Treatment of Varus Deformities of the Lower Limbs in Patients with Achondroplasia and Hypochondroplasia

Abstract

Angular deformities of the lower limbs are a common clinical problem encountered in pediatric orthopaedic practices particularly in patients with osteochondrodysplasias. The varus deformity is more common than the valgus deformity in achondroplasia and hypochondroplasia patients because of the unusual growth of the fibulae than that of the tibiae. We retrospectively reviewed six patients (four patients with achondroplasia and two patients with hypochondroplsia) with relevant limb deformities due to the above-mentioned entities. All patients manifested significant varus deformity of the lower limbs. Detailed phenotypic characterization, radiologic and genetic testing was carried out as baseline diagnostic tool. We described the re-alignment procedures, which have been applied accordingly. Therefore, bilateral multi-level procedures, multi-apical planning and limb lengthening have been successfully applied. While recognition of the underlying syndromic association in patients who are manifesting angular deformities is the baseline for proper orthopaedic management, this paper demonstrates how to evaluate and treat these complex patients.

Optimal management of complications associated with achondroplasia

“…Chronic knee pain and changes in bony alignment can contribute to delays in weight-bearing, an altered gait pattern, and the potential for adult onset osteoarthritis associated with uneven weight distribution across the knee and ankle joints 1,9,31. For those individuals demonstrating pain, and/or an altered gait pattern, corrective surgery such as tibial osteotomies, fibula epiphysiodesis, or continuous distraction epiphysiodesis may be utilized 30,32,33. Hunter et al10 reported that 22% of individuals in their multicenter review had had osteotomies, most commonly between 12 and 20 years of age.…”

Fixator-Assisted Lengthening and Deformity Correction Over an Intramedullary Nail in a Patient with Achondroplasia

“…Varus malalignment is generally more common than genu valga in this syndrome. Realignment can generally be achieved by gradual correction using external fixation devices [6]. Surgical correction is needed for tibial deformities higher than 10 degrees at coranal plane in adolescent with unexpected remodeling potential and adults [7].…”

“…Lower limbs can be lengthened with chondrodiastasis (the growth plate distraction) and callotasis (the callus distraction) successfully [4]. Nowadays in common practice both the tibia and femur are simultaneously lengthened in different sessions [6]. In this study, We report the result of using a combination of fixator-assisted nailing with lengthening over an intramedullary nail in a 17 year old patient with achondroplasia with tibial deformity and shortening.…”

Deformity Correction by Femoral Supracondylar Dome Osteotomy with Retrograde Intramedullary Nailing in Varus Deformity of the Distal Femur after Pathologic Fracture of Giant Cell Tumor

“…Accordingly, in the presence of early arthritis in the medial or lateral compartment, deformity correction techniques can be useful for slowing the progression arthritis3). Depending on the extent and site of an angular deformity, proximal tibial osteotomy or distal femoral osteotomy can be performed7,8).…”

“…To halt the progression of arthritis2), deformity correction techniques can be used, and femoral osteotomy is one of such techniques for unicompartmental arthritis of the knee with a severe angular deformity of the distal femur3).…”