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

Abstract

At present there is no established therapy for treating patients with hereditary hemorrhagic telangiectasia (HHT) and symptomatic hepatic involvement. We present the results of a prospective study with 15 consecutive patients who were treated with staged hepatic artery embolization (HAE). Branches of the hepatic artery were selectively catheterized and embolized in stages using polyvinyl alcohol particles (PVA) and platinum microcoils or steel macrocoils. Prophylactic antibiotics, analgesics and anti-emetics were administered after every embolization. Clinical symptomatology and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 28 months; range 10-136 months). Five patients had abdominal pain and four patients had symptoms of portal hypertension. The cardiac output was raised in all patients, with cardiac failure being present in 11 patients. After treatment, pain resolved in all five patients, and portal hypertension improved in two of the four patients. The mean cardiac output decreased significantly ( P<0.001) from 12.57+/-3.27 l/min pre-treatment to 8.36+/-2.60 l/min at the end of follow-up. Symptoms arising from cardiac failure resolved or improved markedly in all but one patient. Cholangitis and/or cholecystitis occurred in three patients of whom two required a cholecystectomy. One patient with pre-existent hepatic cirrhosis died as a complication of the procedure. Staged HAE yields long-term relief of clinical symptoms in patients with HHT and hepatic involvement. Patients with pre-existing hepatic cirrhosis may be poor candidates for HAE.

The pulmonary vascular complications of hereditary haemorrhagic telangiectasia

“…Surgical hepatic artery ligation [49] and percutaneous hepatic artery embolisation [50] have been used at some centres for the treatment of liver VMs. However, these interventions can lead to the development of biliary ischaemia and in some cases serious hepatic necrosis [41,42].…”

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.…”

Impact of Hereditary Hemorrhagic Teleangiectasia on Quality of Life

“…Doppler Ultrasound has a positive predictive value close to 100% for LVMs in HHT patients [35]. Embo-lization is effective in improving symptoms temporarily but can cause severe side effects like liver transplantation [36].…”