Anna Löf Granström1, Johan Danielson2, Britt Husberg3, Agneta Nordenskjöld4, Tomas Wester4. 1. Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden. Electronic address: anna.lof@ki.se. 2. Institution of Women's and Children's Health, Uppsala University, Uppsala, Sweden. 3. Department of CLINTEC, Karolinska Institutet, Stockholm, Sweden. 4. Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
Abstract
BACKGROUND AND AIMS: Hirschsprung's disease (HSCR) is treated surgically. There is a risk for faecal incontinence and constipation postoperatively. The long-term bowel functional outcome in adults and quality of life are sparsely studied. The aim of this study was to assess bowel function and quality of life in patients who had undergone surgery for HSCR during childhood. METHODS: All patients treated between 1969 and 1994 at St. Göran's Children's Hospital in Stockholm were invited to participate in the study. After consent, the patients received questionnaires containing general questions, validated questions on bowel function, questions about urinary function, SF-36 health survey (SF-36) and the Gastrointestinal Quality of Life Index (GIQLI). Clinical data were extracted from the case records. Controls matched for sex and age were randomly selected from the National Swedish Population Register. RESULTS: 48 of 60 (80%) invited patients responded to the questionnaires. Nine patients were excluded since the HSCR diagnosis could not be confirmed. The median age of the included patients was 28 (20-43) years. Most patients had undergone Soave's operation (73.4%) and two patients had a stoma at the time of follow-up. The bowel function was impaired in the HSCR group compared to controls, especially problems with flatulence, need to strain at defecation and several defecations for emptying. Patients in the HSCR group also had significantly more problems with faecal incontinence than controls. Quality of life according to SF-36 did not differ significantly between patients and controls, but the GIQLI score showed a significantly worse outcome in the HSCR group compared to the controls. CONCLUSION: General quality of life in adults treated for HSCR during childhood is comparable to controls. However, they have impaired bowel function and gastrointestinal quality of life.
BACKGROUND AND AIMS: Hirschsprung's disease (HSCR) is treated surgically. There is a risk for faecal incontinence and constipation postoperatively. The long-term bowel functional outcome in adults and quality of life are sparsely studied. The aim of this study was to assess bowel function and quality of life in patients who had undergone surgery for HSCR during childhood. METHODS: All patients treated between 1969 and 1994 at St. Göran's Children's Hospital in Stockholm were invited to participate in the study. After consent, the patients received questionnaires containing general questions, validated questions on bowel function, questions about urinary function, SF-36 health survey (SF-36) and the Gastrointestinal Quality of Life Index (GIQLI). Clinical data were extracted from the case records. Controls matched for sex and age were randomly selected from the National Swedish Population Register. RESULTS: 48 of 60 (80%) invited patients responded to the questionnaires. Nine patients were excluded since the HSCR diagnosis could not be confirmed. The median age of the included patients was 28 (20-43) years. Most patients had undergone Soave's operation (73.4%) and two patients had a stoma at the time of follow-up. The bowel function was impaired in the HSCR group compared to controls, especially problems with flatulence, need to strain at defecation and several defecations for emptying. Patients in the HSCR group also had significantly more problems with faecal incontinence than controls. Quality of life according to SF-36 did not differ significantly between patients and controls, but the GIQLI score showed a significantly worse outcome in the HSCR group compared to the controls. CONCLUSION: General quality of life in adults treated for HSCR during childhood is comparable to controls. However, they have impaired bowel function and gastrointestinal quality of life.
Authors: Kristiina Kyrklund; Cornelius E J Sloots; Ivo de Blaauw; Kristin Bjørnland; Udo Rolle; Duccio Cavalieri; Paola Francalanci; Fabio Fusaro; Annette Lemli; Nicole Schwarzer; Francesco Fascetti-Leon; Nikhil Thapar; Lars Søndergaard Johansen; Dominique Berrebi; Jean-Pierre Hugot; Célia Crétolle; Alice S Brooks; Robert M Hofstra; Tomas Wester; Mikko P Pakarinen Journal: Orphanet J Rare Dis Date: 2020-06-25 Impact factor: 4.123
Authors: Maggie L Westfal; Ongoly Okiemy; Patrick Ho Yu Chung; Jiexiong Feng; Changgui Lu; Go Miyano; Paul Kwong Hang Tam; Weibing Tang; Kenneth Kak Yuen Wong; Atsuyuki Yamataka; Richard A Guyer; Daniel P Doody; Allan M Goldstein Journal: J Pediatr Surg Date: 2021-07-21 Impact factor: 2.545