Budd-Chiari Syndrome Leading to Cirrhosis in a Young Woman
Henry Ford Health System
Introduction: A young woman with a past medical history of hepatic steatosis and polycystic ovarian syndrome on oral contraceptive pills presents with ascites and diffuse hepatocellular disease of unk..more »
Introduction: A young woman with a past medical history of hepatic steatosis and polycystic ovarian syndrome on oral contraceptive pills presents with ascites and diffuse hepatocellular disease of unknown etiology. Advanced liver disease is rather unusual in young adults because cirrhosis typically develops gradually after years of persistent liver injury. Cirrhosis is commonly caused by alcohol abuse, viral hepatitis, or nonalcoholic fatty liver disease. Other potential etiologies include autoimmune, iatrogenic, veno-occlusive, and genetic disorders such as Wilson disease or hemochromatosis. This case report suggests that veno-occlusive conditions such as Budd-Chiari syndrome should be considered in young adults presenting with cirrhosis, especially those with hypercoagulable risk factors and lack of other contributors such as alcohol use and viral hepatitis. Cirrhosis is rare in this age group, but hypercoagulable states may manifest in this manner.
The Case: The patient initially presented to an outside hospital for right upper quadrant pain, nausea, and vomiting. Imaging of the gallbladder revealed a thickened wall and a decreased gallbladder ejection fraction. For this reason, a laparoscopic cholecystectomy was performed. During the procedure, the liver appeared cirrhotic and ascitic fluid was present. The team drained two liters of fluid and biopsied the liver. A few days later, the patient noted continuous drainage from the port sites and crampy epigastric pain. A week later, her heart rate was 144 and she was readmitted to the hospital at this time. At the hospital, a CT scan showed diffuse hepatocellular disease with mesenteric lymphadenopathy. Two more paracenteses were done, draining four and three and a half liters respectively. The serum-to-ascitic fluid gradient was greater than 1.1 g/dl, indicating portal hypertension. She drinks one alcoholic beverage per week and viral hepatitis serologies were negative. Despite several other tests, no explanation for the advanced liver disease was discovered, and she was transferred to our hospital for escalation of care. On admission, another CT of the liver was done, which found Budd-Chiari syndrome and bilateral lower lobe pulmonary embolisms. Interventional radiology was consulted, and they successfully performed a direct intrahepatic portocaval shunt (DIPS) procedure. She was taken off of oral contraceptive pills and started on apixaban.
Discussion and Conclusion: Budd-Chiari syndrome is uncommon, but when it is diagnosed, the patients are usually hypercoagulable. In primary Budd-Chiari syndrome, a thrombus forms in the hepatic veins, preventing blood from leaving the liver. This patient was likely in a hypercoagulable state secondary to her oral contraceptive use. Treatment of Budd-Chiari syndrome includes addressing the underlying cause, starting anticoagulation, and treating portal hypertension if present. In young patients presenting with a cirrhotic picture, Budd-Chiari syndrome is an important diagnosis to consider in those with hypercoagulable risk factors. Although they may have other risk factors for liver disease, it is unlikely that these conditions would progress rapidly to advanced disease by young adulthood.
Wayne State University
WSU Medical School
Henry Ford Health System