Porto Sinusoidal Vascular Disorder: A Case Report

Abstract

Background: Porto sinusoidal vascular disease (PSVD) is an unusual vascular disorder defined by raised portal venous pressure without cirrhosis or other common causes of liver pathology. The disease pathogenicity is not well understood.

Case report: We report a rare case of Porto Sinusoidal Vascular Disorder (PSVD). This patient presented with hematemesis and melena several times, despite having no prior history of chronic disease. An endoscopic evaluation revealed 4 cords of oesophageal varices. The exact cause of portal hypertension was not identified despite the patient undergoing MRI, liver biopsy, abdominal and hepatic sonography, and other required laboratory tests. This patient was performed band ligation and received beta blockers, proton pump inhibitors and whole blood transfusion.

Conclusion: This case highlights the complexity of the diagnosing and managing of PSVD. Despite the patient receiving symptomatic treatment, he still has pancytopenia due to splenomegaly and needs close monitoring and flow-up.

Keywords: idiopathic non-cirrhotic portal hypertension; pancytopenia; porto sinusoidal vascular disease; splenomegaly; trans-jugular intrahepatic portosystemic shunt.

Figure 1

High-field MRI, the right hepatic lobe is less than 10 cm in size, indicating chronic liver parenchymal abnormalities. There is no delayed washout or restricted diffusion in the posterior region of the right lobe (segments 7–8), which shows a weak bright signal on T1-weighted pre-contrast images and faint heterogeneous enhancement in the early dynamic phases. There are two contiguous subcapsular lesions in the left hepatic lobe (segment 2), the largest of which is 6.5 mm in size. On T2-weighted pictures, these lesions exhibit a weak, bright signal that gets stronger throughout the course of the dynamic study without showing any signs of washout.