Musculoskeletal and skin considerations in children with medical complexity: Common themes and approaches to management

Abstract

The term "children with medical complexity" (CMC) describes a population of heterogeneous genetic and metabolic disorders with multi-system involvement, with shared common abnormalities within the musculoskeletal system. Disorders that affect the central nervous system (CNS) result in abnormal muscular tone, ranging from dystonia and spasticity, to hypotonia, to combinations of both (axial hypotonia with appendicular hypertonia). Abnormal tone can impair movement and function and contribute to development of contractures, progressive scoliosis and hip dislocation. Impaired mobility, chronic illness and polypharmacy can result in fragile bones and increased risk of fracture, which can be difficult to diagnose and associated with pain. Additionally, CMC with impaired ability to communicate, total dependence on caregivers and frequent use of support devices can develop unintended skin injuries. There are a variety of treatments available for CMC with musculoskeletal disorders ranging from physical supports to pharmacologic treatments to surgeries. Medical and surgical treatments can promote changes in tone and support bone health to improve comfort, hygiene and proper positioning. These therapies may also carry significant risk in medically fragile patients. It is important for CMC to have a care team with a good understanding of the patient's overall health and goals of care and that can advocate for the patient to balance optimization of function and reduction of pain while also minimizing risk.