Selection of Operative Approach in Children with Currarino Syndrome

Purpose: To summarize the experience of surgical treatment of children diagnosed with Currarino syndrome, with an emphasis on selection of optimal operative approach. Methods: : The clinical materials of patients diagnosed with Currarino syndrome were recorded. Special attention was paid to the operative management, particularly the different routes for operation. The type of ARM was the critical point. Follow-up was conducted for at least 3 months, and the Rintala score was used for evaluation of bowel function. Results: : The medical records of 26 patients were reviewed and listed in table one. Seven were male, and 19 were female, with a mean age of 19.38±13.80 months (ranging from 2 to 47 months). The anorectal malformation included 17 rectoperineal fistulae without an anus, four rectovestibular fistulae without an anus, two anal stenoses, one anal atresia, one rectourinary fistula without an anus, and one cloaca. The PS was taken in 23 cases. The SPS was taken in eight cases, and LPS was taken in another 15 patients. SPS group included three rectoperineal fistulae without an anus, one anal stenosis, one retraction of the rectum after an anoplasty for rectovestibular fistula without an anus, one anal atresia, one rectourinary fistula without an anus, and one cloaca. In the LPS group, there were 13 perineal fistulae without an anus，one displacement of the rectum and one retraction of the rectum respectively after an anoplasty for rectovestibular fistula without an anus. TA was used in one anal atresia with megacolon and presacral mass. AS was used in one rectovestibular fistula with a presacral mass. A lamina approach was chosen for one patient with an infective fistula secondary to a presacral mass in the spinal canal. The mean follow-up time was 39.48±26.84m, ranging from 6m to 145m. The Rintala score was16.74±2.93, ranging from 12 to 20. Conclusion: For a rectoperineal fistula, SPS or LPS could be used to reach a complete cutback of the posterior wall of the rectum and remove the presacral mass. For a rectovestibular fistula, the AS or LPS could be chosen. For anorectal stenosis, TA, SPS, or LPS could be used to excise the stenosed bowel segment and the presacral mass. TA is not so good for the exposure of the presacral mass, so for a larger one(＞5cm) or one connecting with the spinal canal, the LPS or SPS may be the favorite choice.