This painful vaso-occlusive condition is commonly the 1st clinical manifestation of sickle cell disease in infants. It usually presents in children younger than 5 years of age, with nearly 45% of first cases presenting prior to the age of 2. Patients are acutely ill with fever, leukocytosis, and swollen hands and/or feet that are exquisitely painful due to bone infarction. Some children may present with fussiness and swelling with minimal erythema or fever. X-rays are often normal, and it is not until 1 to 2 weeks later that subperiosteal new bone, cortical thickening, and even complete bone destruction can be seen. The differential diagnosis includes osteomyelitis, trauma, cold injuries, acute rheumatic fever, juvenile rheumatoid arthritis, and leukemia.
Management and Disposition
Treatment of vaso-occlusive crises in sickle cell disease centers around providing adequate fluid balance, oxygenation, and analgesia. Therapy should be individualized. Fentanyl, hydromorphone, morphine, and ketorolac are analgesic agents commonly used in the treatment of children with painful sickle crises. If fever is present, bacterial infection should be assumed until proven otherwise. CBC, reticulocyte count, and blood cultures should be obtained from all febrile sickle cell patients. If the patient is afebrile, you can omit the blood culture. Empiric broad-spectrum antibiotic coverage should be instituted immediately (third-generation cephalosporin). In cases of dactylitis, very close follow-up is necessary not only for the management of sickle cell disease, but also to reevaluate the radiologic changes in the small bones of the hands and feet. In most instances, the previously described changes disappear; however, rarely, shortening of the fingers and toes may occur due to significant infarct.
Most clinical manifestations of sickle cell disease occur after the first 5 to 6 months of life. Hemolytic anemia gradually develops over the first 2 to 4 months (changes that follow the replacement of fetal hemoglobin by hemoglobin S) and leads to the clinical syndromes associated with an increased percentage of abnormal SS hemoglobin.
Sickle cell patients are at high risk for infection from encapsulated organisms due to their functional asplenia. The most common organisms causing osteomyelitis in sickle cell patients are Salmonella species, S aureus, and S pneumoniae.
Dactylitis can often be differentiated from osteomyelitis based on the symmetrical involvement of multiple bones and a negative blood culture.
Dactylitis, severe anemia, and leukocytosis within the first 2 years of life increase the risk for adverse sickle cell–related outcomes by age 10 years (death, stroke, frequent pain crises, or acute chest syndrome).
Hydroxyurea therapy has been shown to significantly reduce the rate of dactylitis in young children with sickle cell disease.
Acute Sickle Dactylitis. Bilateral cylindrical swelling of soft tissue of the hands in sickle cell disease consistent with vaso-occlusive crisis or dactylitis. (Photo contributor: Donald L. Rucknagel, MD, PhD.)