Validation of pediatric sleep questionnaire in children with Chiari malformation and/or spina bifida with or without myelomeningocele.

Sleep-disordered breathing (SDB) is common in Chiari Malformation (CM) and Spina Bifida (SB) and can lead to adverse consequences if untreated. Therefore, screening is very important but the Pediatric Sleep Questionnaire (PSQ) has not been validated in this population. Further, there is limited data on the validity of this tool in children with central nervous system malformations. Retrospective chart review of CM and SB patients evaluated in our sleep center between 2008 and 2018. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the PSQ and several of its components were calculated to predict obstructive sleep apnea (OSA). A total of 149 patients met criteria for analysis. The majority were referred to a sleep specialist due to concern for SDB. OSA was found in 36% (53/149) of all patients. The sensitivity and specificity of the PSQ to predict OSA was 73.58% and 20.83%, respectively. The PPV was 33.91%, and the NPV was 58.82%. Specificity values were higher for PSQ as negative predictors of moderate or severe OSA. In this population, the sensitivity of PSQ for OSA is reasonable but lower than values described in other populations. The specificity and NPV are low. Even with a high prevalence of OSA, symptoms of SDB may overlap with those of other comorbidities leading to a low specificity. A PSQ could be used to prioritize which patients need a PSG more urgently than others. Further studies are needed to define an optimal cut-off value of the PSQ in this population.