Budd-Chiari syndrome

Background

Posterior abdominal wall, after removal of the peritoneum, showing kidneys, suprarenal capsules, and great vessels. (Hepatic veins labeled at center top.)

• Rare
• Caused by occlusion of hepatic veins, 75% primary (thrombosis), 25% secondary (compression by mass)
• Fulminant, acute, chronic, or asymptomatic.

Clinical Features

Classic triad:

1. Abdominal pain
2. Ascites
3. Hepatomegaly

Differential Diagnosis

RUQ Pain

• Gallbladder disease
  • Symptomatic cholelithiasis (biliary colic)
  • Choledocholithiasis
  • Acute cholecystitis
  • Ascending cholangitis
  • Acalculous cholecystitis
• Pancreatitis
• Acute hepatitis
• Pancreatitis
• GERD
• Appendicitis (retrocecal)
• Pyogenic liver abscess
• Bowel obstruction
• Cirrhosis
• Budd-Chiari syndrome
• GU
  • Pyelonephritis
  • Kidney stone
  • Fitz-Hugh-Curtis Syndrome
• Other
  • Hepatomegaly due to CHF
  • Peptic ulcer disease with or without perforation
  • Pneumonia
  • Herpes zoster
  • Myocardial ischemia
  • Pulmonary embolism
  • Abdominal aortic aneurysm

Evaluation

Budd–Chiari syndrome secondary to cancer, note clot in the inferior vena cava and the metastasis in the liver.

• LFTs, BMP, LDH
• Ultrasound, retrograde angiography
• CT and MRI less sensitive

Management

• Sodium restriction, diuretics, anticoagulants
• Venous shunts or TIPS
• Liver transplant

See Also

• Portal vein thrombosis