Successful Two-step Correction for Severe Genu Valgum in Ellis-van Creveld Syndrome: A Case Report

INTRODUCTION: Ellis-van Creveld syndrome (EVC) is a very rare genetic disorder involving a severe, relentlessly progressive genu valgum deformity of the knee and it is usually difficult to correct all of the deformities by elevation of the depressed lateral tibial plateau alone. We are reporting one such case treated using two-step operative technique (first, gradual correction using Taylor spatial frame (TSF) and second, tibia elevation through intra-articular osteotomy). CASE REPORT: We report a case of 10-year-old female with severe genu valgum in EVC using two-step operative technique. First, performing soft tissue release and gradual correction using a TSF. Second, tibia elevation through intra-articular osteotomy resulted in improved joint congruity. At final follow-up, the patient has a good functional result and the Mikulicz line proceeded physiologically to the joint center. CONCLUSION: This two-step technique allowed for the correction of the joint architecture and associated complications are minimal. For severe genu valgum in EVC, joint congruity is improved by the elevation of the anterolateral aspect of the tibia through intra-articular osteotomy. Performing soft tissue release followed by gradual correction allows for correction of the valgus and external rotation deformities without causing neuroparalysis.