Budd Chiari syndrome

Budd-Chiari syndrome
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Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Abdominal pain, ascites, hepatomegaly
Complications	Liver failure, portal hypertension
Onset
Duration
Types	N/A
Causes	Thrombosis of the hepatic veins
Risks	Polycythemia vera, pregnancy, oral contraceptive use
Diagnosis	Ultrasound, CT scan, MRI, venography
Differential diagnosis	N/A
Prevention	N/A
Treatment	Anticoagulation, thrombolysis, angioplasty, liver transplantation
Medication	N/A
Prognosis
Frequency	Rare
Deaths	N/A

Budd-Chiari syndrome is a rare condition characterized by the obstruction of the hepatic veins that drain the liver. This obstruction leads to increased pressure in the liver, resulting in hepatomegaly, ascites, and abdominal pain. The syndrome can lead to severe complications such as liver failure and portal hypertension.

Etiology

Budd-Chiari syndrome is primarily caused by thrombosis of the hepatic veins. Several risk factors have been identified, including:

• Polycythemia vera
• Pregnancy
• Use of oral contraceptives
• Myeloproliferative disorders
• Chronic inflammatory diseases

Pathophysiology

The obstruction of the hepatic veins leads to increased venous pressure in the liver, causing congestion and ischemia. This results in liver cell damage and necrosis. Over time, the liver may develop fibrosis and cirrhosis if the condition is not treated.

Clinical Presentation

Patients with Budd-Chiari syndrome typically present with:

• Abdominal pain
• Ascites
• Hepatomegaly
• Jaundice (in some cases)

Diagnosis

The diagnosis of Budd-Chiari syndrome is based on imaging studies and clinical presentation. Common diagnostic tools include:

• Ultrasound with Doppler studies to assess blood flow in the hepatic veins
• CT scan or MRI to visualize the liver and veins
• Venography to directly visualize the obstruction

Treatment

Treatment of Budd-Chiari syndrome aims to relieve the obstruction and manage complications. Options include:

• Anticoagulation to prevent further thrombosis
• Thrombolysis to dissolve existing clots
• Angioplasty or stenting to open blocked veins
• Liver transplantation in cases of severe liver damage

Prognosis

The prognosis of Budd-Chiari syndrome varies depending on the severity of the obstruction and the underlying cause. Early diagnosis and treatment improve outcomes significantly.

Also see

• Portal hypertension
• Liver cirrhosis
• Hepatic vein
• Thrombosis