Contemporary practice patterns in the surgical management of Hirschsprung's disease

Many options exist in the surgical management of Hirschsprung's disease (HD). To gain insight into contemporary management, we queried pediatric surgeons listed in the American Pediatric Surgical Association Directory on their management for the typical baby with HD.

Surveys were sent electronically to the surgeons concerning a typical newborn diagnosed with HD. Questions included the preferred approach, number of stages, anastomotic technique, length of muscular rectal cuff, point of initiation of the anorectal dissection, and length of colonic resection. Surgeons performing laparoscopy were asked about how the colonic biopsy was performed. Other questions included the type of leveling colostomy, level of residents, and criteria for performing a primary transanal pull-through. The maximum margin of error was calculated using a 95% confidence interval based on the response percentages for discrete variables.

Surveys were sent to 719 surgeons with 270 responses. A minimally invasive approach is currently used by 80%, of which 42.3% favor laparoscopy and 37.7% prefer transanal dissection only. Only 5.4% of respondents prefer the Duhamel technique. A 1-stage approach is used by 85.6%. An average muscular cuff length of 2.4 cm (range, 0.5-6 cm) is reported. A divided muscular cuff is reported by 55%. On average, the anal anastomosis is 0.73 cm (range, 0-4.5 cm) above the top of the anal columns and 3.0 cm (0-12.5 cm) above the biopsy site on the ganglionic colon. Of the respondents using laparoscopy, 80.2% report using an intracorporeal colonic biopsy technique. Participation in a training program, either fellows and/or residents, is reported by 84.8% of respondents. The most common reason given for not performing a primary transanal pull-through is long segment disease (45.6%). Margin of error was no greater than 6% for any of the responses.

A minimally invasive approach with a 1-stage operation has become the most common strategy for the surgical management of the typical baby with HD. Opinions vary about the amount of colonic resection, length of the rectal cuff, and site of initiation of the anorectal dissection, and these represent potential points for future studies.