Introduction

Budd Chiari syndrome (BCS) is a clinical condition due to hepatic venous outflow obstruction. The obstruction can be in the small hepatic veins, inferior vena cava (IVC), and even in the right atrium. Primary Budd Chiari syndrome is due to an endoluminal lesion such as thrombosis or IVC web. Secondary Budd Chiari syndrome is due to extrinsic compression by the tumor, cysts, and abscesses or due to invasion of the hepatic veins or IVC by the tumor.

Pathophysiology

Post hepatic venous obstruction leads to increased sinusoidal pressure and congestion leading to hepatomegaly, hepatic pain, and ascites. Due to congestion, there is perisinusoidal necrosis of hepatocytes in zone 3 which eventually leads to liver failure. Patients also develop portal hypertension.

Prevalence

• 1 in 100,000 of the population worldwide and is more common in adults.
• It is infrequently seen in infants and children less than age 10 years account for 1-7% of all cases of BCS.
• Prevalence varies as per area - higher rate in Nepal, rare in Japan and France
• In infants, the etiology would be primary BCS due to acquired and inherited thrombophilias.

Causes

• Hypercoagulable states
• Infections,
• Malignancies,
• IVC web
• Polycystic liver disease
• Trauma to hepatic veins
• Inflammatory bowel disease (IBD)

Presentation

It can present as an acute, fulminant, or chronic state. Acute BCS presents an acute onset hepatomegaly and ascites. In fulminant BCS, there is rapid liver cell failure and patients may present with encephalopathy. Chronic BCS evolves over three to six months and on presentation, patients are in cirrhosis with decompensation.

Investigations

• Ultrasound Doppler studies, computed tomography (CT) venogram, or magnetic resonance imaging (MRI) venogram are used to check the patency of the hepatic veins, IVC and portal venous system. This helps to determine the line of treatment.
• It is necessary to investigate underlying hypercoagulable states in all patients.

Management

Medical Management

• Medical management consists of anticoagulation. In patients with ascites, sodium restriction, diuretic therapy, and paracentesis may be required.
• In the acute conditions, heparin can be used to achieve anticoagulation while warfarin can be used for long term treatment.
• The management depends on the local expertise, type of presentation, site and number of occlusions.

Interventions

• When the presentation is acute, catheter-directed thrombolytic therapy, angioplasty, and stent placement can be effective.
• Angioplasty has high occlusion rates. Hence, the placement of stents in the IVC or hepatic veins have been recommended. But the small size of vessels in the pediatric age group and complications of the stent such as migration and thrombosis should be considered before the stent placement.
• Transjugular intrahepatic portosystemic shunt surgery (TIPSS) may be employed in the acute or chronic setting. However, it is still used rarely in children.
• Liver transplantation - In cases of BCS with acute fulminant liver failure or decompensated cirrhosis (albumin <3 g/dl, prothrombin time 3s greater than control, and conjugated bilirubin >3 mg/dl), liver transplantation is the treatment of choice.

Algorithm for management

• Step 1 Anticoagulation
• Step 2 Recanalization procedure (percutaneous angioplasty with stenting or thrombolysis)
• Step 3 TIPSS (or surgical shunt)
• Step 4 Liver transplantation

Outcome

• The overall five-year survival rate is nearly 90%
• Medical management alone has varying results.
• Success with angioplasty was seen in 43% of cases, with hepatic vein stenting in 66%, whereas with TIPSS in 72% of cases(Eur J Gastroenterol Hepatol. 2016 May)
• Mortality was 3/18 following angioplasty and 8/18 following surgical shunt(Gut 1999;44:568-574)