Long-term outcomes and quality of life of patients with Hirschsprung disease: a systematic review and meta-analysis.

BACKGROUND

Advances in surgical techniques and perioperative care have improved the short- and mid-term postoperative outcomes of patients with Hirschsprung disease (HD). However, the long-term outcomes of these patients (older than 10 years) have not been fully investigated. The aim of this systematic review is to clarify the prevalence of long-term outcomes and the quality of life of these patients.

METHODS

PubMed, AMED, Cochrane Library, CINAHL and PsycINFO databases were searched from inception to October 2018, following the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guideline. Original studies reporting the outcomes of patients older than ten years with HD were selected and reviewed. The overall prevalence of fecal incontinence, constipation, bowel function score, bladder dysfunction symptoms, and patients' quality of life were extracted from the included studies and pooled through the random-effects meta-analysis model. The heterogeneity and variation in the pooled estimations were evaluated by Cochrane's Q test and the I test. The sensitivity analysis was conducted by the sequential omission of individual studies. Publication bias was evaluated by Egger's linear regression test. The whole procedure was conducted with Stata (version 14).

RESULTS

In total, 3406 articles were identified from the literature search, among which twelve studies, including 625 patients, were included for analysis. The pooled prevalences of fecal incontinence, constipation, and bladder dysfunction symptoms and good to excellent bowel function scores were 0.20 (95% CI 0.13-0.28), 0.14 (95% CI 0.06-0.25), 0.07 (95% CI 0.04-0.12), and 0.95 (95% CI: 0.91-0.97), respectively; the pooled mean score of gastrointestinal-related quality of life was 118 (95% CI: 112.56-123.44).

CONCLUSIONS

HD patients older than ten years old have an overall high prevalence of fecal incontinence and a low quality of life. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research that focuses on the attributes and predictors of the long-term prognosis of patients with HD are necessary.