What is the likely cause of hepatomegaly in a patient with a 2-week history of worsening abdominal pain, jaundice, abdominal distension, epigastric pain, smooth hepatomegaly, and dullness on percussion?

Budd-Chiari Syndrome is the Most Likely Diagnosis

Based on the clinical presentation of worsening abdominal pain, jaundice, abdominal distension with smooth hepatomegaly, and dullness on percussion (indicating ascites), Budd-Chiari syndrome (option b) is the most likely cause of hepatomegaly in this patient.

Clinical Reasoning

The constellation of findings points to hepatic venous outflow obstruction, which is the hallmark of Budd-Chiari syndrome:

Key Diagnostic Features Present

• Smooth hepatomegaly with tenderness: This reflects acute hepatic congestion from venous outflow obstruction, consistent with the "hard, smooth, and tender" liver edge described in congestive hepatopathy 1

• Ascites (dullness on percussion): Develops rapidly in Budd-Chiari syndrome due to post-sinusoidal portal hypertension from hepatic vein obstruction 1

• Jaundice: Results from hepatocellular dysfunction secondary to severe congestion and ischemia 2, 3

• Abdominal distension and epigastric pain: Caused by rapid liver capsule distension (Glisson capsule) from acute congestion 1

• Subacute presentation (2 weeks): Fits the typical timeframe for symptomatic Budd-Chiari syndrome to manifest 1

Why Other Options Are Less Likely

Crohn's disease (a) and Ulcerative colitis (e): While inflammatory bowel disease can have hepatobiliary manifestations, they do not typically present with this acute constellation of smooth hepatomegaly, ascites, and jaundice without prominent gastrointestinal symptoms 1

Left heart failure (c): Although cardiac congestion causes hepatomegaly ("congestive hepatopathy"), the clinical presentation would include prominent signs of heart failure (dyspnea, peripheral edema, elevated jugular venous pressure). The post-sinusoidal portal hypertension in heart failure produces similar findings but requires cardiac symptoms to be present 1

Iron deficiency anemia (d): Does not cause hepatomegaly, jaundice, or ascites 2

Pathophysiology of Budd-Chiari Syndrome

Budd-Chiari syndrome results from hepatic venous outflow obstruction at the level of hepatic veins or inferior vena cava 1. This creates:

• Post-sinusoidal portal hypertension with normal hepatic venous pressure gradient (HVPG) 1
• Rapid ascites formation from increased sinusoidal pressure and hepatic lymph production 1
• Hepatocellular injury from congestion and ischemia, manifesting as jaundice 2, 3

Critical Diagnostic Pitfall

Do not perform blind percutaneous liver biopsy in patients with suspected vascular liver disease and ascites, as this carries significant risk of hemorrhage 4. The diagnosis should be established through imaging first.

Immediate Diagnostic Workup Required

1. Abdominal ultrasound with Doppler as first-line imaging to assess hepatic vein patency and flow 1, 3

2. Hepatic profile with fractionated bilirubin (conjugated vs unconjugated), alkaline phosphatase, ALT, AST 3

3. CT or MRI with venous phase if ultrasound is inconclusive, to visualize hepatic vein thrombosis and assess for caudate lobe hypertrophy (characteristic of chronic Budd-Chiari) 1

4. Ascitic fluid analysis to rule out spontaneous bacterial peritonitis and confirm transudative nature 1

Management Implications

If Budd-Chiari syndrome is confirmed, urgent hepatology consultation is mandatory 1. Treatment options include:

• Anticoagulation as first-line therapy 1
• Hepatic vein stenting for focal stenosis (patency rates >80%) 1
• Transjugular intrahepatic portosystemic shunt (TIPS) for refractory cases 5
• Liver transplantation for fulminant hepatic failure or failed medical/interventional therapy 5

The prognosis depends on early recognition and intervention before irreversible hepatic injury occurs 1, 5.