II. Epidemiology

  1. Often the first vaso-Occlusion crisis finding in young children (as early as 6 months of age)
  2. Occurs in up to 50% of children with Sickle Cell Anemia by the age of 2 years
  3. Rare over age 4 years old

III. Pathophysiology

  1. Not to be confused with Hand Foot Syndrome that occurs with certain Chemotherapy agents in non-sickle disease
  2. Occurs in Sickle Cell Anemia with vaso-Occlusion
  3. Caused by infarction of Bone Marrow
    1. Metacarpal or Metatarsal Bones and phalanges

IV. Symptoms

  1. Painful swelling and tenderness of either the hand or foot
  2. Digits may appear mildly erythematous

V. Signs

  1. Low grade fever (variably present)

VI. Differential Diagnosis

VII. Course

  1. Resolves spontaneously within 1 week

VIII. References

  1. Welsh and Welsh (2016) Crit Dec Emerg Med 30(11): 15-23

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