Gastrointestinal and nutritional care in pediatric neuromuscular disorders

Abstract

Neuromuscular diseases (NMDs) affect the development and growth of the neuromuscular system in children. The pathology can occur anywhere along the neuromuscular pathway, from the brain to the nerves to the muscle fibers. These diseases have a profound impact on the quality of life not only of children but also of their families. The predominant manifestation in NMDs is hypotonia, which leads to muscle weakness and fatigue, reduced mobility, and decreased physical performance. However, multiple organ systems can be affected, with resulting orthopedic, cardiac, infectious, respiratory, and nutritional problems. Children with NMD present an increased risk for several dietary and feeding difficulties because of their neuromuscular diagnosis, presentation, and severity. These problems include chronic gastrointestinal issues (constipation, dysphagia, gastroesophageal reflux, and diarrhea), dysphagia, malnutrition, and body composition alterations. As a result, compared to the overall pediatric population, infants and children with NMD are more likely to be malnourished, ranging from failure to thrive to overweight or obesity. Disease-specific guidelines vary in level of detail and recommendations for dietary management. Overall, nutritional data available are sparse, with the exception of Duchenne muscular dystrophy, spinal muscular atrophy, and congenital muscular dystrophy. The purpose of this review is to describe the spectrum of nutritional challenges in children with NMD and to summarize the main dietary and gastrointestinal recommendations for each neuromuscular disorder to provide guidance for daily clinical practice.