Abstract

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.

Highlights

  • Perineal fistula is one of the most common types of anorectal malformation in pediatric surgery

  • This special type of malformation (SPF) is characterized by a perineal fistula as the principal clinical manifestation but the fistula tube is slender and the proximal bowel is found at a high position

  • We performed a preoperative distal colostogram, which revealed that the fistula tube was slender, and the dilated proximal colon was located in front of the iliac crest (Fig. 1)

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Summary

Introduction

Perineal fistula is one of the most common types of anorectal malformation in pediatric surgery. Most pediatric surgeons agree that this well recognized malformation can be repaired by perineal ­anoplasty[1,2,3]. We experienced a rare case of a perineal fistula associated with dilatation of the bowel found at a high position. This special type of malformation (SPF) is characterized by a perineal fistula as the principal clinical manifestation but the fistula tube is slender and the proximal bowel is found at a high position. The aim of this article is to explore the diagnosis and treatment of this special type of perineal fistula, and to improve its treatment outcomes

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