INTRODUCTION
Its an uncommon condition which occurs from occlusion of hepatic vein or inferior vena cava . it may be acute but usually is chronic.
Budd-Chiari Syndrome may also cause other conditions, including:
- Portal hypertension (increased pressure in the portal vein, which carries blood from the intestines to the liver).
- Esophageal varices (twisted veins in the esophagus, or “food tube”).
- Ascites (a buildup of fluid in the abdomen).
- Cirrhosis (scarring of the liver).
- Varicose veins (abnormal, swollen blood vessels) in the abdomen and/or rectum.
CAUSES
- Myeloproliferative diseases such as polycythemia and thrombocythemia.
- pregnancy
- protein c or S deficiency
- oral contraceptives
- tumours
- congenital venous webs
- trauma
- radiotherapy
- Sickle cell disease
- Inflammatory bowel diseases
CLINICAL FEATURES
- Massive splenomegaly
- Intractable transudative ascites
- Jaundice
- Pain in the upper abdomen
- enlarged and tender liver
- bleeding in the esophagus
- hepatic encephalopathy
- vomiting
- liver failure
- fatigue
DIAGNOSIS
- Doppler ultrasound – It demonstrates the obstruction of hepatic vein with reverse flow.
- CT/MRI – They show enlargement of caudate lobe.
- Liver biopsy – this confirms the diagnosis of cirrhosis.
TREATMENT
- Drug therapy -blood-thinning drug warfarin (Coumadin®) is often prescribed to prevent future clots. When recent thromobolysis is suspected thrombolytic therapy followed by low molecular heparin therapy may be useful.
- Non surgical procedures – ascites is managed with transjugular intrahepatic portosystemic shunt(TIPS) and percutaneous transluminal angioplasty
- Surgery – If you have liver failure (the liver no longer functions adequately), a liver transplant is the usual treatment.
Dentowesome 