P1.081: Pediatric Surgical Care of Hirschsprung Disease: A Global Survey

Background: Hirschsprung disease is a well-known congenital colorectal anomaly treated by pediatric surgeons across the globe, requiring partnership with an experienced pathologist. Patients born with this disease deserve high-quality and equitable care regardless of where in the world they are born. This would include pre-operative rectal irrigations to avoid enterocolitis, appropriate diagnostic workup and surgical planning, and acceptable complication rates. Our purpose is to study the management of Hirschsprung disease by pediatric surgeons around the world, in an effort to determine both the consensus and variability regarding perioperative care.

Methods: A survey was sent out through The Hendren Project, a global networked community of pediatric surgeons and urologists. Surgeons could participate if at least five primary operations for Hirschsprung disease were performed at their institution. Each surgeon who participated was required to retrospectively review operative reports in order to complete the surveys and obtain any necessary approvals from their appropriate ethical review boards.

Results: For this survey, 105 surgeons responded from 67 hospitals, in 58 different cities and 42 different countries, reporting on a total of 345 patients. In this series, Hirschsprung disease was diagnosed utilizing open trans-anal rectal biopsy (53%), more frequently than suction rectal biopsy (23%). Contrast enema was utilized in 60% of patients, and manometry was only utilized in 3% of patients. In 59% of cases, the pull-through was performed without a colostomy in place. Pre-operative rectal irrigations were performed in 86% of patients. Complications were reported in 25% of cases, with dehiscence (14%) and need for re-operation (8%) as the most frequent. Mortality was reported in 3% of patients. Select preliminary results are shown in Table 1 below.

Conclusion:

Conclusions: Our findings revealed a high complication rate for this patient population worldwide, affecting 1 out of 4 patients surgically treated. This data will help guide educational efforts and quality improvement initiatives to decrease surgical care disparity and improve outcomes for patients with Hirschsprung disease.