Maria Hukkinen1, Antti Koivusalo2, Laura Merras-Salmio3, Risto J Rintala2, Mikko P Pakarinen4. 1. Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland. Electronic address: maria.hukkinen@helsinki.fi. 2. Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland. 3. Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland; Section of Pediatric Gastroenterology, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland. 4. Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland; Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland.
Abstract
BACKGROUND: The prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence and extent of small bowel involvement. METHODS: Medical records of TCA patients treated in Helsinki University Children's Hospital during 1984-2013 (n=21) were reviewed. RESULTS: The aganglionosis extended up to cecum or distal ileum (n=12), mid small bowel (n=4), or duodenojejunal flexure (n=5). Patients underwent resection of distal aganglionic bowel with ileoanal (IAA) or jejunoanal anastomosis (JAA) with (n=9) or without J-pouch (n=5), Lester-Martin pull-through (n=1), or were left with an end-jejunostomy (n=6). Further procedures included autologous intestinal reconstruction (n=3) and ITx/listing for ITx (n=2). Compared to distal ileum aganglionosis, patients with more proximal disease required parenteral nutrition (PN) more often (100% vs. 25%) and weaned off PN less frequently (p=0.001). At last follow-up 6.5 (interquartile range 2.5-14.5) years postoperatively, all patients with distal ileum aganglionosis were off PN and alive compared to 78% on PN (p<0.001) and 67% alive (p=0.063) of those with more proximal disease. All had normal plasma bilirubin and patients with preserved intestinal continuity (n=13) were continent. CONCLUSIONS: Outcomes following restorative proctocolectomy for aganglionosis extending up to mid small bowel are promising, whereas long-term outlook in proximal small intestinal disease is dismal without ITx.
BACKGROUND: The prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence and extent of small bowel involvement. METHODS: Medical records of TCA patients treated in Helsinki University Children's Hospital during 1984-2013 (n=21) were reviewed. RESULTS: The aganglionosis extended up to cecum or distal ileum (n=12), mid small bowel (n=4), or duodenojejunal flexure (n=5). Patients underwent resection of distal aganglionic bowel with ileoanal (IAA) or jejunoanal anastomosis (JAA) with (n=9) or without J-pouch (n=5), Lester-Martin pull-through (n=1), or were left with an end-jejunostomy (n=6). Further procedures included autologous intestinal reconstruction (n=3) and ITx/listing for ITx (n=2). Compared to distal ileum aganglionosis, patients with more proximal disease required parenteral nutrition (PN) more often (100% vs. 25%) and weaned off PN less frequently (p=0.001). At last follow-up 6.5 (interquartile range 2.5-14.5) years postoperatively, all patients with distal ileum aganglionosis were off PN and alive compared to 78% on PN (p<0.001) and 67% alive (p=0.063) of those with more proximal disease. All had normal plasma bilirubin and patients with preserved intestinal continuity (n=13) were continent. CONCLUSIONS: Outcomes following restorative proctocolectomy for aganglionosis extending up to mid small bowel are promising, whereas long-term outlook in proximal small intestinal disease is dismal without ITx.
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Authors: Fereshteh Salimi Jazi; Julia M Chandler; Chad M Thorson; Tiffany J Sinclair; Florette K Hazard; John A Kerner; Sanjeev Dutta; James C Y Dunn; Stephanie D Chao Journal: BMC Pediatr Date: 2019-04-05 Impact factor: 2.125
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