Objective To describe the clinical features of children with severe constipation and their outcome after restorative proctocolectomy.

Design Observational study and health status questionnaire using gastrointestinal quality of life score (GIQL).

Setting English regional paediatric surgery service.

Patients Five children were identified, with severe constipation, whose symptoms had not improved with either prolonged medical therapy or colonic lavage using an antegrade colonic enema procedure. All had required a stoma to resolve their constipation.

Intervention All underwent restorative proctocolectomy.

Results All children are stooling through their anus. The mean stool frequency is 6/day. None have daytime incontinence, and none require any further therapy for constipation. Complication rates have been low with no permanent morbidity. The mean GIQL 3 years following restorative proctocolectomy was 89 (SD 29).

Conclusions In highly selected cases, restorative proctocolectomy may allow resolution of the symptoms of severe constipation and avoid leaving a child with a permanent stoma.