title: Isolated liver transplant in infants with short bowel syndrome: insights into outcomes and prognostic factors.
abstract: OBJECTIVE
  Selected infants with short bowel syndrome (SBS) and progressive intestinal failure associated liver disease (IFALD) may benefit from isolated liver transplantation (iLTx). The aim of the study is to identify risk factors for unfavourable outcome in iLTx.
  
  
  METHODS
  A retrospective review of medical records from 1998 to 2005 was undertaken. Risk factors were assessed by comparing long-term survivors with those who died after iLTx.
  
  
  RESULTS
  Fifteen iLTx were performed in 14 infants with IFALD. All were parenteral nutrition (PN) dependent, but had tolerated enterally 54% (38-100) of energy intake before iLTx. Median residual bowel was 60 cm (30-200). Eight out of 14 had intact ileocaecal valve (ICV). Median bilirubin was 298 micromol/L (87-715) and all had portal hypertension. Eight out of 9 survivors were weaned from PN after median 15 months. In 4 out of 9 children, nontransplant surgery after iLTx facilitated intestinal adaptation. Growth velocity had improved at 3 years after iLTx (P=0.001). Five children who died had poor enteral tolerance following iLTx (P<0.002), which correlated with pretransplant dysmotility seen in 4 out of 5 children shown by contrast studies (P=0.02)and increased frequency of line infections before (>6/year P<0.04) and after (P<0.001) iLTx.
  
  
  CONCLUSIONS
  Isolated liver transplantation is a lifesaving option for selected children with SBS and IFALD. Revised criteria are proposed: progressive IFALD; 50 cm functional bowel in absence of ICV or 30 cm with ICV; 50% daily energy intake tolerated enterally for 4 weeks with satisfactory growth; and children with dysmotile bowel should be assessed for combined liver/bowel transplant unless the dysmotility is resolved and associated with minimal line infections.
authors.affiliation: Liver Unit, Birmingham Children's Hospital (BCH), Birmingham, UK.
authors.forename: D
authors.initials: D
authors.lastname: Dell-Olio
authors.forename: S V
authors.initials: SV
authors.lastname: Beath
authors.forename: J
authors.initials: J
authors.lastname: de Ville de Goyet
authors.forename: S
authors.initials: S
authors.lastname: Clarke
authors.forename: P
authors.initials: P
authors.lastname: Davies
authors.forename: C
authors.initials: C
authors.lastname: Lloyd
authors.forename: S
authors.initials: S
authors.lastname: Protheroe
authors.forename: A J W
authors.initials: AJ
authors.lastname: Millar
authors.forename: D A
authors.initials: DA
authors.lastname: Kelly
authors.forename: G L
authors.initials: GL
authors.lastname: Gupte
mesh_terms: D049628:Body Size; D004750:Enteral Nutrition; D005260:Female; D005769:Gastrointestinal Motility; D006128:Growth; D006801:Humans; D007223:Infant; D007410:Intestinal Diseases / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D053208:Kaplan-Meier Estimate; D008107:Liver Diseases / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality; D008297:Male; D010288:Parenteral Nutrition / Q000706:statistics & numerical data; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012778:Short Bowel Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome