Limb lengthening in down syndrome

Abstract

Introduction: The average life expectancy for Down syndrome patients has markedly improved. Musculoskeletal disorders occur in 20%–27% of patients with Down syndrome, with hip or knee instability among the most common presentations. Leg length discrepancy has rarely been reported in Down syndrome but can occur as the result of previous lower extremity surgery. This case report presents our experience with the first documented use of an intramedullary lengthening nail to correct a 4.5 cm iatrogenic leg length discrepancy in a skeletally mature Down syndrome patient. Materials and Methods: A single patient surgically treated for leg length discrepancy was followed over a 1-year period and retrospectively reviewed. Results: An 17-year-old male with Down syndrome presented with a 4.5 cm leg length discrepancy secondary to a previously attempted hip fusion. He had been treated with a shoe lift but had difficulty walking with the lift and resorted to toe walking whenever the lift was not being used. His mother, who was his full-time caregiver, was interested in surgical options for permanently correcting his discrepancy. After long discussions over multiple visits covering the risks and benefits of the various choices, the decision to proceed with lengthening of the short limb using a retrograde femoral intramedullary lengthening nail was made. Distraction osteogenesis of 4.5 cm with full consolidation was achieved in this patient without complications. He was able to return to full independent ambulation without a lift by 4 months. Conclusion: Limb lengthening requires a detailed postoperative protocol that may appear to be difficult for a Down syndrome patient with cognitive developmental delay to manage. However, this report documents that if the patient has a reliable caregiver and close postoperative monitoring is maintained, a successful outcome is possible. This represents the first published case of limb lengthening in a Down syndrome patient.