Home Management of Upper Airway Obstruction in Pierre Robin Sequence Using a Nasopharyngeal Airway

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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“…Discharge from the hospital with an NPA is now viewed as a safe and effective option for infants with RS in many institutions. 72,73 …”

Section: Figurementioning

confidence: 99%

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

et al. 2011

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“…114 This intervention can prevent more invasive operations. 115,116 The family needs to be educated regarding appropriate home care of this nasopharyngeal airway. Most children grow out of airway problems as the mandible develops; however, more invasive intervention, such as tracheostomy, may become necessary in those with persistent difficulty despite a nasopharyngeal airway.…”

Section: Airway: Pierre Robin Sequencementioning

confidence: 99%

Craniofacial Syndromes

Buchanan

Xue

Hollier

2014

Plastic and Reconstructive Surgery

167

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Craniofacial syndromes fall into two major categories-those associated with craniosynostosis, and those associated with clefts. Each has a different set of potential complications requiring a unique approach for surgical management. Craniosynostosis is a congenital disorder in which one or more of the cranial sutures fuses prematurely. The most common syndromes associated with this condition include Crouzon, Apert, Pfeiffer, Muenke, and Saethre-Chotzen syndromes. Surgical management of these children requires a multidisciplinary approach and close involvement of the family. Operations must take into consideration the growing potential of the bony structures. Common syndromes associated with clefts include Pierre Robin, Treacher Collins, Nager, Binder, and Stickler syndromes. Many of these children have severe airway issues requiring immediate address before operative reconstruction. As with syndromes associated with craniosynostosis, the key to management is a multidisciplinary approach focused on the right timing. The purpose of this article is to review the clinical presentation, care, and treatment of these patients.

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“…Parents are trained to maintain the airway at home. 47,52,54,55 Nasogastric (NG) tube feeds are administered until these kids are able to tolerate oral feeding.…”

Section: Diagnosis and Managementmentioning

confidence: 99%

“…This treatment is more likely to succeed with nonsyndromic patients than with syndromic patients. 46,54 Tongue-lip adhesion, popularized by Douglas, still has its strong advocates. 44,51,53,56Y59 Douglas reported a dramatic improvement in survival in his article in 1950 when compared with a group of untreated patients 44,57,60,61 (Fig.…”

Section: Diagnosis and Managementmentioning

confidence: 99%

Controversies in the Diagnosis and Management of the Robin Sequence

Mackay

2011

Journal of Craniofacial Surgery

122

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The Robin sequence, previously known as the Pierre Robin syndrome, is characterized by the sequence of clinical events that result from a small mandible. The tongue becomes posteriorly displaced (glossoptosis) and obstructs the airway. The obstructing tongue also makes oral feeding difficult and, in severe cases, impossible. Patients with Robin sequence may also present with a cleft palate, although this is not necessary to make the diagnosis. The small mandible either results from an inherent growth problem (genetic or syndromic) or is deformational where intrauterine growth of the mandible is restricted. Conditions of syndromic patients are generally more severe and difficult to treat. The phenomenon of "catch-up" growth is disputed, although it is likely to occur in deformational but not syndromic patients. Patients with Robin sequence are best managed by a multidisciplinary team familiar with the diagnoses and treatment of difficult pediatric airways. Approximately 70% of Robin infants are successfully managed by prone positioning alone. Tracheostomy is necessary in approximately 10% of these patients, especially for subglottic obstructions including laryngomalacia and tracheomalacia. Tracheostomy should be avoided if possible. Treatment of the remaining 20% remains highly contentious. Tongue-lip adhesion, nasopharyngeal airways, and distraction osteogenesis all have strong advocates. Ideally, treatment should be individualized. Patients who will have catch-up growth of the mandible will only need a tongue-lip or nasopharyngeal airway as a temporary measure. Patients who we know will not have catch-up growth will benefit from early distraction osteogenesis.

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Home Management of Upper Airway Obstruction in Pierre Robin Sequence Using a Nasopharyngeal Airway

Abstract: This series demonstrates the revised management protocol followed has reduced in-patient stays and remained effective, with infants continuing to thrive after discharge home, and has a low complication rate.

Cited by 53 publications

References 11 publications

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

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

Craniofacial Syndromes

Controversies in the Diagnosis and Management of the Robin Sequence

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