Extreme Genu Recurvatum Deformity in a Pediatric Patient With Spondyloepiphyseal Dysplasia: Gradual Correction With Z-plates and Hexapod Frame

Abstract

Spondyloepiphyseal dysplasia is a type II collagenopathy with resulting spinal and extremity deformities. The clinical manifestations include short stature, hearing loss, kyphoscoliosis, and complex knee deformities. Genu recurvatum can be a challenging surgical problem, especially when the deformity is severe. In this report, we present a case of severe genu recurvatum in a 14-year-old female that was treated with a pediatric circular fixator with the addition of two z-plates. At one year follow-up, the patient demonstrated improved knee range of motion, tibial alignment with the radiographic union, and good ambulatory ability. The hexapod fixator with the use of two Z-plates may help ensure that appropriate ring strut angles are achieved. Larger studies regarding the efficacy of this treatment option in spondyloepiphyseal dysplasia are required.