Hip Displacement in SMA

UK Consensus Statements on Hip Displacement Management in Spinal Muscular Atrophy in the Era of Disease-Modifying Therapies

1. An effort should be made to prevent hip dislocation in children with SMA; this can be more relevant when expected to achieve higher motor abilities (i.e assisted or unassisted standing/walking).

2. Annual radiographic hip surveillance, starting ideally between 6 - 18 months, is recommended for children with SMA.

3. Radiographic hip surveillance can be adjusted to minimise radiation exposure, as we learn more about the natural history of hip displacement in SMA and its prevention and treatment.

4. Radiographic signs to check on Hip X-rays: Hip displacement (Reimer’s Migration Percentage), acetabular dysplasia (Acetabular Index) and Head-Shaft Angle.

5. Multidisciplinary Team Assessment: Children with SMA should undergo assessment in a tertiary referral setting, by a multidisciplinary team (MDT) consisting of an orthopaedic surgeon, a neurologist and a physiotherapist.

6. Inclusion in Registry: All relevant clinical information regarding orthopaedic and radiological assessments should be documented and included

7. SMA is not a contraindication to hip surgery per se: A case by case risk/benefit approach should be in place.

8. Guidelines for Surgical Interventions: An effort should be made to develop evidence-based guidelines around Hip Surgical Interventions in children with SMA, and this should be conducted as part of audit strategies and research activity.

9. Guided Growth hip surgery may be considered for selected patients following regional MDT case by case discussion.

10. Treatment of Painful Displacements: Children with painful displaced hips should be considered for treatment, unless contraindicated.

11. Steroid hip injections can be considered in the management of hip pain for both diagnosis and treatment.

12. Indication for Major Surgery: Major hip surgery may be considered as a treatment option for selected children with painful displaced hips following MDT assessment.

13. Management of Concomitant Contractures: Surgically treating any concomitant muscle or joint contractures should be considered on a case by case basis particularly for those who are achieving higher motor milestones (stander with or without AFOs/KAFOs and use of hands, or ambulant).