HEPATIC VASCULAR DISORDER INTERVENTIONS

WHAT IS BUDD-CHIARI SYNDROME?

Budd-Chiari syndrome is a condition in which the hepatic veins (veins that drain the liver) are blocked or narrowed by a clot (mass of blood cells). This blockage causes blood to back up into the liver, and as a result, the liver grows larger. The spleen (an organ located on the upper left side of the abdomen that helps fight infection by filtering the blood) may also grow larger.

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.

WHAT CAUSES BUDD-CHIARI SYNDROME?

Budd-Chiari syndrome can be caused by conditions and situations that cause your blood to clot (form a blockage). These include:

• Myeloproliferative diseases (those that affect the blood and bone marrow), including polycythemia (the body makes too many red blood cells), and thrombocythemia (the body produces too many platelets).
• Sickle cell disease (a blood disease in which red blood cells change shape from round to sickle-shaped).
• Inflammatory bowel disease (a group of disorders that cause irritation and swelling of the digestive tract).
• Pregnancy

WHAT ARE THE SYMPTOMS OF BUDD-CHIARI SYNDROME?

The symptoms of Budd-Chiari syndrome include:

• Pain in the upper abdomen.
• Ascites (swelling in the abdomen caused by excess fluid).
• Jaundice (skin, whites of the eyes and mucous membranes turn yellow).
• Enlarged and tender liver.
• Bleeding in the esophagus.
• Edema (swelling) in the legs.
• Liver failure.
• Hepatic encephalopathy (reduced brain functioning caused by liver disease).
• Vomiting.
• Enlarged spleen.
• Fatigue (extreme tiredness).

HOW IS BUDD-CHIARI SYNDROME TREATED?

Treatments for Budd-Chiari syndrome are designed to dissolve blood clots and to help improve blood flow in the liver. Treatments are usually drug therapy, non-surgical procedures, and surgery:

• Drug therapy: Your doctor will prescribe drugs to dissolve the blood clots. In addition, the blood-thinning drug warfarin (Coumadin®) is often prescribed to prevent future clots.
• Procedures: There are non-surgical procedures used in treating Budd-Chiari syndrome:
• Hepatic Vein Stenting
• IVC stenting
• Direct Intra hepatic Portosystemic Shunt

In the procedure of IVC STENTING OR HEPATIC VEIN STENTING, the doctor inserts a catheter (a thin, hollow tube with a balloon at the tip) through the skin and into a blood vessel. The catheter is guided to the area where the clot is located. When the catheter reaches the clot, the balloon is inflated to widen the vein. A stent may be placed at the site to keep the vein open.

The Direct intrahepatic portosystemic shunt (DIPS) is a radiologic procedure in which a stent (a tubular device) is placed in the middle of the liver to reroute the blood flow. During the procedure, a radiologist makes a tunnel through the liver with a needle, connecting the portal vein (the vein that carries blood from the digestive organs to the liver) to one of the hepatic veins (the three veins that carry blood from the liver). A metal stent (a small, hollow tube) is placed in this tunnel to keep the track open. The TIPS procedure reroutes blood flow in the liver and reduces pressure in all abnormal veins, including the bowel and the liver.