Osteotomy and intramedullary nailing for the correction of progressive deformity in vitamin D-resistant hypophosphataemic rickets

Abstract

We have reviewed the results of surgical treatment of vitamin D-resistant hypophosphataemic rickets (VDRR) and describe a technique of corrective osteotomy and intramedullary nailing. From 1978 to 1986, epiphysiodesis (n = 4) and osteotomy (n = 8) was performed in 6 children (mean age 13, range 10-16 years) for the correction of progressive lower limb deformity. Realignment and internal fixation of a pathological fracture of the femur was performed in an adult (aged 24). Epiphysiodesis resulted in recurrent deformity in all patients and reapplication of staples for loosening was required in three. Corrective osteotomies were secured with staples (n = 3), plates (n = 4), or plaster alone (n = 1) and were complicated by non-union in one patient, and recurrent deformity in two patients. Double-plating of the femoral fracture resulted in union but recurrent deformity. Compliance to treatment with phosphate and vitamin D was variable. In order to manage progressive recurrent deformity, we have performed corrective osteotomy and closed intramedullary nailing of the tibia (n = 2) and femur (n = 3) in 4 skeletally mature patients (mean age 31). All osteotomies united and no complications were encountered. Deformity has been corrected in all cases and all patients are satisfied with the outcome at least 2 years after surgery. We conclude that rigid methods of fixation spanning the whole length of the bone are required to maintain limb alignment in skeletally mature patients with VDRR. Since the quality of bone in VDRR is variable, experience with intramedullary techniques is essential. We stress the importance of appropriate medical therapy throughout the treatment of these patients.