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Multicenter Study
. 2017 Nov;33(11):1177-1181.
doi: 10.1007/s00383-017-4150-z. Epub 2017 Sep 7.

Mortality in Swedish patients with Hirschsprung disease

Anna Löf Granström  1   2 , Tomas Wester  3   4
Affiliations
Multicenter Study

Mortality in Swedish patients with Hirschsprung disease

Anna Löf Granström et al. Pediatr Surg Int. 2017 Nov.

Abstract

Purpose: Hirschsprung disease (HSCR) has previously been associated with increased mortality. The aim of this study was to assess mortality in patients with Hirschsprung disease in a population-based cohort.

Methods: This was a nationwide, population-based cohort study. The study exposure was HSCR and the study outcome was death. The cohort included all individuals with HSCR registered in the Swedish National Patient Register between 1964 and 2013 and ten age- and sex-matched controls per patient, randomly selected from the Population Register. Mortality and cause of death were assessed using the Swedish National Causes of Death Register.

Results: The cohort comprised 739 individuals with HSCR (565 male) and 7390 controls (5650 male). Median age of the cohort was 19 years (range 2-49). Twenty-two (3.0%) individuals with HSCR had died at median age 2.5 years (range 0-35) compared to 49 (0.7%) controls at median age 20 years (0-44), p < 0.001. Hazard ratio for death in HSCR patients compared to healthy controls was 4.77 (confidence interval (CI) 95% 2.87-7.91), and when adjusted for Down syndrome, the hazard ratio was 3.6 (CI 95% 2.04-6.37).

Conclusions: The mortality rate in the HSCR cohort was 3%, which was higher than in controls also when data were adjusted for Down syndrome.

Keywords: Epidemiology; Hirschsprung disease; Mortality.

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Conflict of interest statement

Funding source

This study was supported by the Foundation Frimurare Barnhuset, Her Royal Highness Crown Princess Lovisa Foundation, and the Sällskapet Barnavård Foundation.

Conflict of interest

The authors have indicated that they have no financial relationships relevant to this article to disclose.

Figures

Fig. 1
Fig. 1
Flow chart for the study
Fig. 2
Fig. 2
Kaplan–Meier estimate for total survival for the cohort
Fig. 3
Fig. 3
Time for deaths in the HSCR cohort
See this image and copyright information in PMC

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