Neurosurgical management of elevated tone in childhood: interventions, indications and uncertainties

Elevated tone (hypertonia) is a common problem in children with physical disabilities. Medications intended to reduce tone often have limited efficacy, with use further limited by a significant side effect profile. Consequently, there has been growing interest in the application of Neurosurgical Interventions for the Management of Posture and Tone (NIMPTs). Three main procedures are now commonly used: selective dorsal rhizotomy (SDR), intrathecal baclofen (ITB) and deep brain stimulation (DBS). This review compares these interventions, along with discussion on the potential role of lesioning surgery. These interventions variably target spasticity and dystonia, acting at different points in the distributed motor network. SDR, an intervention for reducing spasticity, is most widely used in carefully selected ambulant children with cerebral palsy. ITB is more commonly used for children with more severe disability, typically non-ambulant, and can improve both dystonia and spasticity. DBS is an intervention which may improve dystonia. In children with certain forms of genetic dystonia DBS may dramatically improve dystonia. For other causes of dystonia, and in particular dystonia due to acquired brain injury, improvements following surgery are more modest and variable. These three interventions vary in terms of their side-effect profile and reversibility. There are currently populations of children for who it is unclear which intervention should be considered (SDR vs ITB, or ITB vs DBS). Concerns have been raised as to the equity of access to NIMPTs for children across the UK, and whether the number of surgeries performed each year meets the clinical need.