The two patients with observable deficit had their instrumentation left in situ after discussion with them and/or parents. Spinal cord monitoring in this population is possible but potentially unreliable. Surgeons will need to carefully consider the use of monitoring in their management of this challenging population.
Scoliosis surgery in patients with Rett syndrome carries a very high rate of complications and an average hospital stay approaching 3 weeks. Both caregivers and surgeons should be aware of this when planning any intervention. These patients frequently have useful lower limb function and spinal cord monitoring is a valid tool to aid in its preservation. We would suggest aggressive optimisation of these patients prior to surgery, with an emphasis on nutrition.
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