Pernilla Stenström1, Matilda Brautigam2, Helena Borg2, Christina Graneli3, Helene Engstrand Lilja4, Tomas Wester5. 1. Lund University, Department of Pediatric Surgery, Skane University Hospital Lund, 22185 Lund, Sweden. Electronic address: pernilla.stenstrom@med.lu.se. 2. The Queen Silvia Children's Hospital, The Sahlgrenska Academy University of Gothenburg, Gothenburg, Sweden. 3. Lund University, Department of Pediatric Surgery, Skane University Hospital Lund, 22185 Lund, Sweden. 4. Unit of Pediatric Surgery, Department of Women's and Children's Health, University Children's Hospital, Uppsala, Sweden. 5. Unit of Pediatric Surgery, Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
Abstract
BACKGROUND: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20years. METHODS: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients >4years old without stoma answered a questionnaire regarding bowel function (bowel function score, BFS, score 1-20), medical treatment and nutrition. RESULTS: Twenty-seven children were included. Twenty-five children were reconstructed at median age of 56 (4-236) weeks. Reconstruction procedures included Swenson (6), Soave (5), mucosectomy with short muscular cuff with or without J-pouch (9), Duhamel (3) and Rehbein (2). There was no mortality. The median follow-up time was 9.5years (8months-20years). At follow-up 7 (26%) patients had an ileostomy, 4 with a syndrome. Eight patients required parenteral support, until a median age of 11 (2-24) months. Oral energy support was used by 5/27 (15%), still 5/22 (23%) were underweighted. Obstructive symptoms were reported by 7/20 (31%). All 17 patients >4years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24h was 5 (1-30). Fecal accidents at least once per week was reported by 4 (24%), and social problems by 8 (47%). The median BFS was 15 (11-19) without any gender differences. CONCLUSION: One-third of patients with TCA report obstructive symptoms, one-third need additional nutrition and one-fifth require a permanent stoma. TCA have a negative impact on social life. Subsequently, children with TCA need a careful lifelong follow-up of specialized teams.
BACKGROUND: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20years. METHODS: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients >4years old without stoma answered a questionnaire regarding bowel function (bowel function score, BFS, score 1-20), medical treatment and nutrition. RESULTS: Twenty-seven children were included. Twenty-five children were reconstructed at median age of 56 (4-236) weeks. Reconstruction procedures included Swenson (6), Soave (5), mucosectomy with short muscular cuff with or without J-pouch (9), Duhamel (3) and Rehbein (2). There was no mortality. The median follow-up time was 9.5years (8months-20years). At follow-up 7 (26%) patients had an ileostomy, 4 with a syndrome. Eight patients required parenteral support, until a median age of 11 (2-24) months. Oral energy support was used by 5/27 (15%), still 5/22 (23%) were underweighted. Obstructive symptoms were reported by 7/20 (31%). All 17 patients >4years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24h was 5 (1-30). Fecal accidents at least once per week was reported by 4 (24%), and social problems by 8 (47%). The median BFS was 15 (11-19) without any gender differences. CONCLUSION: One-third of patients with TCA report obstructive symptoms, one-third need additional nutrition and one-fifth require a permanent stoma. TCA have a negative impact on social life. Subsequently, children with TCA need a careful lifelong follow-up of specialized teams.
Authors: Akemi L Kawaguchi; Yigit S Guner; Stig Sømme; Alexandria C Quesenberry; L Grier Arthur; Juan E Sola; Cynthia D Downard; Rebecca M Rentea; Patricia A Valusek; Caitlin A Smith; Mark B Slidell; Robert L Ricca; Roshni Dasgupta; Elizabeth Renaud; Doug Miniati; Jarod McAteer; Alana L Beres; Julia Grabowski; Shawn D St Peter; Ankush Gosain Journal: J Pediatr Surg Date: 2021-03-28 Impact factor: 2.549
Authors: P Stenström; K Kyrklund; M Bräutigam; H Engstrand Lilja; K Juul Stensrud; A Löf Granström; N Qvist; L Söndergaard Johansson; E Arnbjörnsson; H Borg; T Wester; K Björnland; M P Pakarinen Journal: BJS Open Date: 2020-07-13