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Published on: October 24, 2019
Polysomnographic findings in infants with Pierre Robin sequence
Abdullah Khayat1, Saadoun Bin-Hassan1, Suhail Al-Saleh1
1Department of Pediatrics, University of Toronto, Ontario, Canada; Department of Pediatrics, Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Nearly half of infants with Pierre Robin sequence (PRS) experience obstructive sleep apnea (OSA). This study highlights the high prevalence of OSA in infants with PRS, underscoring the need for further research.
Area of Science:
- Pediatric Sleep Medicine
- Genetics and Rare Diseases
Background:
- Pierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
- Infants with PRS frequently exhibit sleep-disordered breathing (SDB), including obstructive sleep apnea (OSA) and central sleep breathing abnormalities from birth.
Purpose of the Study:
- To determine the prevalence and severity of SDB in infants diagnosed with PRS.
- To utilize polysomnography (PSG) for a comprehensive evaluation of sleep breathing patterns in this population.
Main Methods:
- Retrospective analysis of sleep laboratory data from May 2007 to March 2016 at The Hospital for Sick Children, Toronto.
- Inclusion of patients with PRS and their initial polysomnography (PSG) records, with referral primarily due to OSA concerns.
- Statistical comparisons between OSA and non-OSA groups using t-tests, Wilcoxon signed ranks test, and Chi-squared tests.
Main Results:
- Out of 46 infants with PRS, 47% (22/46) showed evidence of OSA.
- OSA severity varied, with 10 mild, 3 moderate, and 9 severe cases identified.
- Infants with PRS and OSA were younger than those without OSA, and significant correlations were observed between desaturation/arousal indices and the obstructive apnea-hypopnea index.
Conclusions:
- This study confirms a high incidence of OSA among infants with Pierre Robin sequence.
- Further prospective, longitudinal research is recommended to understand the long-term outcomes of OSA in the PRS population.
Introduction:
Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and upper airway obstruction. It is commonly associated with the secondary cleft palate. Infants with PRS commonly have sleep-disordered breathing (SDB); including obstructive sleep apnea (OSA) as well as central sleep breathing abnormalities that are present from infancy.
Aim Of The Study:
Evaluate the prevalence and severity of SDB in infants with PRS using polysomnography (PSG).
Settings And Design:
We retrospectively reviewed the sleep laboratory database at The Hospital for Sick Children, Toronto, during the period of May 2007 to March 2016.
Statistical Analysis:
Comparisons of PSG data were made between the OSA and non-OSA group using the Student's t-test for age and body mass index, Wilcoxon signed ranks test for the continuous PSG data and Chi-squared test for the categorical variables.
Methods:
Patients with PRS were identified and their initial PSG was selected for this study. The main indication for referral was ongoing concerns regarding OSA symptoms.
Results:
A total of 46 patients (28 females) were included with a mean age (±standard deviation) of 0.8 (±0.3) year. Twenty-two out of 46 (47%) had evidence of OSA of which 10 had mild, 3 had moderate, and 9 had severe OSA. The PRS infants with OSA were younger than the non-OSA group. Significant correlations were found between desaturation and arousal indices with obstructive apnea-hypopnea index.
Conclusion:
This retrospective chart review confirms a high prevalence of OSA in this population. Prospective longitudinal studies are needed to evaluate the outcomes of OSA in PRS population.
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