Medium-long term bowel function and quality of life in complete Currarino syndrome

To evaluate medium-long term bowel function, health-related quality of life (HRQoL), and related prognostic factors in patients with complete Currarino syndrome (CS).

A retrospective cohort study of patients with complete CS treated at Beijing Children's Hospital between January 2008 and December 2023 was conducted. Bowel function and HRQoL were assessed cross-sectionally in December 2025 using the Rintala Bowel Function Score and the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0), respectively. Multivariable linear regression analysis was performed to identify independent predictors of bowel function.

Thirty-two patients with a median follow-up age of 9.0 (6.3, 9.8) years were included. Total Rintala score of these patients was 16.5 (12.0, 19.0), including 78.1% (25/32) with soiling and 50.0% (16/32) with constipation, and total PedsQL 4.0 score was 96.8 (92.7, 98.9). Multivariable analysis identified total sacral dysplasia (β = -3.86, p = 0.026) and perioperative complications related to presacral mass (β = -6.24, p = 0.002) as independent predictors of poorer bowel function. Surgical resection for megarectosigmoid did not improve overall bowel function and was associated with a higher rate of soiling compared with conservative management (100.0% vs. 44.4%, p = 0.034).

Patients with complete CS had significant medium-long term bowel dysfunction, primarily determined by sacral dysplasia and perioperative complications related to presacral mass. In complete CS patients with megarectosigmoid, surgery did not improve overall bowel function.