(iv) Development and treatment of spinal deformity in patients with neurological or myopathic conditions

Children and adolescents with neurological conditions develop severe spinal deformities at a high frequency due to muscle weakness and poor neuromuscular control, frequently associated with spasticity. Scoliosis is the most common type of deformity, occasionally associated with increased kyphosis or lordosis and commonly extending to the sacrum and pelvis. The development of severe spinopelvic imbalance can cause back or costo-pelvic impingement pain, affects the patient's ability to function, impacts on the patient's quality of life and maximizes the need for nursing care. It may give rise to significant respiratory compromise. Surgical correction is indicated in the presence of a progressive deformity that produces functional limitations and severe symptoms especially in growing children. This, by necessity, involves a major procedure with considerable risk of intraoperative and postoperative complications directly related to the severity of deformity, but also to the presence of associated co-morbidities and the degree of neurological disability. A thorough medical assessment is critical in order to establish each patient's suitability for such major surgery. A well-coordinated multidisciplinary approach will reduce the risks of significant morbidity and mortality, which can occur either during scoliosis surgery, often related to excessive blood loss, or post-surgery, mainly due to pulmonary complications, as well as the risk of infection and generalized sepsis in patients with inherently poor immunological response. Deformity correction and spinal fusion should only be performed in major centres with adequate medical support and by well-trained surgeons who are familiar with the surgical techniques in order to minimize the risk of complications and produce an optimum outcome. This review summarizes the current knowledge on neuromuscular deformities of the spine and provides a rational approach to the evaluation and management of this group of patients.