title: Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis.
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.
authors.affiliation: 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.
authors.forename: Maria
authors.initials: M
authors.lastname: Hukkinen
authors.affiliation: Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland.
authors.forename: Antti
authors.initials: A
authors.lastname: Koivusalo
authors.affiliation: 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.
authors.forename: Laura
authors.initials: L
authors.lastname: Merras-Salmio
authors.affiliation: Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, PO Box 281, FIN-00029 HUS, Helsinki, Finland.
authors.forename: Risto J
authors.initials: RJ
authors.lastname: Rintala
authors.affiliation: 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.
authors.forename: Mikko P
authors.initials: MP
authors.lastname: Pakarinen
mesh_terms: D000293:Adolescent; D001663:Bilirubin / Q000097:blood; D002429:Cecal Diseases / Q000401:mortality* / Q000473:pathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D039021:Colonic Pouches; D005260:Female; D006627:Hirschsprung Disease / Q000401:mortality* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007077:Ileal Diseases / Q000401:mortality* / Q000473:pathology / Q000601:surgery*; D007421:Intestine, Small / Q000473:pathology; D007582:Jejunostomy; D008297:Male; D010288:Parenteral Nutrition; D010289:Parenteral Nutrition, Total; D011184:Postoperative Period; D016737:Proctocolectomy, Restorative / Q000379:methods; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D001663:Bilirubin
keywords: Autologous intestinal reconstruction procedures; Hirschsprung disease; Intestinal failure; Intestinal transplantation; Short bowel syndrome