Screening Sleep Abnormalities in Children with Prader–Willi Syndrome: Challenges in the Indian Scenario

Abstract

There is a high prevalence of sleep-related breathing disorders in the form of obstructive and central sleep apnea as well as spontaneous oxygen desaturation in children with Prader–Willi syndrome (PWS). Most cases are asymptomatic and if untreated go on to develop unfavorable neurodevelopmental, cardiovascular, and cerebrovascular outcomes. Hence, sleep study or polysomnography (PSG) is recommended in all children at the time of diagnosis as well as with the development of certain risk factors including symptoms of sleep apnea, before and after initiation of recombinant growth hormone (rGH) therapy. The use of rGH in children with PWS has been shown to improve central sleep apnea but also shown to be associated with worsening of OSA. PSG is ideally performed in a sleep laboratory. Various types of PSG devices are available depending on the biological parameters that are desired to be monitored. Sleep disorders in children are distinct from those seen in adults and have different diagnostic scoring criteria necessitating a trained pediatric sleep specialist to analyze the PSG recording. Through the clinical case vignette of a 14-year-old girl with PWS, severe obesity, and sleep disordered breathing, this review aims to highlight the need, timing, types, analysis, and interpretation of sleep studies in infants and older children with PWS, particularly in relation to rGH therapy. There is a paucity of literature on sleep studies in children with PWS in the local setting. Thus this review also suggests the need for adapting the existing Western guidelines for PSG in Indian children with PWS.