Abdullahi Naima Mukhtar, Ahmed Mohammed A M, Yusuf Mohamed Abdulahi, Warsame Hawa Ali
Department of Medicine, Faculty of Medicine and Surgery, Mogadishu University, Mogadishu, Somalia.
Department of Internal Medicine and Gastroenterology, Alami Specialist Hospital, Mogadishu, Somalia.
Int Med Case Rep J. 2025 Mar 1;18:289-293. doi: 10.2147/IMCRJ.S502206. eCollection 2025.
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.
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.
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.
门静脉窦状隙血管疾病（PSVD）是一种罕见的血管疾病，其特征为门静脉压力升高，但无肝硬化或其他常见的肝脏病理原因。该疾病的发病机制尚不清楚。
我们报告一例罕见的门静脉窦状隙血管疾病（PSVD）。该患者多次出现呕血和黑便，尽管既往无慢性疾病史。内镜检查发现4条食管静脉曲张。尽管患者接受了MRI、肝活检、腹部和肝脏超声检查以及其他必要的实验室检查，但仍未确定门静脉高压的确切原因。该患者接受了套扎治疗，并接受了β受体阻滞剂、质子泵抑制剂和全血输血治疗。
该病例突出了PSVD诊断和管理的复杂性。尽管患者接受了对症治疗，但由于脾肿大仍有全血细胞减少，需要密切监测和随访。
