Cases from the Osler Medical Service at Johns Hopkins University

Presenting features

A 66-year-old man with a history of alcohol-induced cirrhosis was admitted to the Johns Hopkins Hospital with the chief complaints of jaundice and lower extremity swelling. Three weeks before admission, he first noticed gradually worsening jaundice and leg swelling, as well as abdominal distension, fatigue, and anorexia. He denied nausea, vomiting, fevers, night sweats, diarrhea, abdominal pain, hematemesis, hematochezia, and melena.His past medical history was remarkable only for the diagnosis of cirrhosis 8 years before admission, when he had presented with a bleeding esophageal varix. At that time, he decided to abstain from alcohol permanently but to continue smoking cigarettes. His medications at the time of the current admission included spironolactone, megestrol acetate, furosemide, propranolol, pantoprazole, and metoclopramide.On physical examination, he appeared markedly jaundiced and fatigued. He had both scleral and sublingual icterus as well as multiple facial telangiectasias and a right epicanthal xanthoma. His heart and lung examinations were unremarkable. His abdomen was distended but soft and nontender with no organomegaly. He had a palpable fluid wave, shifting dullness, and caput medusae, but no spider angiomata or gynecomastia. His lower extremities showed 2+ pitting edema extending to the lower abdomen. There was no palmar erythema, asterixis, or evidence of hepatic encephalopathy.Initial laboratory studies revealed the following: serum sodium, 130 mEq/L; chloride, 93 mEq/L; ammonia, 97.1 μg/dL; aspartate aminotransferase, 250 U/L; alanine aminotransferase, 102 U/L; alkaline phosphatase, 672 U/L; albumin, 2.8 g/dL; and total bilirubin, 23.6 mg/dL, with a direct fraction of 17.2 mg/dL. The hematocrit was 36.4% with a normal mean corpuscular volume and normal coagulation times. Paracentesis revealed transudative fluid with 303 white blood cells (67% neutrophils), a lactate dehydrogenase level of 93 U/L, a total protein level of 1.4 g/dL, and an albumin level of 0.7 g/dL (serum ascites to albumin gradient, 2.1 g/dL). Hepatitis serologies were negative and the serum alfa-fetoprotein level was 52 ng/mL.A right upper quadrant ultrasound showed ascites around a shrunken, nodular liver with a coarse, heterogeneous echo texture, as well as echogenic material in the region of the portal vein with no portal blood flow. A computed tomographic (CT) scan of the abdomen with three-dimensional reconstruction following the administration of intravenous contrast material (Figures 1 and 2) revealed innumerable indeterminate hypodense lesions measuring 1 cm or less scattered diffusely throughout the liver. In addition, the portal vein was markedly dilated with evidence of thrombus extending both intrahepatically and extrahepatically to the portal confluence.What is the diagnosis?