AIM: The study's aim is to determine long-term outcomes in a large cohort of pediatric and young adult patients who underwent proctocolectomy with ileal pouch anal anastomsis (IPAA) for ulcerative colitis (UC). METHODS: Patients diagnosed with UC in childhood or adolescence (age≤21years) who underwent IPAA in childhood, adolescence, or young adulthood between 1982 and 1997 were contacted to determine pouch history, complications, and quality of life. RESULTS: Data were obtained from 74 patients out of a previously reported cohort. Median age at diagnosis of UC was 15years and at surgery was 18years. Median follow-up was 20years. Complications during follow-up were pouchitis (45%), strictures (16%), fistulae (30%), obstruction (20%), and change of diagnosis to Crohn's (28%). Twenty-three percent reported no complications. Fourteen percent had pouch failure, with Crohn's and fistulae reported to be the most frequent complications. Seventy-nine percent reported being very satisfied at 20years follow-up. CONCLUSION: To our knowledge, this study represents the largest cohort with the longest follow-up of pediatric and young adult patients undergoing IPAA for UC. Change in diagnosis to Crohn's and development of fistulae are risk factors for pouch failure. Despite reported complications, IPAA remains an excellent option for pediatric patients with UC.
AIM: The study's aim is to determine long-term outcomes in a large cohort of pediatric and young adult patients who underwent proctocolectomy with ileal pouch anal anastomsis (IPAA) for ulcerative colitis (UC). METHODS:Patients diagnosed with UC in childhood or adolescence (age≤21years) who underwent IPAA in childhood, adolescence, or young adulthood between 1982 and 1997 were contacted to determine pouch history, complications, and quality of life. RESULTS: Data were obtained from 74 patients out of a previously reported cohort. Median age at diagnosis of UC was 15years and at surgery was 18years. Median follow-up was 20years. Complications during follow-up were pouchitis (45%), strictures (16%), fistulae (30%), obstruction (20%), and change of diagnosis to Crohn's (28%). Twenty-three percent reported no complications. Fourteen percent had pouch failure, with Crohn's and fistulae reported to be the most frequent complications. Seventy-nine percent reported being very satisfied at 20years follow-up. CONCLUSION: To our knowledge, this study represents the largest cohort with the longest follow-up of pediatric and young adult patients undergoing IPAA for UC. Change in diagnosis to Crohn's and development of fistulae are risk factors for pouch failure. Despite reported complications, IPAA remains an excellent option for pediatric patients with UC.
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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