title: Case report of Pierre Robin sequence with severe upper airway obstruction who was rescued by fiberoptic nasotracheal intubation.
abstract: Pierre Robin sequence (PRS) refers to the association of micrognathia, glossoptosis, and airway obstruction. Cases with severe dyspnea due to upper airway obstruction immediately after birth are very rare. We here report two cases with PRS who developed severe dyspnea due to morphological abnormality immediately after birth and were rescued by fiberoptic nasotracheal intubation.
  
  
  
  The patient in case 1 had micrognathia and cleft palate, and his tongue protruded into the nasal cavity via a cleft palate. His invaginated tongue was considered an extreme type of glossoptosis and he was diagnosed as Pierre Robin sequence. The patient in case 2 also had micrognathia and cleft palate same as case 1 and accompanied some anomalad. Her chromosome analysis confirmed a diagnosis of 1p36 deletion syndrome and she diagnosed as 1p36 deletion syndrome complicated with Pierre Robin sequence. In both cases, tongue protruded into the nasal cavity via a cleft palate occupied pharynx and nasal cavity, resulting in severe dyspnea. Only the backside of the tongue was visible by laryngoscopy and oropharyngeal intubation was impossible. Therefore, fiberoptic nasotracheal intubation was done to secure the airway for resuscitation.
  
  
  
  We conclude that extreme type of glossoptosis in PRS concludes tongue invaginated into nasal cavity which have not reported before and that such cases require resuscitation by fiberoptic intubation immediately after birth. As such, neonatologists should obtain the skill of fiberoptic intubation.
authors.affiliation: Perinatal and Neonatal Center, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Takeshita
authors.affiliation: Perinatal and Neonatal Center, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Ueda
authors.affiliation: Perinatal and Neonatal Center, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan.
authors.forename: Tatenobu
authors.initials: T
authors.lastname: Goto
authors.affiliation: Perinatal and Neonatal Center, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Muto
authors.affiliation: Perinatal and Neonatal Center, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Kakita
authors.affiliation: Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8560, Aichi, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Oshima
authors.affiliation: Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8560, Aichi, Japan.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Tainaka
authors.affiliation: Oral Surgery Department, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ono
authors.affiliation: Oral Surgery Department, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kazaoka
authors.affiliation: Perinatal and Neonatal Center, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Aichi, Japan. yamada.yasumasa.387@mail.aichi-med-u.ac.jp.
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Yamada
mesh_terms: D000402:Airway Obstruction / Q000209:etiology / Q000628:therapy*; D005260:Female; D005336:Fiber Optic Technology; D006801:Humans; D007231:Infant, Newborn; D007442:Intubation, Intratracheal / Q000379:methods*; D008297:Male; D010855:Pierre Robin Syndrome / Q000150:complications*
keywords: Airway obstruction; Case report; Fiberoptic nasotracheal intubation; Glossoptosis; Micrognathia; Pierre Robin sequence