Single-Stage Bilateral Hip Reconstruction for Kabuki Hip Dysplasia in a Four-Year-Old: A Case Report
Abstract
Kabuki syndrome (KS) is a rare congenital syndrome characterized by distinctive facies, joint hyperlaxity, and hypotonia. When hip dysplasia manifests, it is usually severe, demonstrating treatment resistance with a recognized risk of later re-dislocation. Limited case numbers with variable outcomes compound uncertainty regarding optimal management. To date, all surgical cases described in the literature have been treated with open reduction, with no documented cases of concurrent bilateral hip surgery. Here, we present the case of a four-year-old boy with Kabuki syndrome who has been unable to walk since the age of three, following a spontaneous left hip dislocation. Clinical examination revealed characteristic facies, hypotonia, and painful and reduced left hip movements. Radiographs demonstrated left hip dislocation and severe bilateral acetabular dysplasia. Arthrographic evaluation demonstrated no barriers to left hip concentric reduction on abduction. Profound acetabular dysplasia was identified as the main structural abnormality causing dislocation. Single-stage bilateral hip reconstruction was undertaken without performing an open reduction of the left hip. Bilateral femoral derotation-shortening osteotomies and Dega pelvic osteotomies were performed in a single stage. A hip spica was applied for six weeks before commencing mobilization. One year postoperatively, the child was weight-bearing. Both hips remained concentrically reduced with improvement in dysplastic…
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