Bilateral valgus-extension osteotomy of hip using hybrid external fixator in spondyloepiphyseal dysplasia: early results of a salvage procedure

Abstract

The treatment of spondyloepiphyseal dysplasia (SED) congenita patients with severe bilateral hip involvement is difficult because it involves deformities of multiple joints and of the spine. The purpose of this study was to evaluate the results of the procedure described below as a method of treatment for bilateral hip involvement in SED congenita patients. We performed proximal femoral valgus-extension osteotomy with distal femoral varus osteotomy using a hybrid external fixator in eight patients (seven male patients and one female patient) or 16 hips. The patients had a mean age of 16.37 years (range, 9-25 years) at the time of surgery. The mean valgus angle at the proximal osteotomy site was 53.4 degrees on the right side and 52.5 degrees on the left side, and the mean varus angle at the distal osteotomy site was 22 degrees . Mean fixator time was 19.8 weeks (range, 10-42 weeks). After an average follow-up of 25.9 months, the mean modified Harris hip score had improved from 67.9 points to 79.1 points, which was statistically significant (P=0.012). Preoperative knee range of motion was achieved at the last follow-up in all patients. Waddling gait was absent in three patients, reduced in four patients, and was the same in one at the last follow-up. Mean limb length gain was 3.5 cm (range, 0-5 cm), mean limb length discrepancy less than 0.5 cm, and the mechanical axis was realigned in all. In conclusion, our early results suggest that proximal valgus-extension osteotomy with distal femoral varusization can be a useful treatment option in young patients with bilateral hip involvement in SED congenita.

Combined, novel management of bilateral varus hip deformity using "Eight-plate" in children with spondyloepiphyseal dysplasia congenita

“…The greater trochanter becomes relatively "overgrown" and prominent, reducing the effective abductor lever arm, limiting hip abduction, and causing gait disturbances. Moreover, the progressive natural history of the CV, pain and early hip osteoarthritis are major reasons why surgical treatment is indicated [2][3][4] .…”

“…The major features of SEDc are a femoral head ossification delay and a proximal femur varus deformity i.e. coxa vara (CV) [2][3][4] . However, two types of CV were described in patients with SEDc.…”

“…Although the data on hip surgery in SEDc are scarce, the accepted treatment is a valgus femoral osteotomy 4,6 . This procedure is indicated due to a progressive natural history of the proximal femur varus deformity, a decreasing neck-shaft angle (NSA), pain, limited hip abduction, gait disturbances, and subsequent development of early hip osteoarthritis [2][3][4] .…”

Best practice guidelines regarding diagnosis and management of patients with type II collagen disorders

“…There are very few studies that report the treatment of coxa vara in type II collagenopathies. 60,61 All have been case series studies. A 2008 report using a hip support osteotomy with an external fixator reported improvements in hip pain, waddling gait, hip range of motion, and the Harris hip score.…”

“…A 2008 report using a hip support osteotomy with an external fixator reported improvements in hip pain, waddling gait, hip range of motion, and the Harris hip score. 61 In a more recent article in 2017, valgus extension/internal rotation osteotomy was achieved with a femoral blade plate. This was an effective treatment that improved postoperative hip pain and hip range of motion as well as improved lumbar lordosis with a low recurrence rate.…”