Management of Infants With Pierre Robin Sequence

Wagener, Silke; Rayatt, Sukh; Tatman, Andy J.; Gornall, P.; Slator, Rona

doi:10.1597/1545-1569(2003)040<0180:moiwpr>2.0.co;2

Cited by 99 publications

(46 citation statements)

References 23 publications

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“…Wagener et al reported successful outcomes in 20 children with PRS. In their study, the children required the airway for 16–104 days, and unfortunately this entire time was spent in hospital14 as processes to discharge children home with NPAs were not in place. Meyer et al in their study used NPAs in 29 of 38 children and found it successful in 48% 3…”

Section: Discussionmentioning

confidence: 99%

The successful use of the nasopharyngeal airway in Pierre Robin sequence: an 11-year experience

et al. 2012

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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.

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Section: Discussionmentioning

confidence: 99%

The successful use of the nasopharyngeal airway in Pierre Robin sequence: an 11-year experience

et al. 2012

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“…70,71 Transitioning home with a stable NPA in place is possible when parents become comfortable with tube care and equipment. Discharge from the hospital with an NPA is now viewed as a safe and effective option for infants with RS in many institutions.…”

Section: Figurementioning

confidence: 99%

Robin Sequence: From Diagnosis to Development of an Effective Management Plan

et al. 2011

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The triad of micrognathia, glossoptosis, and resultant airway obstruction is known as Robin sequence (RS). Although RS is a well-recognized clinical entity, there is wide variability in the diagnosis and care of children born with RS. Systematic evaluations of treatments and clinical outcomes for children with RS are lacking despite the advances in clinical care over the past 20 years. We explore the pathogenesis, developmental and genetic models, morphology, and syndromes and malformations associated with RS. Current classification systems for RS do not account for the heterogeneity among infants with RS, and they do not allow for prediction of the optimal management course for an individual child. Although upper airway obstruction for some infants with RS can be treated adequately with positioning, other children may require a tracheostomy. Care must be customized for each patient with RS, and health care providers must understand the anatomy and mechanism of airway obstruction to develop an individualized treatment plan to improve breathing and achieve optimal growth and development. In this article we provide a comprehensive overview of evaluation strategies and therapeutic options for children born with RS. We also propose a conceptual treatment protocol to guide the provider who is caring for a child with RS. Pediatrics 2011;127:936-948

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“…PRS often develops upper airway obstruction or feeding difficulty secondary to micrognathia, glossoptosis, or a shifted tongue that comes in contact with the pharyngeal wall [2]. Usually, progressive airway obstruction might become more noticeable in the second month of life.…”

Section: Introductionmentioning

confidence: 99%

Case report of Pierre Robin sequence with severe upper airway obstruction who was rescued by fiberoptic nasotracheal intubation

et al. 2017

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BackgroundPierre 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.Case presentationThe 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.ConclusionWe 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.

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Management of Infants With Pierre Robin Sequence

Abstract: Relieving airway obstruction by NPA is an effective and safe treatment for babies with PRS until they have grown out of their respiratory and feeding difficulties. It avoids the need for surgery and can be used on neonatal wards using the monitoring described.

Cited by 99 publications

References 23 publications

The successful use of the nasopharyngeal airway in Pierre Robin sequence: an 11-year experience

The successful use of the nasopharyngeal airway in Pierre Robin sequence: an 11-year experience

Robin Sequence: From Diagnosis to Development of an Effective Management Plan

Case report of Pierre Robin sequence with severe upper airway obstruction who was rescued by fiberoptic nasotracheal intubation

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