Splenomegaly in Children

Background

• The gold standard definition of splenomegaly is based on the weight of the spleen which can only be determined upon its removal from the body.
• Ultrasound can be useful in determining splenic size in infants and children.
• The primary functions of the spleen are immune defense and quality control of circulating red cells.
• The causes of splenomegaly can be divided into several etiologic groups:
  • infection (viral causes most common)
  • hematologic disorders
  • autoimmune disorders and inflammatory causes
  • storage diseases
  • congestive causes
  • nonhematologic malignancy
  • splenic lesions

Evaluation

• Obtain a complete history and perform a thorough physical examination.
• Specific techniques such as the Middleton, Castell, and Nixon methods may help detect splenomegaly on physical examination.
• Consider obtaining initial laboratory testing in children with splenomegaly such as hematologic and biochemical testing, monospot, autoimmune screening, and urine dipstick.
• The presence of cytoses or cytopenias may help to differentiate the various causes of splenomegaly.
• Consider additional laboratory testing in select children depending on historical and clinical features.
• Consider a splenic ultrasound to confirm the spleen size, shape, and features.
• If after an initial evaluation is performed and no cause for the splenomegaly is identified, consider watchful waiting or more invasive testing as guided by a specialist.

Management

• Perform emergency management when signs and symptoms of spontaneous splenic rupture occur such as:
  • diffuse abdominal pain, left upper quadrant pain, left shoulder tip pain
  • hemodynamic instability with rising pulse rate and decreasing blood pressure
• Perform cause-specific management when a diagnosis for the splenomegaly has been identified.