Measures of nocturnal oxyhemoglobin desaturation in children with neuromuscular disease or Prader-Willi syndrome.

Abstract

Evidence for nocturnal oximetry interpretation in patients with abnormal neuromuscular function is limited. We aimed to compare children with neuromuscular disease (NMD) or Prader-Willi syndrome (PWS) to otherwise healthy subjects with obstructive sleep-disordered breathing (SDB) or without respiratory disorder (controls) regarding nocturnal oximetry parameters. We analyzed recordings from children with: (a) NMD; (b) PWS; (c) snoring and adenotonsillar hypertrophy and/or obesity (SDB); and (d) controls. Outcomes included: (a) basal SpO2 ; (b) proportions of subjects with McGill oximetry score (MOS) >1 (clusters of desaturations); and (c) desaturation index (SpO2 drops ≥3%/h-ODI3). Data of 12 subjects with NMD (median age, 5.2 years; IQR, 2.7, 8.2), 14 children with PWS (5 years; 2.3, 6.9), 21 children with SDB (5.8 years; 4.6, 9.6), and 20 controls (6.2 years; 5.4, 11.2) were analyzed. Children with NMD, PWS, and SDB had lower basal SpO2 than controls (95.6% [94.5%, 96.9%], 96.2% [95.1%, 97.4%], 96.1% [95.8%, 97.5%] vs 97.8% [97.2%, 97.9%], respectively; (P < .01). NMD and PWS showed the greatest negative effect on basal SpO2 (P < .05). Children with SDB or PWS had a higher risk of MOS >1 than patients with NMD (OR, 25.9 [95% CI, 3.4-200.4] and 9.5 [1.5-62.6]). NMD, PWS, and SDB were similar regarding ODI3, which was elevated compared to ODI3 in controls (P < .05). Frequent desaturations predominated in NMD, while periods of sustained desaturation were noted in NMD and PWS. PWS and NMD have a negative effect on basal SpO2 , while clusters of desaturations are prevalent in patients with PWS or obstructive SDB.