Single-Stage Bilateral Hip Reconstruction for Kabuki Hip Dysplasia in a Four-Year-Old: A Case Report

TL;DR

A four-year-old with Kabuki syndrome underwent successful single-stage bilateral hip reconstruction without open reduction, achieving improved mobility and hip stability.

Contribution

This is the first documented case of single-stage bilateral hip reconstruction for Kabuki syndrome without open reduction of the dislocated hip.

Findings

Single-stage bilateral hip reconstruction achieved concentric reduction and improved dysplasia in a Kabuki syndrome patient.

The child became weight-bearing one year post-surgery with satisfactory clinical and radiological outcomes.

The procedure avoided open reduction of the left hip and used femoral and pelvic osteotomies for correction.

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 features. This report describes the successful management of hip dysplasia secondary to Kabuki syndrome using a novel approach. Open reduction with capsular plication of the dislocated hip was obviated, and both hips were corrected in a single operation with satisfactory clinical and radiological outcomes at one year.

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