Budd-Chiari syndrome differential diagnosis

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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]

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Differential diagnosis

Budd-Chiari syndrome can be differentiated from other causes of hepatic failure like viral hepatitis and liver cirrhosis. The differential diagnosis is as follows:

Abbreviations: RUQ= Right upper quadrant of the abdomen, LUQ= Left upper quadrant, LLQ= Left lower quadrant, RLQ= Right lower quadrant, LFT= Liver function test, SIRS= Systemic inflammatory response syndrome, ERCP= Endoscopic retrograde cholangiopancreatography, IV= Intravenous, N= Normal, AMA= Anti mitochondrial antibodies, LDH= Lactate dehydrogenase, GI= Gastrointestinal, CXR= Chest X ray, IgA= Immunoglobulin A, IgG= Immunoglobulin G, IgM= Immunoglobulin M, CT= Computed tomography, PMN= Polymorphonuclear cells, ESR= Erythrocyte sedimentation rate, CRP= C-reactive protein

Classification of acute abdomen based on etiology Disease Clinical manifestations Diagnosis Comments Symptoms Signs Fever Rigors and chills Abdominal Pain Jaundice Hypo- tension Guarding Rebound Tenderness Bowel sounds Lab Findings Imaging Abdominal causes Inflammatory causes Pancreato-biliary disorders Acute suppurative cholangitis + + RUQ + + + + N Abnormal LFT WBC >10,000 Ultrasound shows biliary dilatation/stents/tumor Septic shock occurs with features of SIRS Acute cholangitis + − RUQ + − − − N Abnormal LFT Ultrasound shows biliary dilatation/stents/tumor Biliary drainage (ERCP) + IV antibiotics Acute cholecystitis + − RUQ + − − − Hypoactive Hyperbilirubinemia Leukocytosis Ultrasound shows gallstone and evidence of inflammation Murphy’s sign Acute pancreatitis + − Epigastric ± ± − − N Increased amylase / lipase Ultrasound shows evidence of inflammation Pain radiation to back Primary biliary cirrhosis − − RUQ/Epigastric + − − − N Increased AMA level, abnormal LFTs Primary sclerosing cholangitis + − RUQ + − − − N Increased liver enzymes Increased IgM, IgG4 Anti-neutrophil cytoplasmic antibody (p-ANCA) Anti-nuclear antibody (ANA) Anti-smooth muscle antibody (Anti-Sm) Anti-endothelial antibody Anti-cardiolipin antibody Cholelithiasis ± − RUQ/Epigastric ± − + + N to hyperactive for dislodged stone Leukocytosis Ultrasound shows gallstone Murphy’s sign Gastric causes Peptic ulcer disease ± − Diffuse − + in perforated + + N Ascitic fluid LDH > serum LDH Glucose < 50mg/dl Total protein > 1g/dl Air under diaphragm in upright CXR Upper GI endoscopy for diagnosis Gastritis ± − Epigastric Gastroesophageal reflux disease − − Epigastric − − − − Gastric outlet obstruction − − Epigastric − ± Hyperactive Gastrointestinal perforation + ± Diffuse ± + + ± WBC> 10,000 Air under diaphragm in upright CXR Intestinal causes Acute appendicitis + +in pyogenic appendicitis Starts in epigastrium, migrates to RLQ − + in perforated appendicitis + + Hypoactive Leukocytosis Ultrasound shows evidence of inflammation Nausea & vomiting, decreased appetite Acute diverticulitis + ± LLQ − − + − Hypoactive Leukocytosis CT scan and ultrasound shows evidence of inflammation Inflammatory bowel disease ± − Diffuse Irritable bowel syndrome ± − Diffuse − − − − N Tests done to exclude other diseases as it diagnosis of exclusion Tests done to exclude other diseases as it diagnosis of exclusion Symptomatic treatment High dietary fiber Osmotic laxatives Antispasmodic drugs Whipple's disease ± − Diffuse ± ± − − N Thrombocytopenia Hypoalbuminemia Small intestinal biopsy for Tropheryma whipplei Endoscopy is used to confirm diagnosis. Images used to find complications Chest and joint x-ray CT MRI Echocardiography Extra intestinal findings: Uveitis Endocarditis Encephalitis Dementia Hepatosplenomegaly Arthritis Ascites Toxic megacolon + − Diffuse − + ± − Hypoactive Tropical sprue + − Diffuse Celiac disease − − Diffuse ±, also dermatitis herpetiformis − − − Hyperactive (increased sounds) IgA endomysial antibody IgA tissue transglutaminase antibody Anti-gliadin antibody Hepatic causes Viral hepatitis + − RUQ + − + Liver masses + + in Liver abscess RUQ ± + in sepsis Budd-Chiari syndrome + − RUQ ± − - - N Laboratory findings consistent with the diagnosis of acute and fulminant BCS include: Elevated serum aspartate aminotransferase and alanine aminotransferase levels may be more than five times the upper limit of the normal range. Elevated serum alkaline phosphatase and bilirubin levels, decreased serum albumin level. Findings on CT scan suggestive of Budd-Chiari syndrome include: Early enhancement of the caudate lobe and central liver around the inferior vena cava Delayed enhancement of the peripheral liver with accompanying central low density (flip-flop appearance) inhomogeneous mottled liver (nutmeg liver) Peripheral zones of the liver may appear hypoattenuating because of reversed portal venous blood flow Inability to identify hepatic veins In the chronic phase, there is caudate lobe enlargement and atrophy of the peripheral liver in affected areas Ascitic fluid examination shows: Total protein more than 2.5 g per deciliter White blood cells are usually less than 500/μL. Hemochromatosis − − RUQ Dull / aching + in cirrhotic patients may be in cardicmyopathy − − >60% TS >240 μg/L SF Raised LFT Hyperglycemia Ultrasound shows evidence of cirrhosis Extra intestinal findings: hyperpigmentation Diabetes mellitus Arthralgia Impotence in males Cardiomyopathy Atherosclerosis Hypopituitarism Hypothyroidism Extrahepatic cancer Prone to specific infections Cirrhosis + − RUQ + − Peritoneal causes Spontaneous bacterial peritonitis + − Diffuse + in cirrhotic patients ± − − Hypoactive Ascitic fluid PMN>250 cells/mm³ Culture: Positive for single organism Ultrasound for evaluation of liver cirrhosis Hollow Viscous Obstruction Small intestine obstruction − − Diffuse − − + ± Hyperactive then absent Leukocytosis Abdominal X ray Nausea & vomiting associated with constipation, abdominal distention Volvulus − − Diffuse − − + − Hypoactive Leukocytosis CT scan and abdominal X ray Nausea & vomiting associated with constipation, abdominal distention Biliary colic − − RUQ + − − − N Increased bilirubin and alkaline phosphatase Ultrasound Nausea & vomiting Renal colic − − Flank pain − − − − N Hematuria CT scan and ultrasound Colicky abdominal pain associated with nausea & vomiting Vascular Disorders Ischemic causes Mesenteric ischemia ± − Periumbilical − ± − − Hyperactive Leukocytosis and lactic acidosis CT scan Nausea & vomiting, normal physical examination Acute ischemic colitis ± ± Diffuse − + + Hyperactive then absent Leukocytosis CT scan Nausea & vomiting Hemorrhagic causes Ruptured abdominal aortic aneurysm − − Diffuse − + − − N Normal CT scan Unstable hemodynamics Intra-abdominal or retroperitoneal hemorrhage − − Diffuse − + − − N Anemia CT scan History of trauma Gynaecological Causes Tubal causes Torsion of the cyst − − RLQ / LLQ − ± ± N Increased ESR and CRP Ultrasound Sudden onset sever pain with nausea and vomiting Acute salpingitis + ± RLQ / LLQ − − ± ± N Leukocytosis Pelvic ultrasound Vaginal discharge Cyst rupture − − RLQ / LLQ − + ± ± N Increased ESR and CRP Ultrasound Sudden onset sever pain with nausea and vomiting Pregnancy Ruptured ectopic pregnancy − − RLQ / LLQ − + − − N Positive pregnancy test Ultrasound History of missed period and vaginal bleeding Extra-abdominal causes Pulmonary disorders Pleural empyema + ± RUQ/Epigastric − − − − N Cardiovascular disorders Myocardial Infarction − − Epigastric − + in cardiogenic shock − − N

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