Management and operative strategy for Currarino syndrome associated with thickening of the internal anal sphincter, megarectum and presacral tumor: A case report and literature reviews

Currarino syndrome is characterized by anorectal malformation, a presacral tumor and sacralmalformation. A funnel-shaped anal stenosis causes chronic constipation resulting in the development of megarectum and requires surgical intervention. We present a three-year-old girl with Currarino syndrome consisting of a presacral tumor, anal stenosis andmegarectum associatedwith the thickening of the internal anal sphincter. After transverse loop colostomy, excision of the presacral tumor was performed via a posterior sagittal approach and posterior anoplasty with sphincterotomy was done for the anal stenosis. After discharge, anastomotic stenosis in the anal ring remained and anal dilatation, along with closure of colostomy, was performed with an extended skin graft method. Five months postoperatively, there had not been any recurrence of her tumor and she felt the desire to defecate and smoothly defecated with a laxative. 2016 The Author. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). In Currarino syndrome, the typical patient has a combination of sacral, anorectal, intraspinal and presacral anomalies. In most cases of Currarino syndrome, presentation occurs in infancy or childhood. Symptoms such as intractable constipation and bowel obstruction in infancy are frequently associated with this condition [1]. In its classic form, the anorectal anomaly consists of a very characteristic anal stenosis which is funnel-shaped up to the dentate line, resulting in the development ofmegarectum. The presacral anomaly is described as amass typically consisting of a teratoma, ameningocele, an enteric cyst or any combination of these [2]. We present a three-year-old girl with Currarino syndrome consisting of a presacral tumor, anal stenosis andmegarectum associated with the thickening of the internal anal sphincter, and describe themanagement and operative strategy.