SIR 2006 Annual Meeting Film Panel Case: Budd-Chiari Syndrome in a Patient with Cogan Syndrome

Abstract

A 15-year-old girl presented with abdominal pain and distension. Her medical history was remarkable for bilateral lower-extremity deep vein thrombosis at the age of 10 and a transient episode of headache associated with right-hand numbness at age 12. At age 13, she experienced loss of visual acuity and a hearing deficit. Evaluation at that time with magnetic resonance (MR) imaging disclosed thinning of the optic chiasm. Fluorescein angiography showed enlargement of blood vessels and leakage of both optic nerves. Physical examination revealed hypertension (blood pressure, 138/100 mm Hg) and a normal heart rate. There was hepatosplenomegaly and abdominal distension. Engorged superficial veins were noted over the abdomen and both lower extremities. Her current medications included warfarin, prednisone, amlodipine, aspirin, and spironolactone. Findings of laboratory evaluation were as follows: hemoglobin, 10 g/dL (normal, 12–15.5 g/dL); hematocrit, 32.6% (normal, 36%-46%); prothrombin time, 36 seconds (normal, 11.5–14.5 sec); partial thromboplastin time, 38.6 seconds (normal, 24.1–34.8 sec); International Normalized Ratio, 3.3 (normal, 0.9–1.2); serum fibrinogen level, 353 mg/dL (normal, 175–433 mg/dL); alkaline phosphatase, 257 U/L (normal, 36–122 U/L); antinuclear antibody titer, 40 (normal, 40); immunoglobulin M, 413 mg/dL (normal, 39– 333 mg/dL); -1 antitrypsin, 201 mg/dL (normal, 79–207 mg/dL); cytomegalovirus antibody positive; varicella zoster antibody positive; hepatitis A, B, and C antibodies negative. Selected images from an abdominal MR imaging study, inferior vena cavogram, and transhepatic hepatic venogram are shown in Figures 1–6. In accordance with our institutional guidelines, this report does not require approval from the institutional review board.