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Sleep Disturbances in Pediatric Craniopharyngioma: A Systematic Review
Ramona Cordani1,2, Marco Veneruso1, Flavia Napoli3
1Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy.
Insights
Children with craniopharyngioma (CP) frequently experience sleep disorders, including excessive daytime sleepiness and narcolepsy, due to hypothalamic involvement. Understanding these mechanisms is key to developing effective therapeutic strategies.
Area of Science:
- Pediatric Neurology
- Sleep Medicine
- Endocrinology
Background:
- Craniopharyngiomas (CP) are rare sellar region tumors, common in children, often causing severe clinical issues due to hypothalamic involvement.
- The hypothalamus is vital for regulating bodily functions and the sleep-wake cycle, making its involvement in CP significant.
- Sleep disorders are a critical but often under-recognized comorbidity in pediatric craniopharyngioma patients.
Approach:
- A systematic literature review was conducted following PRISMA guidelines.
- Searched PubMed/MEDLINE and Scopus databases for peer-reviewed articles on childhood craniopharyngioma and sleep disorders.
- Included 32 articles focusing on sleep disturbances in pediatric CP patients.
Key Points:
- High prevalence of excessive daytime sleepiness (25-100%) in CP patients, with objective measures showing higher rates.
- Significant incidence of secondary narcolepsy (14-35%) and sleep-disordered breathing (4-46%) reported.
- Sleep-wake rhythm dysregulation is noted, with potential mechanisms including hypothalamic injury, hypocretin deficiency, and obesity.
Conclusions:
- Childhood craniopharyngioma frequently leads to diverse sleep disorders with significant impact.
- Pathophysiology involves hypothalamic damage affecting key regulatory pathways.
- Accurate diagnosis and management of these sleep disorders are crucial and challenging, necessitating a structured approach.
Abstract:
Craniopharyngiomas are rare brain tumors of the sellar region and are the most common non-neuroepithelial intracerebral neoplasm in children. Despite a low-grade histologic classification, craniopharyngiomas can have a severe clinical course due to hypothalamic involvement. The hypothalamus plays a crucial role in regulating vital functions, and it is a critical component of the sleep-wake regulatory system. This systematic review aims to provide an overview of the current knowledge on sleep disorders in patients with craniopharyngioma to unravel their underlying mechanisms and identify possible therapeutic strategies. A comprehensive electronic literature search of the PubMed/MEDLINE and Scopus databases was conducted in accordance with the PRISMA® statement. Extensively published, peer-reviewed articles involving patients with childhood craniopharyngioma and focused on this specific topic were considered eligible for inclusion. Thirty-two articles were included; a high prevalence of excessive daytime sleepiness was reported in CP patients, with wide variability (25-100%) depending on the diagnostic method of detection (25-43% by subjective measures, 50-100% by objective investigations). In particular, secondary narcolepsy was reported in 14-35%, sleep-disordered breathing in 4-46%. Moreover, sleep-wake rhythm dysregulation has been notified, although no prevalence data are available. Possible mechanisms underlying these disorders are discussed, including hypothalamic injury, damage to the suprachiasmatic nucleus, low melatonin levels, hypocretin deficiency, and hypothalamic obesity. The diagnosis and management of sleep disorders and associated comorbidities are challenging. This review summarizes the pathophysiology of sleep disorders in childhood-onset CP and the main treatment options. Finally, a possible diagnostic algorithm in order to accurately identify and treat sleep disorders in these patients is proposed.
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