Case report: A 42-year-old man with Budd-Chiari syndrome due to a myeloproliferative neoplasm

Introduction: The Budd-Chiari syndrome is a rare disorder with the classical triad of abdominal pain, ascites and hepatomegaly. It is caused by hepatic venous outflow obstruction and results in a hepatic dysfunction.

Material and methods: A 42-year-old man presented himself in our hospital with abdominal pain caused by ascites. The patient had suffered from multiple vein thrombosis in the past and was therefore under sufficient anticoagulation with Phenprocoumon

Results: The ultrasound study showed an occlusion of the hepativ venous outflow. In a contrast enhanced ultrasound study there were no liver veins presentable. A reverse flow was detected in the portal vein. An aiming laparoscopic liver biopsy also showed multiple thromboses of the liver veins. Therefore the diagnosis of a Budd-Chiari-Syndrome was approved. Analysis of serum and bone marrow showed a JAK2-positiv myeloproliferative neoplasm. Further a beginning cirrhosis of the liver, oesophageal varices and a gastropathy due to the portal hypertension were found.

Discussion: Sufficient anticoagulation does not except the occurrence of a Budd-Chiari syndrome. Because of the threatening cirrhosis of the liver and the complications due to the portal hypertension, the diagnosis of the Budd-Chiari syndrome should be set as early as possible. A sufficient anticoagulation is absolutely necessary

Conclusion: The Budd-Chiari syndrome is a rare disorder which can be reliabley diagnosed by ultrasound studys. Further analysis and studys concerning coagulation disorders are necessary.