BACKGROUND/ PURPOSE: Hirschsprung disease (HD) is considered curable, but the postoperative bowel function is not always satisfactory. In this study, we evaluated the general condition, bowel function, and social performance in adults who were older than 17years of age who had undergone definitive operations in childhood. METHOD: From 1984 to 2016, 110 patients with HD underwent definitive surgery at our institute. Sixty-three patients who had undergone the transabdominal Soave procedure reached 18years of age. Their present status and symptoms, anorectal function, genitourinary function, and social performance were evaluated during the clinical follow-up via a questionnaire survey. RESULT: The mean age of the questionnaire respondents was 25.0 (19-37) years. The bowel function was mostly good. However, 56% of patients had abdominal pain more than once a week. Regarding evacuation symptoms, incontinence and soiling occurred in 18.7%. Among the respondents 33.3% were married, and 60% of those who were married had children. The respondents had achieved success in their education and professional careers. CONCLUSION: The bowel function of most patients was satisfactory, although some had chronic abdominal symptoms. Pediatric surgeons should continue trying to achieve complete bowel function after definitive surgery of HD. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: Level III.
BACKGROUND/ PURPOSE:Hirschsprung disease (HD) is considered curable, but the postoperative bowel function is not always satisfactory. In this study, we evaluated the general condition, bowel function, and social performance in adults who were older than 17years of age who had undergone definitive operations in childhood. METHOD: From 1984 to 2016, 110 patients with HD underwent definitive surgery at our institute. Sixty-three patients who had undergone the transabdominal Soave procedure reached 18years of age. Their present status and symptoms, anorectal function, genitourinary function, and social performance were evaluated during the clinical follow-up via a questionnaire survey. RESULT: The mean age of the questionnaire respondents was 25.0 (19-37) years. The bowel function was mostly good. However, 56% of patients had abdominal pain more than once a week. Regarding evacuation symptoms, incontinence and soiling occurred in 18.7%. Among the respondents 33.3% were married, and 60% of those who were married had children. The respondents had achieved success in their education and professional careers. CONCLUSION: The bowel function of most patients was satisfactory, although some had chronic abdominal symptoms. Pediatric surgeons should continue trying to achieve complete bowel function after definitive surgery of HD. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: Level III.
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