Nutmeg liver and ascites resulting from a membranous web obstruction at the hepatic origin of the inferior vena cava
Authors: Christopher P. Farrell, Gary Newman, Robert Atkins
Journal: Hepatology (2012)

A 30-year-old female presented to the hospital for an evaluation of anemia and intermittent abdominal distention. Laboratory studies revealed a profound iron-deficiency anemia with a hemoglobin level of 6.8, mean cell volume of 62, and a ferritin value of 3. BCS, Budd-Chiari syndrome; CAT, computerized axial tomography; IR, interventional radiology; IVC, inferior vena cava; PH, portal hypertension; US, ultrasound. On physical exam, her temperature was 97.9°F, pulse was 82, respiratory rate was 17, and blood pressure was 94/70. She was found to have hepatomegaly and moderate ascites on abdominal exam and was heme negative on rectal exam. An esophagogastroduodenoscopy and colonoscopy were unremarkable for a source of blood loss, and small bowel biopsies were not consistent with celiac sprue. A right upper quadrant ultrasound (US) revealed moderate ascites and hepatosplenomegaly. The liver had a lobular contour to it with increased echogenicity. A subsequent computerized axial tomography (CAT) scan illustrated a "nutmeg liver" and ascites (Fig. 1). A repeat US with Doppler showed patent hepatic and portal veins with normal direction of flow. Her liver function studies were all within normal limits, along with hepatitis serologies, antinuclear antibody, and Epstein-Barr virus titers. The ascites was sampled, revealing a serum ascites albumin gradient of >1.1 and a protein level of <2.5, consistent with portal hypertension (PH). Hepatic venous pressures were attempted by interventional radiology (IR). The pressure in the intrahepatic portion of the inferior vena cava (IVC) was elevated to 14 mmHg, and the right heart pressure was normal at 4 mmHg, yielding a 10-mmHg venous pressure gradient between the supra- and intrahepatic portion of the IVC. Attempts were made to cannulate the hepatic veins to obtain free hepatic pressures; however, these were unsuccessful because of a narrowing in the intrahepatic portion of the IVC. This prompted a third US in IR, demonstrating a web and turbulent flow at the origin of the hepatic vein from the IVC. IR performed a transhepatic venogram, and the web was found at the confluence of the middle hepatic vein and the IVC. The initial pressure gradient between the middle hepatic vein and the right atrium was significantly elevated at 20 mmHg. IR executed a successful angioplasty of the web with a 12 mm × 4 cm balloon, and the pressure gradient dropped to 4 mmHg (Fig. 2). The patient recovered nicely, with resolution of her ascites and symptoms. A 1-month follow-up US illustrated no residual stenosis (Fig. 3). A CAT scan 5 months later showed a dramatic improvement in the appearance of her liver (Fig. 4). As for her anemia, it was believed to be secondary to her menses and corrected nicely with iron supplementation. Budd-Chiari syndrome (BCS) occurs as a result of PH from hepatic venous outflow obstruction, usually from a hepatic vein thrombosis. Membranous webs can also rarely form in the hepatic venous system, eliciting the same organic response. Venous thrombosis can require pharmacologic thrombolysis and even liver transplantation. However, BCS resulting from membranous web obstruction can be successfully treated with angioplasty and, sometimes, with stenting.