Acute long bone diaphyseal infarcts in sickle cell disease

Abstract

One hundred and ninety-eight acute diaphyseal cortical infarcts in 81 patients with S and C haemoglobinopathies are reviewed. The great majority (65 patients) were genotype SS. Chronic forms of diaphyseal infarction, such as “tram lines” or cortical thickening, are not included. Cases of dactylitis alone are not included. Fifty-nine of the 65 SS patients in this series, unselected as to age, were less than nine years old. In sickle cell disease this is clearly a complication of the young. The SC patients were older than the SS patients. Two predominant patterns are seen: infarction of almost the entire shaft, primarily in young infants, and localised infarctions more commonly in older infants, children and young adults. Eighty-five per cent of the localised infarctions occurred in the metadiaphyseal region. Some specific sites appear to be more commonly involved than others, the distal femora and proximal tibiae being the most commonly involved. Multiple sites of involvement and symmetrical lesions are common. A frequent pattern is bilateral symmetrical infarcts of the distal femora often with fractures. The way the vascular anatomy of the diaphysis changes with age and other developmental factors is discussed in relation to the location, and radiological appearances of acute and sub-acute long bone diaphyseal infarcts in sickle cell disease.