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.

Critical infantile hepatic hemangioma: results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group

“…According to the United Network for Organ Sharing (UNOS) report [10] surveying patients of all ages, 110 patients with an average age of 36 years underwent 126 liver transplantations for hepatic hemangioendothelioma between 1987 and 2005, and the 5-year survival rate was 64%. Markiewicz-Kijewska et al [11] reported 4 infantile cases of urgent liver transplantation for hemodynamic failure. Thus, transplantation may be indicated also for urgent cases with hemodynamic failure.…”

Hypergiant Hepatic Hemangiomas: Case Series

“…Hepatic irradiation has been reported to produce complete regression of hepatic hemangioma with minimal morbidity. Liver transplantation is described as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency, and when conventional therapy is unsuccessful and multiorgan failure develops [8].…”

Effectiveness of Propranolol in a Newborn with Liver Hemangiomatosis

“…Visceral hemangiomatosis is a potentially life-threatening disease. In the past, invasive treatments such as liver-artery ligation [3 -5] or urgent liver transplantation [10] were used. Medical treatments such as corticosteroids, interferon alpha 2a and cytotoxic drugs may be associated with severe side-eff ects ranging from failure to thrive and paralysis to secondary acute myeloid leukemia, respectively [15] .…”

“…Cases of visceral involvement with many liver arteriovenous fi stulas may cause high output cardiac failure with life-threatening consequences. To treat progressive congestive heart failure, many therapies have been introduced: hepatic artery ligation (fi rst report in 1967) [3 -5] , radiation therapy [6] , partial liver resection [7] , special compression sutures for aff ected liver tissue [8] , arterial hepatic embolization [9] , urgent liver transplantation [10] and various medical treatments with prednisone, interferon alpha 2a [11] , cyclophosphamide [12 -14] and vincristine [15] . In June 2008, 2 French groups from Montpellier and Bordeaux presented their fi rst compelling results on the treatment of IH with a non-selective beta-blocker at the 17 th International Workshop of the International Society for the Study of Vascular Anomalies (ISSVA) in Boston [16] .…”