Kristin Bjørnland1, Mikko P Pakarinen2, Pernilla Stenstrøm3, Kjetil J Stensrud4, Malla Neuvonen2, Anna L Granström5, Christina Graneli3, Are H Pripp6, Einar Arnbjörnsson3, Ragnhild Emblem4, Tomas Wester5, Risto J Rintala2. 1. Department of Paediatric Surgery, Oslo University Hospital-Rikshospitalet and University of Oslo, Oslo, Norway. Electronic address: Kristin.bjornland@medisin.uio.no. 2. Department of Paediatric Surgery, Children's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. 3. Department of Paediatric Surgery, Skåne University Hospital, Lund, Sweden. 4. Department of Paediatric Surgery, Oslo University Hospital-Rikshospitalet and University of Oslo, Oslo, Norway. 5. Department of Paediatric Surgery, Karolinska University Hospital, Stockholm, Sweden. 6. Department of Paediatric Surgery, Oslo Centre of Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital, Oslo, Norway.
Abstract
OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS: Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. RESULTS: 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. CONCLUSION: A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.
OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS:Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. RESULTS: 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. CONCLUSION: A significant number of HDpatients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HDpatients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HDpatients is warranted. Prognosis Study: Level II.
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