| Summary: | Currarino syndrome is characterized by anorectal malformation, a presacral tumor and sacral malformation. 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 and megarectum associated with 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.
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