A national analysis of operative treatment of adult patients with Hirschsprung's disease

Purpose Hirschsprung's disease is primarily a disease of infancy, but in rare cases, adults with this condition require surgery. The aim of this study is to identify the types of operations and postoperative outcomes in adults with Hirschsprung's disease on a national level. Methods The National Surgical Quality Improvement Program database was used to perform a retrospective review of all adult patients diagnosed with Hirschsprung's disease. Patients were divided into two groups depending on the type of operation: restoration of bowel continuity or diversion of fecal stream; clinicopathologic data and 30-day outcomes were compared between the two groups. Results A total of 32 patients were analyzed. Fourteen patients (43.8%) underwent diversion and 18 (56.2%) underwent restorative procedures. The median age was 49.5 years old for the diversion group and 23.5 years old for the reconstructive group (p = 0.001). The restorative surgery group was more likely to have an ASA 1-2 while the diversion group had a higher frequency of ASA 3-5 (p = 0.011). The median length of stay for the diversion surgery was 9.5 days and 5 days for the restoration group (p = 0.045). Complications occurred in 57% of patients in the diversion group and in 22% of patients in the restoration group (p = 0.049). There were otherwise no statistically significant differences in intraoperative data and postoperative complications. Conclusion This is the first study using a national database to evaluate the surgical treatment of Hirschsprung's disease in adult patients. Complications are common and were more frequent in the older, sicker diversion group, with restoration of continuity being better tolerated in the younger, healthier patient population.