Aseptic osteonecrosis of the femoral head in patients with sickle cell anemia

Abstract

Sickle cell disease (SCD) is a genetic trouble of the hemoglobin synthesis inherited as an autosomal recessive trait, whose prevalence can vary from 5 to 25% in the different parts of the world. It is characterized by the presence of abnormal hemoglobin HbS instead of hemoglobin A. Patients suffering from major forms of SCD present the risk of developing epiphyseal necrosis. Aseptic osteonecrosis of the femoral head (AOFH) caused by ischemia, or bone infarction can affect between 20 and 50% of SCD patients. The femoral head is the most frequent epiphyseal location with a range of 74.6%. AOFH can affect patients at any age, but is mainly detected in men under 50 years. Indeed, a large majority of cases, in a range of 60%, have been diagnosed at an early radiological stage in young adults whose average age varies, in the literature, between 27 and 36 years. A surgical procedure becomes sometimes necessary due to the severity of pain and the functional consequences, frequently following the mechanical collapse of the osteonecrosis area. It is estimated that approximately 25-30% of SCD patients will undergo a total hip arthroplasty before 50y. Although the mortality rate, between 0.2-2.6%, tends to be similar to the general population rate undergoing a prosthetic surgery, the perioperative complications vary from 11.5 to 67%. Here, we clarify the epidemiological data and present an exhaustive update on the different preventive and therapeutic strategies, as well as the perioperative management in patients with an AOFH caused by SCD and risking multiple complications.

Keywords: Orthopedic surgery; Osteonecrosis; Sickle cell disease; Total hip arthroplasty.