BUDD CHIARI SYNDROME :

It is a condition in which the hepatic venous outflow of the blood is obstructed due to thrombus formation.

It is a serious condition and needs urgent diagnosis and treatment to prevent it from getting worse and to improve the prognosis of the patient.

 

 

CLASSIFICATION :

1) PRIMARY BUDD CHIARI SYNDROME – This syndrome is termed as primary if the cause or risk factor for obstruction is from the venous system.

CAUSES-

A ) PRIMARY BUDD CHIARI SYNDROME –

1) MYELOPROLIFERATIVE NEOPLASM – It is the carcinoma of blood cells in which the body produces extra red blood cells, white blood cells, or platelets. It is the most common cause for the development of Budd chairi syndrome.

2) ANTIPHOSPHOLIPID ANTIBODIES: It is an autoimmune disorder that can lead to clotting.

3) PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

4) BECHET’S DISEASE – In this disease also body’s immune system attacks its own cells.

5) ORAL CONTRACEPTIVE TABLETS

6) PREGNANCY – Pregnant women are at high risk of developing clots which can lead to obstruction of hepatic venous blood flow.

B) SECONDARY BUDD CHIARI SYNDROME –

1) CARCINOMA – Liver cancer, and renal cancer can either cause thrombus formation or the tumor can press the hepatic vein which can cause obstruction in the blood flow.

2) LIVER ABSCESS – It can be either an amoebic or pyogenic liver abscess.

3) ABDOMINAL SURGERY can cause clot formation.

4) INJURIES can also lead to thrombus formation.

CLINICAL FEATURES :

There is no specific symptom that points towards Budd Chiari syndrome and that is what makes its diagnosis difficult and leads to delay in treatment. Sometimes the patient usually remains without any symptoms.

Some of the signs and  symptoms of this syndrome are :

1) Fever

2) Abdominal pain especially on the right side.

3) Hepatomegaly on palpation can be present

4) Tenderness in the right upper quadrant can be increased during palpation too.

5) Swelling of the abdomen or ascites can be seen.

6) swelling of the legs.

7) Loss of appetite

8) weakness

9) Nausea or vomiting which can be accompanied by the blood which can be due to gastrointestinal tract bleeding.

10) Melena which is a coffee-colored stool can be present too due to gastrointestinal bleeding.

11) Patients can complain of itching.

12) yellowish discoloration of skin, nails, and mucus membranes can be seen in some patients.

13) Some patients can go into a coma if not treated in time which is known as hepatic encephalopathy.

INVESTIGATIONS :

Routine blood and urine investigations should be done to check the status of the liver. These tests include :

1) RBC, WBC, AND PLATELETS should be checked.

2) SERUM PROTHROMBIN TIME

3) SERUM BILIRUBIN ( both conjugated and unconjugated)

4) SERUM ALKALINE PHOSPHATES

5) SERUM TRANSAMINASE

6) SERUM ALBUMIN

7) FASTING AND POSTPRANDIAL BLOOD SUGAR LEVEL SHOULD BE CHECKED TOO.

8) URINE TEST

IMAGING TECHNIQUE USED FOR DIAGNOSIS :

Doppler ultrasound, CT SCAN, and MRI SCAN are usually the best ways to look for any blood clots or any tumor pressing on the venous outflow system of the liver which can help to diagnose Budd-Chiari syndrome.

LIVER BIOPSY is not of great significance and it might lead to uncontrolled bleeding. It is limited to little diagnostic significance.

TREATMENT :

Treatment of the patient should be started early to improve the prognosis and with treatment, the cause for the disease should be investigated to remove the cause if it is treatable.

1) ANTICOAGULATION – As thrombus formation is the factor leading to obstruction of the hepatic veins in Budd Chiari syndrome, so anticoagulation treatment should be started in these patients as it helps in improving the outcome. Anticoagulants are started by the doctor as a prophylactic measure too as this prevents clot formation.

2) ANGIOPLASTY: It is a procedure that is used to open up narrowed blood vessels due to clot formation. It can be either balloon angioplasty or stent angioplasty.

3) FLUID TAPPING: If the patient presents with ascites then it should be treated medically first but if not responding then fluid tapping can be performed.

4) ANTIBIOTICS: These are generally started to prevent any secondary infection from developing due to ascites.

5) TIPS:  TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNTS (TIPS) is a procedure in which a channel is made between the inflowing portal vein and the outflowing portal vein to bypass the obstructed area and improve the blood supply.

6) LIVER TRANSPLANT: when the above-mentioned treatment methods don’t improve the condition of the patient then it’s important to perform a liver transplant surgery.

MEASURES TO IMPROVE THE OUTCOME OF BUDD-CHIARI SYNDROME:

1) Avoid using oral contraceptives without consulting the doctor as these medicines can damage the liver if not taken properly.

2) Avoid smoking as it can increase the chances of clot formation in the patient suffering from Budd-Chiari syndrome.

3) Stop drinking alcohol as it damages the liver and the patient can develop ascites quickly and also other liver-related problems.

4) Reduce oily and fatty food as these food are known to increase the chances of atherosclerosis and clot formation.

5) If suffering from another disease like hypertension and diabetes then take the medicine properly as prescribed by the doctor.

6) Extra care should be taken in pregnant women to check for any signs of clotting or liver problems occurring during pregnancy.