Treatment of Airway Obstruction in Pierre Robin Syndrome: A Modified Lip-Tongue Adhesion

Late presentation of upper airway obstruction in Pierre Robin sequence

“…Gilhooly et al reported that one infant had a normal initial polysomnogram but subsequently an abnormal study at 13 days of age with significant upper airway obstruction 8. In support of this, Smith also reported that respiratory obstruction in Pierre Robin sequence may be present at birth or may become progressively worse at 6 to 8 weeks of age 9. These findings suggest that the absence of clinical upper airway obstruction in the early neonatal period may not guarantee that obstruction will not develop.…”

Epidemiology of Robin sequence in the UK and Ireland: an active surveillance study

“…However, UAO in RS may not be diagnosed at birth and can progressively worsen over several weeks, or can be present intermittently, for example, when feeding or during supine sleep. Additionally, UAO can be subclinical, presenting with growth faltering which resolves after an airway intervention is introduced 3 5 7 27. Epidemiological studies that exclude cases without overt UAO in the neonatal period may consequently underestimate birth prevalence 8 9.…”

Robin sequence without cleft palate: Genetic diagnoses and management implications

“…The resulting list was cross‐referenced with an internal genetics craniofacial clinic patient list. Multiple studies have demonstrated that airway obstruction cannot reliably be diagnosed clinically (Anderson et al, 2011; Gilhooly et al, 1993; Smith, 1981; Wilson et al, 2000). Therefore, to ensure that the cohort members met 2016 consensus criteria for RS (micrognathia, glossoptosis, and airway obstruction), we only included patients with demonstration of obstructive apnea on polysomnography, unless severity of airway obstruction necessitated urgent intervention (Breugem et al, 2016).…”