Fertility in patients with Hirschsprung's disease: population-based cohort study.

BACKGROUND: The aim of this study was to assess fertility in patients treated for Hirschsprung's disease. METHODS: This was a nationwide, population-based cohort study, including all patients with Hirschsprung's disease registered in the Swedish National Patient Register between 1964 and 2004. Five age- and sex-matched controls per patient were randomly selected by Statistics Sweden. Outcome data were retrieved from the Multi-Generation Register, and the Swedish National Patient Register. Study exposure was Hirschsprung's disease and the primary outcome was fertility, defined as having one or more children. Individuals with chromosomal anomalies were excluded. RESULTS: The study cohort comprised 597 patients with Hirschsprung's disease (143 female) and 2969 controls (714 female). The mean(s.d.) age at follow-up was 29.6(10.0) years for patients and 29.8(10.1) years for the controls. A total of 191 (32.0 per cent) patients compared with 1072 (36.1 per cent) controls had one or more children (P = 0.061). The analysis showed that fewer female patients with Hirschsprung's disease had a child (29.4 versus 38.7 per cent, P = 0.037), they were older when they gave birth to their first child (28.1 versus 26.4 years, P = 0.033), and they had fewer children. Of the female patients with Hirschsprung's disease, 19 (45.2 per cent) had only one child, compared with 79 (28.6 per cent) of the female control group (P = 0.047). No difference was noted in the male group in this regard. CONCLUSION: Female patients with Hirschsprung's disease were less likely to have a child, had fewer children, and were older when they gave birth to their first child compared with the controls, indicating impaired fertility. There was no significant difference between male patients with Hirschsprung's disease and controls.

Evaluation of Bowel Function, Urinary Tract Function, and Quality of Life after Transanal Endorectal Pull-Through Surgery for Hirschsprung's Disease.

INTRODUCTION: The objective of this study is to determine short-term complications and evaluate long-term bowel function, lower urinary tract symptoms, and quality of life (QoL) in patients treated for Hirschsprung's disease (HSCR) with transanal endorectal pull-though (TERPT) compared with healthy controls. MATERIALS AND METHODS: This cross-sectional case-control study included 30 HSCR patients treated with TERPT in 2006 to 2014 at Karolinska University Hospital, and 30 healthy controls matched for age and gender. Data on short-term complications were compiled from medical records and classified according to Clavien-Dindo. Bowel function and QoL were evaluated with the validated questionnaires bowel function score and KIDSCREEN-52. Lower urinary tract symptoms were evaluated through an 8-item lower urinary tract symptoms (LUTS) questionnaire. RESULTS: Six (20%) patients had a short-term postoperative complication according to Clavien-Dindo, with insufficient pain management being the most common complication. The median age at follow-up was 7 years (range = 4-11). Median bowel function score was significantly lower in HSCR patients than in controls, 14 versus 19 (p < 0.001). Twenty-one of the HSCR patients reported impaired bowel function compared with two of the controls (p < 0.001). The overall prevalence of LUTS was 11 (38%) in the HSCR patients compared with seven (23%) in the controls (p = 0.751). HSCR patients reported a slightly lower QoL in the KIDSCREEN domain "financial resources" compared with controls (p = 0.008). CONCLUSION: According to Clavien-Dindo, short-term postoperative complications occurred in 20% of the patients. Impaired bowel function persists throughout childhood for most HSCR patients. The prevalence of LUTS and QoL is not affected in HSCR patients compared with controls.