Budd Chiari Syndrome Treatment in Jacksonville, FL
Also called hepatic (liver) vein thrombosis (clotting), Budd Chiari syndrome is a rare blockage of a liver vein usually due to clotting or overgrowth of fibrous tissue in the veins. Other identified causes may be exposure to radiation, arsenic, trauma, blood poisoning (sepsis), vinyl chloride monomer, cancer, or some cancer chemotherapy drugs, as well as birth control pills. In some cases it has been caused by Pyrrolizidine plant alkaloids (bush tea). Often the exact cause is unknown.
While hepatic vein thrombosis can have widespread complications —including an enlarged liver, abdominal fluid, and varicose esophageal veins—a variety of treatments can address the blockage, and its complications while preventing further damage to your liver . The natural course of the disease is very severe (less than 10% of patients survive for more than 3 years without treatment.
There are many tests that help explain abdominal symptoms and develop a treatment plan. To find a Jacksonville Budd Chiari syndrome specialist, call (770) 674-6311 or contact Dr. Gail Ravello online today.
What are the symptoms of Budd Chiari syndrome?
The blockage and symptoms of Budd Chiari syndrome can develop either over a few weeks or months or much more quickly. Sudden blockage and symptoms are more common in pregnant patients.
The severity of symptoms will vary depending on where the blockage is and the number of veins that are affected. These symptoms include:
- fluid in the abdomen (ascites)
- enlarged liver (hepatomegaly)
- enlarged spleen
- abdominal pain
- fluid that causes swelling (edema) in the legs
- enlarged and twisted (varicose) esophageal veins
- kidney disease
Ascites may cause symptoms like nausea or vomiting, loss of appetite, shortness of breath, and fever. Edema is the accumulation of fluid in the interstitium, located beneath the skin and in the cavities of the body, which can cause severe pain. Esophageal varices can bleed, and cause lightheadedness, black or bloody stools, and shock due to blood loss. If you start regurgitating blood, seek emergency medical attention.
If left untreated, hepatic vein thrombosis can cause cirrhosis , jaundice , and liver failure.
What causes Budd Chiari syndrome?
The formation of a blood clot in a liver vein can cause blood to accumulate in the liver, which begins to swell up, along with surrounding organs like the spleen. Fluid may also build up in the abdomen.
Increased blood pressure in the portal vein (portal hypertension) can lead to varicose esophageal veins and ascites.
In most cases, the exact cause of hepatic vein thrombosis is unknown, though it’s often a result of these blood clotting disorders:
- antiphospholipid syndrome: the immune system attacks your blood proteins
- polycythemia vera: the overproduction of red blood cells
- sickle cell disease: this causes red blood cells to be misshapen
- paroxysmal nocturnal hemoglobinuria: this rare bone marrow disorder causes blood cell death and increased clotting
Other causes of Budd-Chiari syndrome include:
- inflammatory disorders like inflammatory bowel disease and lupus
- vein inflammation (phlebitis)
- connective tissue disorders
- liver injuries
- parasites pressing against hepatic veins
- liver or kidney tumors, as these can block blood vessels
Birth control pills3 can also cause hepatic vein thrombosis. Although rare, pregnancy1 can increase your risk of hepatic vein thrombosis. This is because your blood will clot more easily in order to protect against blood loss during labor. Pregnancy also carries the risk of postpartum sepsis.2
How is Budd Chiari syndrome diagnosed?
Besides observing symptoms, and gently examining the abdomen for an enlarged liver or abdominal fluid, these tests can help diagnose Budd-Chiari syndrome:
- ultrasound : detects liver inflammation and damage
- angiography: radiographic dye injected into your veins so they appear clearly on an x-ray
- magnetic resonance angiography: imaging of blood vessels
- CT scan: detects liver damage and blocked veins
- liver function test: monitors liver function by measuring liver-produced enzymes in the blood
- endoscopy: to identify varicose esophageal veins, collect abdominal fluid, and confirm presence of ascites
- liver biopsy: to confirm a diagnosis and detect cirrhosis
- hepatic vein catheterization: to measure pressure with a catheter
Budd Chiari syndrome treatments
Budd Chiari syndrome treatment is most effective if diagnosed early. In fact, the five-year survival rate of hepatic vein thrombosis has increased from 80%-90% due to increasing therapeutic options that have been developed over the last few decades.4
As with any medical procedure, results of Budd Chiari syndrome treatment will vary from patient to patient depending on age, genetics, general health, condition severity, follow-up care, and environmental factors. The following pharmaceutical, regenerative, nutritional, and herbal treatments may present contraindications with one another, and/or with other medical conditions. Always consult your healthcare professional before deciding which treatment to try first.
Medication for Budd Chiari syndrome
There are several medications available to treat Budd Chiari syndrome. These include:
- prednisone: reduces inflammation; side effects include insomnia, night sweats, increased appetite, and headaches
- fibrinolytic drugs: urokinase and streptokinase1 dissolve blood clots immediately; side effects include internal bleeding and low blood pressure
- anticoagulants: warfarin and heparin prevent blood clots and are used in long-term treatment; side effects include severe bleeding and headaches, and easy bruising or skin discoloration
Most patients will need to take anticoagulant drugs for the rest of their lives. However, anticoagulation medication is only effective in about 10% of patients, especially in those with mild cases.4
Surgical procedures for Budd Chiari syndrome
There are several ways to treat the narrowed or blocked vein with surgery.
percutaneous transluminal angioplasty
A catheter is inserted into the obstructed vein then inflated to widen it. A stent can also be inserted into to keep it open. Angioplasty has shown to be very successful for patients with blockages in their inferior vena cava; out of 453 patients reported, there was a success rate in the 95% range with an almost instantaneous improvement in symptoms and liver function.4 While severe complications are rare, there is a possibility of the stent moving towards the right atrium.4
transjugular intrahepatic portosystemic shunting (TIPS)
This procedure can relieve pressure on the portal vein by creating an alternative route for the blood. This connection is created between the hepatic vein and the portal vein with a stunt, allowing the blood to avoid the liver altogether. The success rate of a TIPS procedure is about 80%.4 Complications include blood vessels rupturing and bleeding (hemorrhage), the gallbladder being punctured by breaching the connective tissue that covers the liver (Glisson's capsule), brain disease (encephalopathy), and the shunt migration or blockage.5
A transplant of a healthy donor liver (orthotopic liver transplantation4) is required in 10-20% of patients whose liver deterioration progresses despite other treatments. The reported five-year survival rate is between 71-89.4%.4 Visit our liver transplant page to learn more about the potential side effects of the procedure.
Budd Chiari syndrome complications
Ascites is the excessive build-up of fluid in the abdominal cavity which can limit a patient's ability to eat, walk and perform daily activities. In addition to preventing the progression of the vein clot and addressing its cause, managing the ascites is imperative, as salt causes fluid to accumulate.4
The way to treat ascites is to drastically reduce your salt intake to 2,000 mg or less a day. Seeing a nutritionist is helpful, as salt content in foods is difficult to determine. Salt substitutes – that do not contain potassium -- can be also used.
Foods to avoid include:
- salted nuts
- salted meats
- dairy products
- pickled vegetables
- canned foods
- frozen foods
Foods to eat include:
- fresh meat
- dairies like milk and yogurt
- dried fruit
- fresh vegetables
Often, patients will require diuretics ("water pills") to treat ascites. These water pills can cause problems with your electrolytes (sodium, potassium) and kidney function (creatinine). Taking water pills is not a substitute for reducing your salt intake. Both are needed to treat ascites. Common diuretics are:
- furosemide: this diuretic removes excess water or fluid from your body via the kidneys; side effects include vomiting, diarrhea, lightheadedness, and blurred vision
- spironolactone: this diuretic performs the same function as furosemide; side effects include vomiting, diarrhea, dry mouth, thirst, dizziness, and sexual dysfunction
If these medications do not remove the fluid, the patient may require needle syringe removal (paracentesis). Side effects include discomfort in the needle insertion area, dizziness, and infection.
Bleeding esophageal varices
Medications for bleeding esophageal varices include:
- propranolol: this beta blocker can lower blood pressure to treat bleeding esophageal varices; side effects include slow heart rate, dizziness, nausea, and fatigue
- octreotide: this synthetic hormone is injected via an endoscope to restrict the blood vessels of the esophagus to reduce pressure and bleeding; side effects include nausea and constipation
- surgical extraction of a tumor affecting the hepatic veins; side effects include pain, swelling, bleeding, and infection
- endoscopic variceal ligation (tying off bleeding esophageal veins with temporary rubber bands); side effects include blood or fluid blocking the esophagus, difficulty swallowing, chest pain, and a tear in the esophagus
Request your appointment now
Although rare, Budd Chiari syndrome can affect your liver and body in numerous ways. Medication and surgery can help to reverse these symptoms and prevent further damage. Maintenance of complications is imperative. To find hepatic vein thrombosis treatment in Jacksonville, call (770) 674-6311 or contact Dr. Gail Ravello online.
- Zhang, Qingqiao, et al. “Catheter-Directed Thrombolytic Therapy Combined with Angioplasty for Hepatic Vein Obstruction in Budd-Chiari Syndrome Complicated by Thrombosis.” Experimental and Therapeutic Medicine, vol. 6, no. 4, 30 July 2013, pp. 1015–1021., doi:10.3892/etm.2013.1239.
- Wang, Zhonggao, et al. “Recognition and Management of Budd-Chiari Syndrome: Report of One Hundred Cases.” Journal of Vascular Surgery, vol. 10, no. 2, 1989, pp. 149–156., doi:10.1016/0741-5214(89)90348-0.
- Lewis, James H., et al. “Budd-Chiari Syndrome Associated with Oral Contraceptive Steroids.” Digestive Diseases and Sciences, vol. 28, no. 8, Aug. 1983, pp. 673–683., doi:10.1007/bf01312555.
- Martens, Pieter, and Frederik Nevens. “Budd-Chiari Syndrome.” United European Gastroenterology Journal, vol. 3, no. 6, 2015, pp. 489–500., doi:10.1177/2050640615582293.
- Suhocki, Paul, et al. “Transjugular Intrahepatic Portosystemic Shunt Complications: Prevention and Management.” Seminars in Interventional Radiology, vol. 32, no. 02, 28 June 2015, pp. 123–132., doi:10.1055/s-0035-1549376.
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