Interventional approach to splenic vascular malformation, a rare manifestation of Rendu-Osler-Weber syndrome

Abstract

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant genetic disorder that interferes with angiogenesis and leads to abnormal vascular development. It presents as mucocutaneous telangiectasia when small or arteriovenous malformations (AVMs) when large. AVMs involve different organs, such as the lungs, brain, liver, spinal cord, and gastrointestinal tract, and can cause various life-threatening complications depending on their location and size.

Splenic vascular malformations are rare in patients with HHT and require treatment owing to the risk of rupture and spontaneous hemoperitoneum. This case report details the management of an 8-cm atypical AVM located in the spleen, which was treated with selective transarterial embolization using a liquid embolic agent.