Functional outcome, quality of life, and 'failures' following pull-through surgery for hirschsprung's disease: A review of practice at a single-center

Abstract

Aims: The purpose of this study was to assess bowel function and quality of life (QoL) in patients with Hirschsprung's disease (HD) and identify patients who have 'failed' treatment.

Methods: A review of a single-center HD cohort treated with pull-through surgery from 2004 to 2017 was completed. Bowel function of patients five years and above and QoL of all patients were assessed using validated questionnaires. Patients who 'failed' treatment were defined as above five years with one or more of: a) long-term stoma, b) needing an antegrade continence enema or transanal irrigation, c) severe soiling, or d) severe constipation. Statistical analysis was performed with P < 0.05 deemed significant. Data are given as mean [range].

Results: Seventy-one patients presented with HD within the study period. Mean follow-up was 5.4 years [0.7-13.3]. Of 38 eligible patients, bowel function was assessed in 24 patients (nine had a stoma, five lost to follow-up). The mean incontinence score was 17 [0-28)], and the mean constipation score was 17 [5-25]. Incontinence and constipation scores were worse than healthy controls (P < 0.001 and P = 0.001, respectively) and did not improve with age. Fifty-six patients had QoL assessed with no difference between our cohort (81 [25-100]) and healthy controls (81 [unknown]); (P = 0.85). Thirty-three patients were assessed for 'failure' (bowel function score n = 24; stoma n = 9). Thirty of 33 (91%) children older than five years can be considered to have 'failed' treatment.

Conclusions: Patients have worse bowel function than healthy children, which does not improve with age. QoL is comparable to healthy controls. A significant proportion of patients have poor outcomes and have 'failed' treatment.

Level of evidence: Level III.

Keywords: Constipation; Hirschsprung's disease; Incontinence; Long-term outcome; Quality of life.