SPR Unknown #40 -- FINAL

Bhavika Patel, MD

Kiery Braithwaite, MD

Emory University and Children's Healthcare of Atlanta

Keywords

SPR unknown 40, sickle cell dactylitis, hand foot syndrome


Publication Date: 2010-11-09

History

17 month old with fever and bilateral hand and feet swelling

Findings

There is circumferential periosteal new bone formation along the diaphyses surrounding several metacarpals bilaterally.

Diagnosis

Sickle cell dactylitis (hand foot syndrome)

DDx

Differential diagnosis for sickle cell dactylitis or hand and foot syndrome includes: acute osteomyelitis, cellulitis, leukemia, and rheumatic fever

Discussion

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

References

  1. Worrall, VT, Butera, V. Sickle-cell dactylitis. The Journal of Bone and Joint Surgery. 1976; 58: 1161-1163.
  2. Leikin SL, Gallagher D, Kinney TR, et al. Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease. Pediatrics. Sep 1989;84(3):500-8.
  3. Johnson L, Carmona-Bayonas A, Tick L. Management of pain due to sickle cell disease. J Pain Palliat Care Pharmacother. 2008;22(1):51-4.

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