Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Meta-Analysis
. 2020 Mar 12;20(1):67.
doi: 10.1186/s12876-020-01208-z.

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

Ying Dai  1 , Yongfang Deng  2 , Yan Lin  3 , Runxian Ouyang  1 , Le Li  4
Affiliations
Meta-Analysis

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

Ying Dai et al. BMC Gastroenterol. .

Abstract

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 I2 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.

Keywords: Children; Constipation; Fecal incontinence; Follow-up; Hirschsprung disease; Long-term outcome; Prevalence; Quality of life.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
PRISMA flowchart of the screening process
Fig. 2
Fig. 2
a. Pooled prevalence of fecal incontinence. b. Pooled prevalence of constipation. c. Pooled mean score of Bowel Function Score (BFS score). d. Pooled prevalence of patients with excellent to good Holschneider Score. e. Pooled prevalence of patients with bladder dysfunction symptom. f. Pooled mean score of gastrointestinal quality of life index score (GIQLI score)
Fig. 3
Fig. 3
a. Sensitivity analysis of combined relative frequency of patients with fecal incontinence. b. Sensitivity analysis of combined relative frequency of patients with constipation. c. Sensitivity analysis of combined relative frequency of patients with bladder dysfunction symptom
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Deng X, Wu Y, Zeng L, Zhang J, Zhou J, Qiu R. Comparative analysis of modified laparoscopic Swenson and laparoscopic soave procedure for short-segment Hirschsprung disease in children. Eur J Pediatr Surg. 2015;25(5):430–434. - PubMed
    1. Xia X, Li N, Wei J, Zhang W, Yu D, Zhu T, Feng J. Laparoscopy-assisted versus transabdominal reoperation in Hirschprung's disease for residual aganglionosis and transition zone pathology after transanal pull-through. J Pediatr Surg. 2016;51(4):577–581. doi: 10.1016/j.jpedsurg.2015.10.085. - DOI - PubMed
    1. Ademuyiwa AO, Bode CO, Idiodi-Thomas HO, Elebute OA. Early outcome of open primary pull through versus staged pull through in Hirschsprung's disease: a single Centre experience from Nigeria. Nig Q J Hosp Med. 2012;22(3):164–167. - PubMed
    1. Gunnarsdottir A, Larsson LT, Arnbjornsson E. Transanal endorectal vs. Duhamel pull-through for Hirschsprung's disease. Eur J Pediatr Surg. 2010;20(4):242–246. doi: 10.1055/s-0030-1252006. - DOI - PubMed
    1. Lukac M, Antunovic SS, Vujovic D, Petronic I, Nikolic D, Radlovic V, Krstajic T, Krstic Z. Effectiveness of various surgical methods in treatment of Hirschsprung's disease in children. Vojnosanit Pregl. 2016;73(3):246–250. doi: 10.2298/VSP140516002L. - DOI - PubMed

LinkOut - more resources