title: Hemodynamic failure as an indication to urgent liver transplantation in infants with giant hepatic hemangiomas or vascular malformations--report of four cases.
abstract: The aim of this study was to present acute hemodynamic failure as a rare indication for liver transplantation in neonates and infants with liver hemangiomatosis. We report four patients aged one to six months with giant liver hemangiomas, with huge arterio-venous shunting within these malformations. In three, many skin hemangiomas were found. All children developed right ventricular failure. In two, a trial of pharmacological reduction was attempted with corticosteroids and cyclophosphamide. In one patient, the arterio-venous fistulas were embolized without any improvement in hemodynamic status. Two children underwent rescue hepatic artery surgical ligation, which did not prevent heart and then multiorgan failure including liver failure. After unsuccessful conventional therapy, all infants were considered for urgent liver transplantation; in three cases, it was performed with a living-related donor, and in one case with a deceased donor. All patients are alive and well with the follow-up between nine and 37 months after transplantation. Liver transplantation should be considered as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency when conventional therapy is unsuccessful and multiorgan failure develops.
authors.affiliation: Departments of Pediatric Surgery and Organ Transplantation, The Children's Memorial Health Institute, Warsaw, Poland. gonia.mark@wp.pl
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Markiewicz-Kijewska
authors.forename: Weronika
authors.initials: W
authors.lastname: Kasprzyk
authors.forename: Dorota
authors.initials: D
authors.lastname: Broniszczak
authors.forename: Ludmila
authors.initials: L
authors.lastname: Bacewicz
authors.forename: Anna
authors.initials: A
authors.lastname: Ostoja-Chyzynska
authors.forename: Hor
authors.initials: H
authors.lastname: Ismail
authors.forename: Andrzej
authors.initials: A
authors.lastname: Kosciesza
authors.forename: Bozenna
authors.initials: B
authors.lastname: Dembowska-Baginska
authors.forename: Joanna
authors.initials: J
authors.lastname: Teisseyre
authors.forename: Przemyslaw
authors.initials: P
authors.lastname: Kluge
authors.forename: Grazyna
authors.initials: G
authors.lastname: Brzezinska-Rajszys
authors.forename: Irena
authors.initials: I
authors.lastname: Jankowska
authors.forename: Piotr
authors.initials: P
authors.lastname: Kalicinski
mesh_terms: D001164:Arteriovenous Fistula / Q000473:pathology; D005260:Female; D006352:Heart Ventricles / Q000503:physiopathology; D006391:Hemangioma / Q000601:surgery* / Q000628:therapy; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000601:surgery / Q000628:therapy*; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000601:surgery* / Q000628:therapy