We reported two PRS cases that developed severe upper airway obstruction immediately after birth and were rescued by fiberoptic nasotracheal intubation. PRS refers to the association of micrognathia and glossoptosis and is characterized by varying degrees of upper airway obstruction, due mainly to glossoptosis. Usually, progressive airway obstruction might become more noticeable in the second month of life [
2]. In our cases, micrognathia and an extreme type of glossoptosis resulted in severe airway obstruction and difficult oropharyngeal intubation at birth. Based on fiberoptic endoscopy, mechanism of upper airway obstruction in PRS is classified into four types according to position of tongue or palate attaching to pharyngeal wall [
3]. None of the four types in this classification was applicable to a novel type of our cases. Extreme posterior shifting of the tongue is considered an obstacle in fusion of the bilateral palate in the early fetal stage, resulting in a cleft palate. In these cases, the tongue protruding into the nasal cavity occupied not only the pharynx, but also the nasal airway, resulting in severe dyspnea. To our knowledge, there have been no reports on PRS patients experiencing severe dyspnea immediately after birth due to critical airway obstruction and who were rescued by fiberoptic nasotracheal intubation.
Case 2 had a diagnosis of 1p36 deletion syndrome with low anorectal anomaly, severe hearing loss, and hypoplastic auricle, in addition to the airway anomalies. 1p36 deletion syndrome affects approximately 1 in 5000 newborns and is the most common terminal chromosomal deletion in humans [
4]. Medical problems commonly caused by this anomaly include developmental delay, intellectual disability, seizures, vision loss, short stature, distinctive facial features, brain anomalies, orofacial clefting, congenital heart defects, and renal anomalies. Case 2 had 1p36 deletion syndrome complicated with PRS. Guidelines for difficult airway management in neonatal cardiopulmonary resuscitation have not been established. Fiberoptic intubation is the gold standard for pediatric difficult airways. However, the American Society of Anesthesiologists suggested that during anesthetic induction for emergent tracheotomy, fiberoptic intubation is the second choice for patients in which oropharyngeal intubation is difficult [
5]. The anatomical features of infants include a cephalad larynx, fragile epiglottis, and a large tongue relative to the oropharyngeal space [
6]. Fiberoptic intubation allows a more straightforward pathway to the larynx and can compensate for these features. In our cases, it was difficult to predict the occurrence of severe upper airway obstruction before birth because fetal ultrasound findings were normal, except for polyhydramnios in Case 2. For cases in which airway compromise at birth cannot be predicted, preparation and acquisition of fiberoptic intubation equipment may be necessary for neonatologists.