Department of Pediatric Surgery, Xinhua hospital affiliated to Shanghai Jiao Tong University School of Medicine, No. 1665, Kongjiang Road, 200092, Shanghai, China.
Department of Pediatric Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, No. 355, Luding Road, 200000, Shanghai, China.
Sci Rep. 2021 Jan 18;11(1):1706. doi: 10.1038/s41598-021-81056-3.
The anorectal malformation with long perineal fistula is a rare anomaly in the spectrum of anorectal malformations. Aim of the study is to describe the series of patients with anorectal malformation with long perineal fistula and compare the outcome with patient with standard perineal fistula. From March 2012 to January 2019, 7 patients who suffered from anorectal malformation with long perineal fistula were retrospectively reviewed. Three were operated on primarily by our department, and 4 cases were re-operated after a perineal anoplasty repair performed elsewhere. Four were operated by laparoscopy assisted anorectoplasty, and 3 cases were repaired by posterior sagittal anorectoplasty. The follow-up outcomes were compared with 71 cases of normal perineal fistula (NPF) in the same period. 7 cases have been followed up for 0.5-4 years (M = 2.57 ± 1.26) after definitive surgery. Their bowel function score was lower than normal perineal fistula (SPF = 12, range: 5-18; NPF = 18.5, range: 18-20). Four cases underwent anorectomanometry. The incidence of rectoanal inhibitory reflex was lower in the special type group. (p = 0.14). Three cases of contrast enema using barium: 2 cases of colorectal dilatation and thickening changes, 1 case showed no obvious abnormalities. Anorectal perineal fistula should be examined by distal colostogram at preoperation. This should be altered in: When suspecting a case of anorectal malformation type long perineal fistula a preoperative contrast enema could give insight of the anatomy befor performing a anoplasty.
直肠肛门畸形伴会阴长瘘是肛门直肠畸形谱中的一种罕见异常。本研究旨在描述一组患有会阴长瘘的肛门直肠畸形患者，并将其结果与标准会阴瘘患者进行比较。2012 年 3 月至 2019 年 1 月，回顾性分析 7 例患有会阴长瘘的肛门直肠畸形患者。3 例由我科首次手术治疗，4 例在其他地方行会阴肛门成形术修复后再次手术。4 例采用腹腔镜辅助肛门直肠成形术，3 例采用后路矢状肛门直肠成形术。将随访结果与同期 71 例正常会阴瘘（NPF）进行比较。7 例患者在确定性手术后随访 0.5-4 年（M=2.57±1.26）。他们的肠功能评分低于正常会阴瘘（SPF=12，范围：5-18；NPF=18.5，范围：18-20）。4 例行肛门直肠测压。特殊型组直肠肛门抑制反射的发生率较低（p=0.14）。3 例行钡剂对比灌肠：2 例大肠扩张增厚改变，1 例未见明显异常。术前应通过远端结肠造口术检查肛门会阴瘘。当怀疑存在直肠肛门畸形伴会阴长瘘时，应进行术前对比灌肠，以便在进行肛门成形术前了解解剖结构。
