BUDD-CHIARI SYNDROME

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.

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