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ObjectiveTo test the hypothesis that children with Prader-Willi syndrome (PWS) and obstructive sleep apnoea syndrome (OSAS) have hypercapnia for higher proportion of total sleep time (TST) than non-syndromic children with similar obstructive apnoea–hypopnoea index (OAHI).DesignCross-sectional study.SettingTwo tertiary care hospitals.PatientsPatients with PWS and non-syndromic children with snoring who underwent polygraphy and were of similar age, body mass index (BMI) z-score and OAHI.Main outcome measureThe two groups were compared regarding %TST with transcutaneous CO2 (PtcCO2) >50 mm Hg. The interaction between PWS diagnosis and OSAS severity (OAHI 5 episodes/h) regarding %TST with PtcCO2 >50 mm Hg was tested using multiple linear regression.Results48 children with PWS and 92 controls were included (median age 2.3 (range 0.2–14.1) years vs 2.2 (0.3–15.1) years; BMI z-score 0.7±1.9 vs 0.8±1.7; median OAHI 0.5 (0–29.5) episodes/h vs 0.5 (0–33.9) episodes/h; p>0.05). The two groups did not differ in %TST with PtcCO2 >50 mm Hg (median 0% (0–100%) vs 0% (0–81.3%), respectively; p>0.05). However, the interaction between PWS and OSAS severity with respect to duration of hypoventilation was significant (p2 >50 mm Hg between children with PWS and controls for OAHI 5 episodes/h were +0.2%, +1% and +33%, respectively.ConclusionIncreasing severity of upper airway obstruction during sleep in children with PWS is accompanied by disproportionately longer periods of hypoventilation when compared with non-syndromic children with similar frequency of obstructive events.

We read with interest the article by Leong et al on the use of polysomnography (PSG) in children,1 covering indications for PSG, along with limitations of oximetry, and clearly outlining how to undertake and interpret PSG in paediatric patients. It briefly discusses limited channel recordings (respiratory polygraphy, RP) and concludes that this ‘is not standard practice’. In many paediatric centres RP is standard practice, and routinely used for assessment of sleep-disordered breathing (SDB) in children, with the most common diagnosis being obstructive sleep apnoea (OSA). In a recent survey …

Pierre Robin sequence (PRS) is a congenital anomaly presenting with micrognathia, glossoptosis and a cleft palate. This study describes a decade's experience of the management of upper airway obstruction (UAO) in PRS patients with a nasopharyngeal airway (NPA).This study was conducted by paediatric respiratory and otolaryngology departments. Children with PRS referred with UAO were evaluated according to a standard protocol. Data collected included the degree of airway obstruction, method of airway management, polysomnography data before and after intervention, and longer term follow-up.Data were collected on 104 PRS patients referred to us for airway assessment in 2000-2010. 64/104 were aged4 weeks at referral. Airway symptoms were managed conservatively in 27 patients (25.9%), with an NPA in 63 (60.6%) and a tracheostomy in 14 (13.4%). The average duration of NPA use was 8 months (3 weeks to 27 months). Polysomnography results improved in all 63 patients with an NPA. Fourteen severely obstructed patients underwent a tracheostomy. 86.5% (90/104) of PRS patients were managed conservatively or with the help of an NPA. There were no NPA related complications.There is a spectrum of UAO in PRS. This study reports on long-term outcomes in 104 children with PRS and airway obstruction. In most children (86.5%), airway obstruction was managed by conservative measures or with an NPA for a few months. The natural history shows that with normal growth, airway compromise resolves without immediate surgical intervention as advocated by some practitioners. Few PRS children require a tracheostomy.