Fibromuscular Dysplasia, Giant Splenic Artery Aneurysm, Extrahepatic Portal Hypertension and Hypersplenism: A Rare Quartet

Fibromuscular dysplasia (FMD) is a non-inflammatory, non-atherosclerotic vascular lesion. FMD is presumed to have multifactorial origins including female hormones, chronic mechanical stress and tobacco smoking. The etiology of splenic artery aneurysm (SAA) is quite uncommon. Hematemesis, malaena, pancytopenia, and splenomegaly were found in an eighteen-year-old girl. A splenic infarct and a large splenic artery aneurysm were discovered on CT angiography. With increased periportal and lienorenal collaterals, the portal vein had cavernous metamorphosis. The SAA was microscopically shown to have intimal fibroplasia and medial dysplasia. Aneurysmectomy alleviated the symptoms of extrahepatic portal hypertension, demonstrating SAA to be the underlying cause. This is the first study to link fibromuscular dysplasia, splenic artery aneurysm, extrahepatic portal hypertension and hypersplenism.