Total hip replacement in patients with Gaucher’s disease with symptomatic osteonecrosis of the femoral head is controversial because of the high early failure rates. We describe four patients who had an uncemented total hip replacement following enzyme replacement therapy for a median of two years and one month (1 to 9.8 years) prior to surgery, and who remained on treatment. At operation, the bone had a normal appearance and consistency. Histopathological examination showed that, compared with previous biopsies of untreated Gaucher’s disease, the Gaucher cell infiltrate had decreased progressively with therapy, being replaced by normal adipose tissue. The surfaces of viable bone beyond the osteonecrotic areas showed osteoblasts, indicating remodelling. In one case acetabular revision was carried out after 11 years and eight months. The three remaining patients had a mean follow-up of six years and four months (3.3 to 12 years). We recommend initiating enzyme replacement therapy at least one to two years prior to total hip replacement to facilitate bone remodelling and to allow implantation of uncemented components in these young patients.
Authors present their experience with different surgical methods. The choice of surgery depends on the age of the growing child and on the site of the neoplasm.
Females were eight and males were five. After ten years only ten patients were disease free (seven osteogenic sarcomas and three Ewing’s sarcomas).
In five cases of o.s. the age of the patients was over fourteen years, the remaining growing period was limited and for this reason a mechanical growing prosthesis was employed. In the four cases of diaphyseal Ewing’s sarcoma the reconstruction was performed in two cases employing an autologous graft taken from the iliac crest and in two cases a microvascularized fibular graft. Functional results of the affected limb, in the surviving patients, depend on the sacrifice of the joint (arthro-dhesis) or in the use of a modular prosthesis that, in AA. experience have a follow-up of 12 years; in diaphyseal reconstruction an anatomical result was obtained either with the mcrovascularized fibular graft or with the autologous graft from the iliac crest, the only difference between these two methods depending on the time of bone healing.