title: Neurologic outcome of urea cycle disorder liver transplant recipients may be predicted by pretransplant neurological imaging.
abstract: Liver transplantation treats the hepatic affectation of UCDs; however, irreversible neurologic damage pretransplant is difficult to assess providing transplant teams with ethical dilemmas for liver transplantation. The purpose of our study was to determine whether pretransplant neuroimaging can predict developmental outcomes post-liver-transplant in children with UCDs.
  
  
  METHODS
  Patients undergoing liver transplantation for UCDs at Cincinnati Children's Hospital Medical Center between 2002 and 2012 were identified. Neurologic assessments prior to and after transplantation were categorized into mild, moderate, or severe disability. Neuroimaging data were categorized into mild, moderate, or severe by a single pediatric neuroradiologist.
  
  
  RESULTS
  Fifteen patients were identified of whom eight had neuroimaging prior to transplantation. Of the eight patients that had neuroimaging, four were categorized as severe, one moderate, and three no-to-mild delay. All four patients whose imaging was severe were found to have moderate-to-severe neurologic delay. Of the three patients with no-to-mild changes on neuroimaging two of three were found to have no-to-mild delay on developmental assessments after transplantation.
  
  
  CONCLUSIONS
  Neuroimaging may be a helpful tool in determining developmental prognosis and outcomes post-liver-transplantation for UCDs. Further studies maybe needed to validate our preliminary findings.
authors.affiliation: Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Scott M
authors.initials: SM
authors.lastname: Bolton
authors.affiliation: Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: Campbell
authors.affiliation: Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Marcia
authors.initials: M
authors.lastname: Kukreja
authors.affiliation: Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Rohit
authors.initials: R
authors.lastname: Kohli
mesh_terms: D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D017093:Liver Failure / Q000601:surgery*; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications / Q000601:surgery*; D059906:Neuroimaging; D009820:Ohio; D057234:Preoperative Period; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome; D056806:Urea Cycle Disorders, Inborn / Q000150:complications / Q000601:surgery*
keywords: inborn error of metabolism; liver transplantation; pediatric