19 Avascular Necrosis of the Femoral Head Secondary to Sickle Cell Disease Case Reports of Two Caucasian Sisters

Background:Sickle cell (SC) disease leading to endarteritis induces skeletal changes in the form of osteitis, sclerosis of femoral canal and osteonecrosis of the femoral head. All these make total hip arthroplasty (THA) difficult and prolonged. There is increased risk of infection, SC crisis and increased complication rate. Our paper aims to highlight preoperative, intraoperative and postoperative hurdles encountered in performing THA in sicklers and the short term outcome using cementless implants.Materials and Methods:Thirty-nine patients with SC disease, who had osteonecrosis of the femoral head, were operated between 2007 and 2011. The mean age of patients was 22 years (range 13–49 years). There were twenty eight females and 11 males. Bilateral cementless total hip replacement (THR) was performed in 11 patients (22 hips) and in the rest unilateral (28 hips). Preoperative and postoperative modified Harris hip score was evaluated. The average followup was 3.8 years (range 2-6 years).Results:The average operating time was 96 min (range 88–148 min). The average blood loss was 880 ml (range 650–1200 ml). The average intraoperative blood transfused was 2.3 units (range 2–5 units). All patients showed an improvement in Harris hip score from 42 points preoperatively to 92 points at latest followup. Intraoperatively, one patient had a periprosthetic fracture. Six patients developed acute SC crisis and were managed in intensive care unit. Three patients developed wound hematoma. Three patients developed limb length discrepancy less than 1 cm. None had early or late dislocations, infection, heterotopic ossification, sciatic nerve palsy and aseptic loosening.Conclusion:THA in sicklers involves considerable challenge for the orthopedic surgeon. Management requires a multidisciplinary approach involving the anesthetist, hematologist and the orthopedic surgeon. Contrary to previous reports, THA in sicklers now has a predictable outcome especially with the use of cementless implants.

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“… 9 10 Avascular necrosis of femoral head has been reported more commonly in SC disease (29%) and 19% in adults with SS disease. 11 12 13 …”

Section: Discussionmentioning

confidence: 99%