length of follow up were recorded.
During the study period 28 patients were referred for evaluation after primary ARM repair elsewhere and 15 patients (60%) met inclusion criteria. Thirteen patients (86.6%) originally had a rectovestibular fistula with prior PSARP and 2 patients (13.4%) originally had a cloacal malformation with prior posterior sagittal anorectovaginourethroplasty. The mean age at the time of the subsequent perineoplasty was 4.6years (0.5-12). Patients had an inadequate perineal body requiring reoperative perineoplasty due to: anterior mislocation of the anus (n=11, 73.3%), prior perineal wound dehiscence with perineal body breakdown (n=2, 13.4%), acquired rectovaginal fistula (n=1, 6.6%), and posterior mislocated introitus with invasion of the perineal body (n=1, 6.6%). During the preoperative evaluation, additional gynecologic abnormalities were identified that required concomitant surgical intervention including: introital stenosis (n=4, 26.6%), retained vaginal septum (n=3, 20%) and remnant recto vestibular fistula (n=2, 13.3%).
Patients with a previously repaired ARM may present with gynecologic concerns that require subsequent surgical intervention. The most common finding was an inadequate perineal body, but other findings included introital stenosis, retained vaginal septum and remnant recto vestibular fistula. Multidisciplinary evaluation to assess and identify abnormalities and coordinate timing and surgical approach is crucial to assure optimal patient outcomes.
Case series with no comparison group.
IV.
背景/目的：患者在先前接受后矢状位肛门直肠成形术（PSARP）修复肛门直肠畸形（ARM）后可能出现妇科问题。常见表现包括会阴体不足或缩短，以及阴道口狭窄、阴道纵隔残留和直肠前庭瘘残留。会阴体不足或缩短可能影响大便失禁、性功能以及关于产科分娩方式的建议。我们描述了转诊至我们中心评估其先前修复的ARM的女性患者的情况，特别关注会阴体解剖结构和伴随的妇科异常。我们概述了我们的协作评估过程、结果以及后续修复和结局。
材料/方法：对2014年5月至2016年5月进行了单中心回顾性病历审查。纳入有先前ARM修复史且需要随后进行会阴成形术再次手术修复的女性患者。在结直肠外科、泌尿外科和妇科进行多学科评估后共同做出再次手术的决定，评估包括麻醉下检查（EUA），同时进行膀胱镜检查、阴道镜检查、直肠检查和肛门括约肌电刺激。记录原始畸形类型、再次会阴成形术的指征、会阴成形术时导致额外手术的发现、术后并发症以及随访时间。
在研究期间，28例患者在其他地方接受原发性ARM修复后转诊进行评估，15例患者（60%）符合纳入标准。13例患者（86.6%）最初患有直肠前庭瘘并接受过PSARP，2例患者（13.4%）最初患有泄殖腔畸形并接受过后矢状位肛门直肠阴道尿道成形术。随后会阴成形术时的平均年龄为4.6岁（0.5 - 12岁）。患者因以下原因会阴体不足需要再次会阴成形术：肛门前移位（n = 11，73.3%）、先前会阴伤口裂开与会阴体破损（n = 2，13.4%）、后天性直肠阴道瘘（n = 1，6.6%）以及阴道口后移位侵犯会阴体（n = 1，6.6%）。在术前评估中，发现了需要同时进行手术干预的其他妇科异常，包括：阴道口狭窄（n = 4，26.6%）、阴道纵隔残留（n = 3，20%）和直肠前庭瘘残留（n = 2，13.3%）。
先前修复过ARM的患者可能出现需要后续手术干预的妇科问题。最常见的发现是会阴体不足，但其他发现包括阴道口狭窄、阴道纵隔残留和直肠前庭瘘残留。多学科评估以评估和识别异常并协调时间和手术方法对于确保最佳患者结局至关重要。
无对照组的病例系列。
IV级。
