title: Surgical rehabilitation of short and dysmotile intestine in children and adults.
abstract: OBJECTIVE
  This is a descriptive study aiming to compare outcomes of intestinal rehabilitation surgery among pediatric and adult intestinal failure (IF) patients with either primary intestinal motility disorders or short bowel syndrome (SBS) treated by our nationwide program.
  
  
  METHODS
  Medical records of IF patients (n = 31, 71% children) having undergone autologous intestinal reconstructions (AIR) (n = 25), intestinal transplantation (ITx) (n = 5), or being listed for ITx (n = 2) between 1994 and 2014 were reviewed.
  
  
  RESULTS
  At surgery, median age was 3.4 (interquartile range, 1.0-22.1) in SBS (n = 22) and 16.5 (3.2-26.7) years in dysmotility patients (n = 9) who received median 60% and 83% of energy requirement parenterally, respectively. Median small bowel length was shorter in SBS than dysmotility patients (34 versus 157 cm, p < 0.001). Following AIR, none of the dysmotility patients achieved permanent intestinal autonomy, whereas 68% of SBS patients weaned off parenteral nutrition (PN) (p = 0.022) and none required listing for ITx. Five dysmotility patients who underwent ITx achieved intestinal autonomy. Regarding both AIR and ITx procedures, no significant difference in PN weaning was observed between the two subgroups. At last follow-up, 3.3 (0.6-8.0) years postoperatively, median plasma bilirubin was 6 (4-16) µmol/l, while liver biopsy showed fibrosis (Metavir stage 1-2) in 50% and cholestasis in 8%. Proportion of PN energy requirement had reduced significantly (p = 0.043) among PN-dependent SBS (n = 7) but not among dysmotility patients (n = 5). Overall survival was 90%.
  
  
  CONCLUSIONS
  AIR surgery was beneficial among selected SBS patients, whereas in intestinal dysmotility disorders, permanent PN weaning was only achieved by ITx.
authors.affiliation: Pediatric Liver and Gut Research Group, University of Helsinki , Helsinki , Finland.
authors.forename: Maria
authors.initials: M
authors.lastname: Hukkinen
authors.forename: Laura
authors.initials: L
authors.lastname: Merras-Salmio
authors.forename: Taina
authors.initials: T
authors.lastname: Sipponen
authors.forename: Annika
authors.initials: A
authors.lastname: Mutanen
authors.forename: Risto J
authors.initials: RJ
authors.lastname: Rintala
authors.forename: Heikki
authors.initials: H
authors.lastname: Mäkisalo
authors.forename: Mikko P
authors.initials: MP
authors.lastname: Pakarinen
mesh_terms: D000293:Adolescent; D000328:Adult; D001663:Bilirubin / Q000097:blood; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000503:physiopathology; D013505:Digestive System Surgical Procedures / Q000379:methods; D005260:Female; D006801:Humans; D007223:Infant; D007422:Intestines / Q000503:physiopathology* / Q000637:transplantation*; D008103:Liver Cirrhosis / Q000503:physiopathology; D008297:Male; D010289:Parenteral Nutrition, Total; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000534:rehabilitation* / Q000601:surgery*; D013534:Survival; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001663:Bilirubin
keywords: autologous intestinal reconstruction; intestinal failure; intestinal transplantation; motility disorders; short bowel syndrome