When Cholangitis Reveals Liver Involvement in Hereditary Hemorrhagic Telangiectasia: A Case Report

TL;DR

A case report shows how cholangitis can indicate liver issues in a patient with hereditary hemorrhagic telangiectasia, emphasizing the need for detailed imaging.

Contribution

This case highlights the rare biliary complications of HHT and the value of multimodal imaging in diagnosing hepatic involvement.

Findings

The patient had hepatic artery dilatation, AVMs, and biliary dilation confirmed by imaging.

Cholangitis was managed, and the patient is under surveillance to prevent complications.

Multimodal imaging revealed both vascular and biliary abnormalities in HHT.

Abstract

Hereditary hemorrhagic telangiectasia (HHT) syndrome is a rare autosomal dominant hereditary disorder characterized by multiple arteriovenous malformations (AVMs), resulting from capillary dysplasia, responsible for arteriovenous shunting. It can affect various organs, including the lungs, liver, and central nervous system. Clinical manifestations are epistaxis, telangiectasias, and complications of visceral AVMs. Biliary involvement is uncommon and typically results from biliary ischemia. This can lead to complications such as cholangitis, biliary strictures, cysts, necrosis, and bilomas. Although less frequent, biliary complications may present similarly to bile duct obstruction or sepsis and necessitate careful management. We report the case of a 55-year-old patient with a history of unexplored epistaxis who presented with hepatic colic. Clinical and laboratory findings revealed telangiectasias, cholestasis, and an inflammatory syndrome. Imaging revealed hepatic artery dilatation, multiple AVMs in hepatic telangiectasias and a porto-hepatic shunt, and segmental cystic dilation of the bile ducts. The patient was treated for cholangitis and remains under surveillance. This case highlights the importance of multimodal imaging in evaluating hepatic involvement in patients with HHT. Indeed, a combination of ultrasound, multiphasic CT, and dynamic MRI helps reveal not only typical vascular abnormalities, including hepatic artery dilatation, telangiectasias, and arteriovenous shunts, but also biliary involvement. Regular follow-up is necessary to tailor management and prevent potential complications associated with this hereditary vascular syndrome.

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