Giant Symptomatic Aneurysm Secondary to Hereditary Hemorrhagic Telangiectasia of a Main Hepatic Artery with Aberrant Origin in Superior Mesenteric Artery.

BACKGROUND

Hereditary hemorrhagic telangiectasia (HHT) is a rare disease consistent in vascular dysplasias affecting different organs. Liver involvement includes telangiectases, arteriovenous shunting, and ischemic cholangitis, and its prevalence ranges from 8% to 31%. Spontaneous pseudoaneurysms have never been reported associated to HHT. Several variations in the origin of the main hepatic artery (MHA) have been described in large radiological series and can be found in around 4% of general population.

METHODS

A 64-year-old woman with history of HHT was diagnosed of acute symptomatic saccular aneurysm involving the MHA. The associated portovenous fistulae suggested that liver perfusion could be impaired if embolization of the MHA without revascularization was performed. Because of an aberrant origin on the superior mesenteric artery, a hybrid procedure was designed, consistent on endovascular exclusion of the MHA associated with a surgical aortic-hepatic bypass graft.

RESULTS

Angio computed tomography (Angio CT) was performed at discharge, 1 month, and 6 months after intervention, revealed complete exclusion of the aneurysm, which reduced 50% in diameter after 6 months. Aneurysms of the MHA can be associated with HHT when intrahepatic arteriovenous fistulae are present. This complication is rare, and the reported cases have been treated by surgical and totally endovascular procedures. The confluence of this very rare condition with an uncommon vascular anatomy makes this a unique case that required the design of a customized intervention.

CONCLUSIONS

Hybrid procedures are useful to treat conditions that cannot be solved with standard interventions. Longer series of cases with long-term surveillance are needed to asses the results of these techniques in these complex pathology.