Porto Sinusoidal Vascular Disorder: A Case Report

Naima Mukhtar Abdullahi,1,* Mohammed AM Ahmed,1 Mohamed Abdulahi Yusuf,2,3 Hawa Ali Warsame4,* 1Department of Medicine, Faculty of Medicine and Surgery, Mogadishu University, Mogadishu, Somalia; 2Department of Internal Medicine and Gastroenterology, Alami Specialist Hospital, Mogadishu, Somalia; 3Department of Internal Medicine, Somali National University, Mogadishu, Somalia; 4Department of Medicine, Faculty of Medicine and Surgery, Salaam University, Mogadishu, Somalia*These authors contributed equally to this workCorrespondence: Naima Mukhtar Abdullahi; Hawa Ali Warsame, Email [email protected]; [email protected]: 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: porto sinusoidal vascular disease, idiopathic non-cirrhotic portal hypertension, splenomegaly, pancytopenia, trans-jugular intrahepatic portosystemic shunt