An Splenic Artery Aneurysm and Focal Nodular Hyperplasia Associated with an Abdominal Vascular Abnormality of Hereditary Hemorrhagic Telangiectasia

Abstract

In October 2021, a 51-year-old woman developed a skin rash. Abdominal computed tomography revealed a large splenic artery aneurysm and an intrahepatic portovenous shunt. As her splenic artery aneurysm was at risk of rupture, she was referred to the Kindai University Hospital and underwent coiling surgery. In October 2023, approximately two years after she had been initially referred, contrast-enhanced ultrasound revealed findings suggestive of focal nodular hyperplasia. No reports have confirmed the occurrence of liver masses in patients with hereditary hemorrhagic telangiectasia, which is considered to be an interesting finding when investigating the mechanism of tumor development.

Keywords: Rendu-Osler-Weber disease; aneurysm; hepatic focal nodular hyperplasia; hereditary hemorrhagic telangiectasia; splenic artery; vascular malformations.

Figure 1.

Skin findings. (a) Multiple hemangiomas were observed on the back. (b) High magnification revealed a protuberant hemangioma.

Figure 2.

Abdominal ultrasound examination. (a) An anastomosis between the portal vein and vein was observed in the left lobe of the liver in B-mode. (b) An anastomosis between the portal vein and vein was also observed in Color Doppler imaging.

Figure 3.

Contrast-enhanced computed tomography (CT) and abdominal angiography at the time of referral. (a) A porto-venous shunt in the left hepatic lobe was observed in the early arterial phase (b) and portal venous phase. (c) Two aneurysms measuring up to 3 cm in size were observed in the splenic artery. (d) Similarly, the blood vessel construction image revealed two aneurysms with a maximum diameter of 3 cm. (e) Abdominal angiography revealed two aneurysms measuring up to 3 cm, and (f) the blood flow into the aneurysms disappeared after coiling surgery.

Figure 4.

Upper endoscopy. Scattered telangiectasia was observed mainly in the middle-to-lower curvature of the gastric body, and gastric mucosal findings associated with HHT were observed.

Figure 5.

Cephalic vascular catheterization examination. An aneurysm was found in the M1 region of the right middle cerebral artery. An aneurysm was identified in the left internal carotid artery (ICA).

Figure 6.

Contrast-enhanced CT and a contrast-enhanced ultrasound (US) examination 2 years after referral. A 2 cm hypovascular mass was observed in the liver S2 in the early (a) and late (b) arterial phases. (c) On contrast-enhanced US, the contrast is stained in an axle-like manner from the center in the arterial phase. (d) A central scar is observed in the post-vascular phase.