Pediatric Pitfalls-Dr. Nguyen - Pediatric Pitfalls

Dr. Nguyen - Pediatric Pitfalls
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This document discusses musculoskeletal (MSK) issues in pediatric patients with neuromuscular diseases (NMD). It covers contributing factors to MSK conditions, common MSK conditions, prevention strategies, orthotic use, and therapy interventions.<br /><br />Contributing factors to MSK conditions in NMD include steroid use, deficiencies in hormones (testosterone and growth hormone), muscle loss, reduced mobility, and weight gain.<br /><br />Common MSK conditions in NMD include osteopenia/porosis (reduced bone mass), scoliosis (abnormal curvature of the spine), hip dysplasia, and contractures (permanent tightening of muscles and tendons).<br /><br />Osteopenia/porosis is characterized by reduced bone mass and can lead to fractures. Factors influencing bone health in NMD include genetics, endocrine function, lifestyle, and neurological factors. Steroid use in NMD can negatively impact bone health.<br /><br />Prevention strategies for MSK conditions include calcium and vitamin D supplementation, bisphosphates (not FDA approved in children), deflazacort (a steroid with bone-sparing activity), weight-bearing exercises, and weight reduction.<br /><br />Scoliosis, particularly in Duchenne muscular dystrophy (DMD), is strongly related to age. Steroid use may decrease the incidence of severe progressive scoliosis. Surgical management, such as spinal fusion, is common and aims to improve sitting position and comfort. Bracing is used to improve sitting stability but does not correct scoliosis.<br /><br />Hip dysplasia can affect children with NMD, particularly those with spinal muscular atrophy or Charcot Marie Tooth disease. Management options include conservative approaches (early standing and hip range of motion exercises) or surgical interventions (proximal varus osteotomy).<br /><br />Contractures are permanent tightening of soft tissue and are associated with wheelchair reliance. Conservative management involves early diagnosis, passive range of motion exercises, ankle and hamstring stretching, and splinting.<br /><br />Orthotic use can prevent contractures and enable feet to fit in shoes. However, the use of ankle-foot orthoses (AFOs) in DMD is not recommended as they restrict ankle strategies for walking and can lead to slower gait and falls. Orthotic approach varies based on the stage of the disease.<br /><br />Therapy interventions aim to prevent or delay contractures, establish home exercise programs, and assist with activities of daily living. Equipment such as standing devices, walkers, wheelchairs, and lifts can help with mobility and transfers.<br /><br />Getting equipment funded by insurance or government programs can be challenging. Medicaid may cover certain equipment, and charity resources can be considered if denied by funding sources.<br /><br />Overall, early recognition and management of MSK issues in pediatric patients with NMD are crucial for improving quality of life and preventing complications.
Keywords
musculoskeletal issues
pediatric patients
neuromuscular diseases
contributing factors
common conditions
prevention strategies
orthotic use
therapy interventions
osteopenia/porosis
scoliosis