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April 17, 2026JBJS Case Connector0 citations

Spastic Hip Dislocation in a Child With Aicardi-Goutières Syndrome

VSVivek Sodhai AWAmit WakekarSancheti Institute For Orthopaedics & Rehabilitation ABAbhijeet Botre

Key Points

To report a case of spastic hip dislocation in a child with Aicardi-Goutières syndrome and the surgical interventions used.
Clinical assessment of a 40-month-old girl with Aicardi-Goutières syndrome.
Radiographs to evaluate hip dislocation and acetabular dysplasia.
Surgical interventions included adductor tenotomy, open reduction, and varus derotation osteotomy.
Right hip dislocation and acetabular dysplasia were diagnosed.
Post-surgery, acetabular index improved from 42° to 26°.
Migration percentage improved from 100% to 31% at the 40-month follow-up.

Abstract

Case: A 40-month-old nonambulatory girl with Aicardi-Goutières syndrome presented with spastic dystonic quadriplegia and painful right hip dislocation. Radiographs showed right hip dislocation with acetabular dysplasia. She underwent adductor tenotomy, open reduction, and proximal femur varus derotation osteotomy, achieving stable hip containment. Acetabular index and migration percentage improved from 42° and 100% at presentation to 26° and 31%, respectively, at the 40-month follow-up. Conclusion: This is the first report of spastic hip dislocation in Aicardi-Goutières syndrome. The case underscores the importance of structured hip surveillance to detect early hip migration in children with severe spasticity and prevent late dislocation.

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