Krutika Lakhoo, MD1, Grace Guzman, MD2, Adam Mikolajczyk, MD2 1University of Illinois, Chicago, IL; 2University of Illinois Medical Center, Chicago, IL
Introduction: Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction at the hepatic venules, hepatic veins or IVC that causes right-upper quadrant (RUQ) abdominal pain, hepatomegaly and ascites. It can present with a wide spectrum of imaging findings. We present a novel case of metastatic ocular melanoma that mimicked several clinical and radiographic features of BCS
Case Description/Methods: 78-year-old man with a history of uveal melanoma treated with radiation therapy 8 years ago presented with abdominal pain, distention and jaundice. He had no history of liver disease, new medications or heavy alcohol use. Physical examination showed jaundice, RUQ abdominal pain and abdominal distention with bulging flanks. Labs showed elevations in total bilirubin (7.8 mg/dL), alkaline phosphatase (131 U/L), alanine aminotransferase (158 U/L), aspartate aminotransferase (272 U/L) and prothrombin time (15.6 seconds). Doppler ultrasound showed hepatomegaly, ascites and no blood flow in the right hepatic vein. MRI of the abdomen revealed hepatomegaly with heterogeneous enhancement of the liver parenchyma, several hyper-enhancing hepatic nodules and evidence of thrombosis in the posterior right lobe hepatic veins (Figure 1). Given a high suspicion for BCS based on this, venography was done, showing nonocclusive thrombus in the peripheral branches of the right hepatic vein and patent central and left hepatic veins. The calculated sinusoidal pressure gradient was 12 mmHg. Liver biopsy was done and showed an effaced liver architecture from multi-nodular malignant pigmented cell infiltrates preferentially distributed in the centrilobular sinusoids compressing adjacent hepatocytes, and displaying histopathological features indicative of metastatic melanoma (Figure 1). The patient had progressive liver failure and was discharged to hospice care
Discussion: Imaging findings and clinical presentation are often needed to definitively diagnose BCS. Typical imaging findings include occlusion of the hepatic veins and IVC, caudate lobe hypertrophy, inhomogenous liver enhancement and intrahepatic collateral vessels and hypervascular nodules. But, as in this case, other causes, like invasive intrahepatic malignancy, can present with similar radiographic findings and venography may be needed to definitively diagnose BCS. Uveal melanoma, albeit rare, commonly metastasizes to the sinusoids in the liver and can mimic clinical and radiographic features of BCS, especially when the metastases preference the centrilobular sinusoids
Figure: Figure 1: Top left panel shows diffuse enhancement of liver parenchyma and multiple enhancing liver lesions; Top right panel suggestive of posterior right hepatic vein occlusion (arrow). Bottom panel shows nests of malignant tumor cells infiltrating the centrilobular sinusoids between hepatocytes.
Disclosures:
Krutika Lakhoo indicated no relevant financial relationships.
Grace Guzman indicated no relevant financial relationships.
Adam Mikolajczyk indicated no relevant financial relationships.
Krutika Lakhoo, MD1, Grace Guzman, MD2, Adam Mikolajczyk, MD2. C0594 - Metastatic Ocular Melanoma Mimicking Budd-Chiari Syndrome, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.