Single-stage repair of Hirschsprung's disease: a comparison of 109 patients over 5 years

Current management of Hirschsprung's disease (HD) typically involves staged therapy, which necessitates multiple hospital admissions and associated costs. The authors therefore investigated the course and outcome of treating such children using a single-staged (SS) approach, and compared them with those treated via multiple-staged (MS) therapy. The cases of one hundred nine consecutive patients who presented with HD from 1991 to 1996 were reviewed. Four patients were excluded (two unrelated deaths, two with small intestinal aganglionosis). Twenty-one of the remaining 105 patients underwent SS repair. Both groups were similar in gender, age at diagnosis, and frequency of comorbidities. Repair was possible in 100% of the SS patients. Complications, including enterocolitis, occurred in 63% of patients, and did not significantly differ between groups. The outcome in SS patients was unaffected by whether the repair was performed before or after 30 days of life. The outcome was unaffected by operative weight in either group. By contrast, the number of hospital admissions and total length of stay was significantly higher in the MS group, which resulted in a twofold increase in total costs associated with MS repair compared with SS repair. These data indicate that primary repair of HD is efficacious (even in the newborn), with morbidity equal to MS repair, and requires fewer hospital admissions. The significant savings to the patient and the health care system suggest that SS repair may be an improved strategy for treating HD.