Indian Journal of Critical Care Medicine

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VOLUME 25 , ISSUE 1 ( January, 2021 ) > List of Articles

CASE REPORT

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

Sweety Shinde

Keywords : Extrahepatic portal hypertension, Fibromuscular dysplasia, Hypersplenism, Non-cirrhotic portal hypertension, Pancytopenia, Portal cavernoma, Splenic artery aneurysm

Citation Information : Shinde S. A Rare Quadruple Association: Fibromuscular Dysplasia, Giant Splenic Artery Aneurysm, Extrahepatic Portal Hypertension, and Hypersplenism. Indian J Crit Care Med 2021; 25 (1):100-103.

DOI: 10.5005/jp-journals-10071-23710

License: CC BY-NC 4.0

Published Online: 18-01-2021

Copyright Statement:  Copyright © 2021; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular lesion. It is a very rare cause of splenic artery aneurysm (SAA). An 18-year-old girl presented with hematemesis, melena, pancytopenia, and splenomegaly. Endoscopy showed esophageal varices. Computed tomography angiography showed splenic infarct and a giant splenic artery aneurysm. Portal vein showed cavernous transformation with enlarged periportal and lienorenal collaterals. The liver and pancreas were unremarkable. Microscopy of the SAA revealed intimal fibroplasia and medial dysplasia. Symptoms of extrahepatic portal hypertension were relieved by aneurysmectomy, thus proving SAA as the underlying cause. Pancytopenia was reversed post-splenectomy, thus proving hypersplenism. This is the first-ever report showing a quadruple association of FMD, splenic artery aneurysm, extrahepatic portal hypertension, and hypersplenism. Key messages: Fibromuscular dysplasia can present as a giant aneurysm of the splenic artery. The resultant extrahepatic portal hypertension and splenomegaly can result in hypersplenism. Splenectomy and aneurysmectomy can reverse pancytopenia and portal hypertension.


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