Surgical management of hirschsprung disease: results of a worldwide survey.

PURPOSE: Hirschsprung disease (HD) is a rare condition. It requires well-oriented care regardless of birthplace. We aimed to understand global HD management and identify areas of consensus and variability in perioperative care.

METHODS

The Hendren Project distributed a survey to all members of its global network of pediatric surgeons and invited those who had performed five or more HD operations at their institution to participate. Surgeons were asked to retrospectively review their five most recent operations and answer a series of questions regarding diagnostic workup, surgical intervention, and complications.

RESULTS

105 surgeons completed the survey, representing 81 hospitals in 58 cities and 42 countries, reporting on 403 distinct patients. HD diagnosis was most often made by open transanal biopsy (52.4%). Preoperative rectal irrigations, used as medical treatment for obstruction, were administered to 84.8% of patients. Primary pull-through was performed in 58.8% of patients, and staged repair with a previously created colostomy was performed in 41.2%. Postoperative complications occurred in 23.6% of patients, most commonly stricture and reoperation.

CONCLUSION

A global survey of pediatric surgeons identified variation in perioperative management of patients with HD, with 25% of patients experiencing surgical complications. Opportunities for quality improvement and educational efforts to improve outcomes and surgical disparities were detected.