Differentials
Sinusoidal obstruction syndrome (SOS) (veno-occlusive disease)
SIGNS / SYMPTOMS
Usually occurs in the setting of recent bone marrow transplant.
According to Seattle criteria, 2 of 3 of the following findings occurring within 20 days of transplantation can diagnose SOS: bilirubin >34.2 micromol/L (2 mg/dL); hepatomegaly or right upper quadrant pain of liver origin; >2% weight gain due to fluid accumulation.[47]
An international panel of multidisciplinary experts has published diagnosis, grading, and treatment recommendations for children, adolescents, and young adults with sinusoidal obstructive syndrome.[44]
INVESTIGATIONS
Bilirubin >34.2 micromol/L (2 mg/dL).
Plasma plasminogen activator inhibitor-1 level above 100 nanograms/mL.[48]
Detection of reverse blood flow in a segment of portal vein by colour-Doppler sonogram is useful for early diagnosis of veno-occlusive disease.[49]
Liver biopsy: early histological findings are narrowing of the sublobular and central veins due to subendothelial oedema, possibly secondary to disruption of sinusoidal endothelial cell barrier and congestion of hepatic sinusoids, surrounded by pale necrotic hepatocytes.[50]
Liver biopsy, portal venous wedge pressure, and reversal of portal venous flow on Doppler ultrasonography are not recommended for diagnosis in paediatrics.[44]
Fulminant hepatic failure due to other aetiologies
SIGNS / SYMPTOMS
Often clinically indistinguishable.
May have history of alcohol misuse, risk factors for viral hepatitis (e.g., intravenous drug user, blood transfusion, travel to endemic areas), or family history of liver disease (e.g., Wilson's disease). Drugs such as paracetamol and halothane may cause acute liver failure.
INVESTIGATIONS
Viral serology (hepatitis A virus IgM, hepatitis E virus IgM, HBsAg, anti-HBc IgM, hepatitis delta IgM, and CMV IgM): positive.
Serum copper and ceruloplasmin levels: abnormal in Wilson's disease.
Drug levels elevated (e.g., paracetamol).
Antinuclear antibodies, acid sphingomyelinase, and liver/kidney microsomal antibody 1 and 2: elevated.
Normal-appearing hepatic veins and portal vein shown with imaging.
Congestive hepatopathy
SIGNS / SYMPTOMS
History of exertional dyspnoea, orthopnoea, and angina.
Jugular venous distention, heart murmurs, rales, tender hepatomegaly, sometimes massive, with a firm and smooth liver edge, and peripheral oedema. Jugular venous distention with hepatojugular reflux may be present. Splenomegaly is uncommon.
INVESTIGATIONS
Elevation of total serum bilirubin level, most of which is unconjugated; serum aminotransferase levels show a mild elevation unless cardiac output is impaired (extremely high); prothrombin time may be mildly abnormal; albumin level may be decreased; serum ammonia level may be elevated.
Echocardiogram: depressed and dilated left and/or right ventricle with low ejection fraction; may reveal underlying cause.
Tricuspid regurgitation
SIGNS / SYMPTOMS
History of coronary artery disease/previous myocardial infarction, infective endocarditis, or carcinoid disease.
Lower left parasternal systolic murmur that increases on inspiration. Jugular vein distention with hepatojugular reflux, peripheral oedema, pulsatile hepatomegaly (very characteristic feature).
INVESTIGATIONS
Echocardiogram: ventricular and annular dilation, regurgitant jet on Doppler.
ECG: atrial flutter/fibrillation; evidence of previous myocardial infarction.
Constrictive pericarditis
SIGNS / SYMPTOMS
History of tuberculosis, transmural myocardial infarction, cardiac surgery, neoplasm, viral or bacterial infection, uraemia, dialysis treatment, radiotherapy, or systemic autoimmune disorders.
Sharp, stabbing, pleuritic, or aching chest pain. Trapezius ridge pain.
Jugular venous distention, Kussmaul's sign (jugular venous distension rises with inspiration), pulsus paradoxus, pericardial knock. Pericardial rub described as high-pitched or squeaky; best heard at the left sternal edge with the patient leaning forwards at end-expiration.
INVESTIGATIONS
ECG: low voltage with upwards concave ST segment elevation globally and PR depressions.
Echocardiogram: thickened pericardium, pericardial effusion, and limitation to ventricular filling.
Calcification of pericardium sometimes seen on CXR.
Chest CT: pericardial effusion or the pericardial cavity completely replaced by granulation tissue in chronic cases.
Right and left heart catheterisation with haemodynamic evaluation may be required to confirm the diagnosis.
Right atrial myxoma
SIGNS / SYMPTOMS
Systolic murmur loudest in inspiration, with tumour 'plop', may be heard.
INVESTIGATIONS
FBC: Hb and haematocrit decreased; leukocytosis, thrombocytopenia.
Echocardiogram: atrial mass seen.
CT or MRI: differentiation between mass and thrombus.
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