Multifocal Joint Osteonecrosis in Sickle Cell Disease

The purpose of this study was to evaluate the frequency of multifocal osteonecrosis in patients with sickle cell disease. Between 1980 and 1989, 200 patients with sickle cell disease were treated in our institution for osteonecrosis. The patient population consisted of 102 males and 88 females with a mean age of twenty-six years at the time of presentation (range, eighteen to thirty-five years) and was followed until the year 2005. This cohort of patients was follow-up during average 15 years (until the year 2005). Multifocal osteonecrosis was defined as a disease of 3 or more anatomic sites. At the time of presentation, 49 patients were identified as having multifocal osteonecrosis. At the most recent follow-up, 87 patients had multifocal osteonecrosis. So at the last follow up among these eighty-seven patients, the occurrence of osteonecrosis was 158 lesions of the proximal femur associated with 151 proximal humerus osteonecroses, thirty-three lateral femoral condyle osteonecroses, twenty-eight distal femoral metaphysis osteonecroses, twenty-seven medial femoral condyle osteonecroses, twenty-three tibial plateau osteonecroses, twenty-one upper tibial metaphysis osteonecroses and forteen ankle osteonecroses. The total number of osteonecrosis was 455 in these 87 patients. The epiphyseal lesions were more frequent than the metadiaphyseal lesions excepted in the proximal tibia (Table 3). In conclusion, in patients with sickle cell disease, the risk of multifocal osteonecrosis is very high. In patients with hip osteonecrosis, the other joints should be evaluated with radiograph and MRI if the joint is symptomatic. In patients with osteonecrosis of the knee, shoulder or ankle, the patients’ hip should be evaluated by radiographs or MRI, regardless of whether the hip is symptomatic.