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Published on: December 22, 2016
Central Sleep Apnea and Hypoventilation Disorders in Children
Indra Narang1, Chun Ting Au2, Maida Lynn Chen3
1Department of Pediatrics, The Hospital for Sick Children, 175 Elizabeth Street, Toronto, Ontario, M5G 2G3, Canada.
Insights
This chapter details pediatric central sleep apnea and hypoventilation disorders, focusing on complex medical conditions and genetic syndromes like CCHS and ROHHAD. It offers insights into causes, diagnosis, and evidence-based management strategies for affected children.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Genetics
Background:
- Central sleep apnea (CSA) and central hypoventilation disorders affect children, particularly those with complex medical needs.
- Genetic syndromes like congenital central hypoventilation syndrome (CCHS) and rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome are key areas of focus.
- Related conditions include Prader-Willi syndrome and hypoventilation due to structural issues like Chiari malformations.
Purpose of the Study:
- To provide a comprehensive overview of pediatric CSA and central hypoventilation disorders.
- To emphasize the impact of complex medical conditions and genetic syndromes on these respiratory disorders.
- To review current knowledge and offer evidence-based management guidance.
Main Methods:
- Literature review and synthesis of current knowledge.
- Focus on pathogenesis, epidemiology, clinical presentation, and diagnostic evaluation.
- Examination of management and treatment strategies.
Main Results:
- Detailed overview of pediatric CSA and central hypoventilation disorders.
- Emphasis on specific genetic syndromes (CCHS, ROHHAD) and related conditions.
- Compilation of current understanding of disease mechanisms and clinical features.
Conclusions:
- Effective management requires understanding the specific disorder, its genetic basis, and clinical presentation.
- Evidence-based guidance is crucial for optimizing treatment outcomes in affected children.
- Further research into pathogenesis and treatment is warranted.
Abstract:
This chapter presents a comprehensive overview of central sleep apnea (CSA) and central hypoventilation disorders in the pediatric population. It places particular emphasis on children with complex medical conditions and genetic syndromes, including congenital central hypoventilation syndrome (CCHS) and rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome.In addition, the chapter examines related conditions such as Prader-Willi syndrome and other hypoventilation disorders arising from structural or functional abnormalities, including Chiari malformations and Leigh syndrome. It reviews current knowledge of the pathogenesis, epidemiology, clinical presentation, and diagnostic evaluation of these disorders in children, and concludes with evidence-based guidance for their management and treatment.
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