Pierre Robin sequence causes position-dependent obstructive sleep apnoea in infants

Abstract

IntroductionObstructive sleep apnoea (OSA) and feeding difficulties are key problems for Pierre Robin sequence (PRS) infants. OSA management varies between treatment centres. Sleep positioning represents the traditional OSA treatment, although its effectiveness remains insufficiently evaluated.DesignTo complete a polysomnographic (PSG) evaluation of effect of sleep position on OSA in PRS infants less than 3 months of age. We analysed a 10-year national reference centre dataset of 76 PRS infants. PSG was performed as daytime recordings for 67 in the supine, side and prone sleeping position when possible. In most cases, recording included one cycle of non-rapid eye movement (NREM) and rapid eye movement (REM) sleep in each position.ResultsOne-third of infants (9/76, 12%) had severe OSA needing treatment intervention prior to PSG. During PSG, OSA with an obstructive apnoea and hypopnoea index (OAHI) >5 per hour was noted in 82% (55/67) of infants. OSA was most severe in the supine and mildest in the side or in the prone positions. The median OAHI in the supine, side and prone positions were 31, 16 and 19 per hour of sleep (p=0.003). For 68% (52/67) of the infants, either no treatment or positional treatment alone was considered sufficient.ConclusionsThe incidence of OSA was 84% (64/76) including the nine infants with severe OSA diagnosed prior to PSG. For the most infants, the OSA was sleep position dependent. Our study results support the use of PSG in the evaluation of OSA and the use of sleep positioning as a part of OSA treatment.

Obstructive sleep apnea in young infants: Sleep position dependence and spontaneous improvement

“…The nature of OSA in most infants studied was different from that of infants with a clear anatomical predisposal to OSA such as PRS. On contrasts to infants with PRS, most of these study infants did not show clear evidence of partial upper airway obstruction with increased WOB, as evident in chin or diaphragm EMG but evidence more of separated obstructive apneas and hypopneas in REM‐sleep 2 …”

“…In the current study, we focused on infants in whom the appearance of OSA was unexpected, based on infants' general appearance or facial structure. In syndromes such as PRS or laryngomalacia, the natural course of OSA is likely to be specific to the syndrome or defect 2,4 …”

“…In our current study infants, our clinical judgment on treatment modality was based on the number and degree of airway obstruction, the presence and degree of SpO 2 desaturations, the estimated WOB, on growth, or on the sleep‐position dependency of OSA (Figure 4). 16 In PRS infants, side‐ or prone‐sleep positioning is a widespread treatment for OSA 2,30 . Since the degree of OSA was worse in the supine and better in the side‐sleeping position, we introduced side‐sleep positioning as OSA treatment for the majority (59%) of patients, together with home pulse‐oximeter monitoring (Figure 5).…”

“…In most infant groups, infant OSA seems to be alleviated by aging. In PRS patients, OSA is often significantly alleviated by age 3−4 months following the forward growth of a retrognathic mandible 2 . Moreover, in other patient groups with a clear anatomical predisposal to OSA, improvement of OSA often occurs by the age of 8 months 20 .…”

Humidified versus nonhumidified low-flow oxygen therapy in children with Pierre-Robin syndrome: A randomized controlled trial

“…The incidence of Pierre-Robin syndrome varies from 1/8500~1/14,000. [ 8 , 9 ] Pierre-Robin syndrome mainly manifests as mandibular deformity, tongue that’s placed further back toward the throat, cleft palate, difficulty in breathing air, and limited humidification space for inhaled air. [ 10 ] Children with Pierre-Robin syndrome generally may not tolerate milk intake and may experience breathing problems.…”