Liver transplantation as definitive therapy for complications after arterial embolization for hepatic manifestations of hereditary hemorrhagic telangiectasia

Abstract

Experience with hepatic artery embolization for the treatment of symptomatic hepatic arteriovenous malformations (AVMs) in Rendu-OslerWeber disease is limited. We report 2 cases of hepatic AVMs that caused mesenteric angina-like symptoms that were treated with embolization. Both patients developed parenchymal and bile duct necrosis, intrahepatic bilomas, and refractory biliary sepsis, subsequently leading to liver failure. We hypothesize that the pathophysiological cause of biliary necrosis in this setting is similar to that which occurs in the setting of hepatic artery thrombosis of the liver allograft. Progressive liver failure in these patients was treated successfully by liver transplantation. Liver transplantation offers definitive therapy by removing the source of ongoing sepsis, restoring normal liver function, and eliminating the intrahepatic AV shunt.

Hereditary hemorrhagic telangiectasia: An overview of diagnosis and management in the molecular era for clinicians

“…7,56 • Symptomatic liver involvement is difficult to treat, with embolization resulting in a high mortality due to liver infarction. 34,58,59 At present, the treatment of choice is liver transplantation. 60 • Medications that interfere with normal coagulation such as aspirin and ibuprofen should be avoided.…”

Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations

“…b Repeat chest radiograph 2 years following five sessions of HAE; marked reduction in cardiac size. The cardiac output decreased from 13 l/min to 6 l/min subjected to liver transplantation [12,25,[29][30][31][32][33][34][35][36]. Of these patients, one underwent hepatic artery ligation as well as liver transplantation [12], and another patient was subjected to HAE followed by hepatic artery ligation [11]; five patients underwent HAE followed by liver transplantation [25,29,30].…”

“…A review of the relevant literature has yielded 7 cases that have been treated with hepatic artery ligation or banding [9][10][11][12][13][14], 25 patients who have undergone HAE [11,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] and 16 patients who have been . 4 a Chest radiograph of a patient with HHT presenting with cardiac failure and intractable abdominal pain.…”

Preoperative arterial embolization of large liver hemangiomas

“…During the follow-up period, we did not observe ischemic cholangitis, ischemic cholecystitis, pyogenic liver abscess, or focal biliary or hepatic necrosis with or without biliary sepsis. Those biliary complications are well-described as main complications (1.4%-60%) related to hepatic arterial embolization (40)(41)(42)(43). However, most patients displaying biliary complications after hepatic arterial embolization have extensive parenchymal diseases, like hereditary hemorrhagic telangiectasia.…”