SPR Unknown #40 -- FINAL
SPR unknown 40, sickle cell dactylitis, hand foot syndrome
Publication Date: 2010-11-09
Sickle cell dactylitis (hand foot syndrome)
Sickle cell dactylitis is often the first manifestation of sickle cell disease in children. It is caused by episodic occlusion of the microcirculation by abnormal sickle cells resulting in stasis of blood and sequestration of cells. Ischemia and tissue hypoxia worsen the sickling process leading to cell death. A typical clinical presentation includes tender, swollen hands and feet, reduction in movement, and fever.
In young children, bone infarctions tend to occur in the diaphyses of small tubular bones of the hands and feet, including the metacarpals, metatarsals, and proximal phalanges. In particular, these regions tend to be involved due to the persistence of red marrow in the young child. Hand and foot syndrome is most commonly see in children 6 months- 24 months of age. It is rare after 6 years of age because of the regression of red marrow in these areas with increasing age.
Radiologic findings: Initial radiographs usually are normal or show soft tissue edema in these patients. Subperiosteal new bone formation appears in 1-2 weeks and typically resolves within 2-3 months. Later manifestations can include both lytic and sclerotic lesions
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