title: Isolated liver transplantation in infants with end-stage liver disease associated with short bowel syndrome.
abstract: OBJECTIVE
  To evaluate experience with isolated orthotopic liver transplantation in children with liver failure associated with short bowel syndrome (SBS).
  
  
  BACKGROUND
  Infants who have liver failure as a result of SBS are frequently referred for consideration for combined liver and small bowel transplantation. In a few patients the liver disease develops despite a seemingly adequate bowel, which if given time and appropriate management has the potential for full enteral adaptation. There is a limited literature suggesting the utility of OLT without replacement of the native bowel. The advantages over combined liver and small bowel transplantation are clear: organ availability is greater, liver-reduction techniques are well established, lower immunosuppression is required, and there is greater experience in the care of children after orthotopic liver transplantation.
  
  
  METHODS
  Eleven infants, considered to have a good prospect of eventual gut adaptation to full enteral nutrition if it were not for their advanced liver disease, underwent isolated orthotopic liver transplantation. Age range was 6.5 to 17.7 months. All patients had been dependent on parenteral feeding but had also shown significant enteral tolerance at some time before listing for transplantation. Advanced liver disease was apparent both clinically and on histologic examination. All were jaundiced and had low albumin levels, and most had coagulopathy. As a group the infants had growth retardation. Estimated remaining length of small bowel beyond the ligament of Treitz was in the range of 25 to more than 100 cm. Six infants retained their ileocecal valve.
  
  
  RESULTS
  Thirteen liver transplants were performed in the 11 patients. A combination of whole livers (n = 6) and reduced-size grafts, of which three were from living-related donors, were used. Biliary anastomosis was duct-to-duct in eight instances and involved a short Roux limb in the others. Eight patients are alive with follow-up of 15 to 66 months. Three deaths have occurred after transplantation as a result of sepsis. Of eight surviving patients, only two continue to receive intravenous support and in both there is increasing enteral tolerance. Since transplantation, all surviving children have shown adequate growth with maintenance of pretransplant centiles.
  
  
  CONCLUSIONS
  In selected infants with liver failure secondary to short bowel syndrome in whom complete enteral autonomy is anticipated, isolated liver transplantation can offer long-term survival.
authors.affiliation: Department of Pediatrics, University of Nebraska, Omaha, Nebraska 68198-3285, USA. shorslen@surgery.unmc.edu
authors.forename: Simon P
authors.initials: SP
authors.lastname: Horslen
authors.forename: Debra L
authors.initials: DL
authors.lastname: Sudan
authors.forename: Kishore R
authors.initials: KR
authors.lastname: Iyer
authors.forename: Stuart S
authors.initials: SS
authors.lastname: Kaufman
authors.forename: Angie K
authors.initials: AK
authors.lastname: Iverson
authors.forename: Ira J
authors.initials: IJ
authors.lastname: Fox
authors.forename: Byers W
authors.initials: BW
authors.lastname: Shaw
authors.forename: Alan N
authors.initials: AN
authors.lastname: Langnas
mesh_terms: D006801:Humans; D007223:Infant; D017093:Liver Failure / Q000209:etiology* / Q000601:surgery*; D016031:Liver Transplantation; D012778:Short Bowel Syndrome / Q000150:complications*; D016896:Treatment Outcome