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


A non-inflammatory, non-atherosclerotic vascular lesion is fibromuscular dysplasia (FMD). FMD is thought to have multiple causes, such as female hormones, long-term mechanical stress, and tobacco use. Splenic artery aneurysms (SAA) have a rare cause. An 18-year-old woman with hematemesis, malaena, pancytopenia, and splenomegaly. On CT angiography, a splenic infarct and a sizable splenic artery aneurysm were found. The portal vein underwent cavernous metamorphosis with increasing periportal and lienorenal collaterals. The SAA had medial dysplasia and intimal fibroplasia, which could be seen under a microscope. Extrahepatic portal hypertension’s symptoms were reduced by aneurysmectomy, which proved SAA to be the underlying cause. This is the first study to establish a connection between hypersplenism, extrahepatic portal hypertension, fibromuscular dysplasia, and splenic artery aneurysm.

Author(s) Details:

Sweety Shinde,
B. Y. L Nair Hospital, Maharashtra, India.

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Keywords: Fibromuscular dysplasia, splenic aneurysm, hypersplenism, non-cirrhotic portal hypertension

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