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Case Reference No. CC-0498-09 A 22-year-old woman presented to the hospital with a 2-week history of epigastric pain. She had a prior history of hypothyroidism and a vague history of mild ascites 1 year earlier. The ascites had resolved on diuretic therapy. There was no history of blood dyscrasia or autoimmune disease. The patient did not take oral contraceptive agents. Physical examination was remarkable for splenomegaly and mild right upper quadrant tenderness. Laboratory evaluation was remarkable for a prolonged prothrombin time of 16.0 seconds (normal 10.412.8 seconds) and a prolonged partial thromboplastin time of 86 seconds (normal 2839 seconds). In addition, she had a mild anemia (hemoglobin 10.5 g/dL) and was thrombocytopenic (platelets 116,000). Serum total bilirubin was slightly elevated at 1.3 mg/dL (normal 0.11.2 mg/dL). All additional liver function tests were within normal limits. An ultrasound examination revealed heterogeneity of the hepatic parenchyma, enlargement of the caudate lobe, splenomegaly, minimal pelvic ascites, and nonopacification of the hepatic veins. A magnetic resonance imaging study confirmed occlusion of the central hepatic veins (Fig. 1). The patient was referred to us for venography. An inferior vena cavogram performed from the right jugular approach showed mild compression of the intrahepatic cava by an enlarged caudate lobe with no reflux into the hepatic venous system (Fig. 2). There was no evidence of a web at the atriocaval junction. Attempts at selective catheterization of the hepatic venous system were unsuccessful despite considerable probing of the hepatic sinus with guide wires and catheters. A transjugular biopsy was then obtained from the anticipated region of the right hepatic vein entry. Following this, percutaneous transhepatic venography was performed in an attempt to delineate the obstructed hepatic venous system (Fig. 3). This demonstrated a number of portal vein branches as well as collateral veins, but failed to demonstrate the hepatic venous system. The biopsy revealed areas of focal fibrosis with centrolobular prominence, sinusoidal dilatation, and hepatocyte atrophy, consistent with chronic hepatic venous outflow obstruction. Additional laboratory evaluation revealed the presence of a lupus anticoagulant with reduced activity of factors II, IV, VII, and X. |
Fig. 1. Axial T1-weighted gadolinium-enhanced MR image reveals no patent central hepatic veins. Heterogeneous enhancement of the hepatic parenchyma is evident.
Fig. 2. Transjugular inferior vena cavogram depicts mild compression of the intrahepatic cava by the caudate lobe. No reflux into patent hepatic veins is seen, nor is there washout of contrast material within the hepatic sinus from nonopacified blood.
Fig. 3. Percutaneous transhepatic venogram opacifies portal veins as well as additional veins extending into a rounded structure (arrow) adjacent to the porta hepatis. The anatomic origin of this structure is unknown. No hepatic veins were visualized despite multiple passes. | |
Questions: 1) Is any therapy other than anticoagulation indicated at this time? 2) If the decision is to attempt a therapeutic intervention, should it be TIPS or a surgical shunt? 3) Are there any additional diagnostic studies that should be performed? Related Case: | ||
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