Budd-Chiari syndrome differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2] Amandeep Singh M.D.[3]

Differential diagnosis

Budd-Chiari syndrome should be differentiated from other causes of hepatic failure like viral hepatitis and liver cirrhosis, splenic vein thrombosis, portal vein thrombosis and inferior vena cava obstruction that may have similar presentation with right upper quadrant abdominal pain and ascites. The differential diagnosis is as follows:

Condition	Differentiating signs and symptoms	Differentiating Tests
Budd-Chiari Syndrome	Abdominal pain Diarrhea Ascites	Doppler ultrasound and CT of the abdomen shows absence of the hepatic vein filling Abdominal CT scan will show a rapid clearing of the caudate lobe of the liver
Cirrhosis	Abdominal pain and bloating Menstrual irregularities Symptoms of hypogonadism: Impotence Infertility Testicular atrophy Loss of libido Easy bruisability Decompensated cirrhosis may present with Jaundice Increase in abdominal girth due to ascites Pruritus Signs of upper gastrointestinal bleeding: Hematemesis Hematochezia Melena Symptoms due to hepatic encephalopathy: Altered mental status Confusion Sleep disturbances	Ultrasound findings in cirrhosis are as follows: Changes in liver contour: shrunken, irregular appearance Non homogeneous appearance of the hepatic tissue Increased surface nodularity Increase in echogenecity from focal fatty infiltration Atrophy of the right lobe and hypertrophy of the caudate or left lobe Splenomegaly Ascites Varices Hepatic or portal vein thrombosis Abdominal MRI may also be helpful in the diagnosis of portal hypertension. Findings on MRI suggestive of cirrhosis with portal hypertension include: Cirrhotic liver, as shrinkage and atrophy in liver Re-canalized umbilical vein--pathognomonic Dilated portal vein and/or splanchnic veins Esophageal varices Collaterals in any abdominal organ Splenomegaly Ascites Transient elastography and the Acoustic Radiation Force Impulse (ARFI) technique are well-established methods for the staging of fibrosis in various liver diseases:   The FibroScan (transient elastography) uses elastic waves to determine liver stiffness which theoretically may be converted into a liver score. The FibroScan produces an ultrasound image of the liver (from 20-80mm) along with a pressure reading (in kPa). Transient elastography is much faster than a biopsy (usually lasts 2.5-5 minutes) and is completely painless. Findings on transient elastography may show reasonable correlation with the severity of cirrhosis: Increasing scarring of the liver is associated with increasing "stiffness" of the tissue.
Splenic vein thrombosis	Signs and symptoms of: Upper abdominal pain radiating to the back Vomiting Hypoactive bowel sounds Fever Shock Cullen's sign Grey-Turner's sign	Ultrasound of the abdomen and CT scan will show evidence of a splenic vein thrombosis Normal hepatic venous pressure gradient is present
Portal vein thrombosis	Symptoms depend on the underlying cause: If pancreatitis is present, the patient may develop: Upper abdominal pain radiating to the back Vomiting Hypoactive bowel sounds fever shock Cullen's sign Grey-Turner's sign If the cause is ascending cholangitis, symptoms include: Fever Rigors Right upper quadrant pain Dark urine Pale stools If abdominal sepsis is the cause, symptoms include: Fever Abdominal pain Signs of peritonitis	Doppler ultrasound and abdominal CT scan show a portal vein filling defect and absence of flow in the portal vein. MR or direct angiography show a normal hepatic venous pressure gradient.
Schistosomiasis	Patients may have a history of travel to endemic areas Constitutional symptoms such as Malaise Rigors Anorexia Weight loss Vomiting Diarrhea Headache Muscular aches Weakness Abdominal pain Urticaria Fever Lymphadenopathy	MR or direct angiography shows a normal hepatic venous pressure gradient
Inferior vena cava obstruction	Signs and symptoms of renal cell carcinoma: Hematuria Flank pain Flank or abdominal mass Weight loss Hypertension	Ultrasound of the abdomen shows evidence of inferior vena cava obstruction
Nodular regenerative hyperplasia	None	Liver biopsy shows small regenerative nodules with little or no fibrosis on reticulin staining
Idiopathic portal hypertension (hepatoportal sclerosis)	None	Liver biopsy shows no evidence of cirrhosis