A New Grading of Pierre Robin Sequence

Cole, Annie; Lynch, Patricia; Slator, Rona

doi:10.1597/07-129.1

Cited by 76 publications

(47 citation statements)

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“…A possibility for the lack of correlation between mandible size and airway obstruction is the presence of additional lower airway anomalies, a common finding in prior studies. 32,33 Classification systems based on airway obstruction and feeding difficulties 34 are helpful but are not statistically associated with any outcome measures. Furthermore, feeding difficulties can be related to obstructive breathing patterns or to an underlying syndrome or disease state outside the tongue base and jaw.…”

Section: Discussionmentioning

confidence: 99%

Robin Sequence

Costa

Murage

et al. 2014

Plastic and Reconstructive Surgery

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Section: Discussionmentioning

confidence: 99%

Robin Sequence

Costa

Murage

et al. 2014

Plastic and Reconstructive Surgery

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“…6,62,100 Cole et al 101 suggested a unique classification system for RS based on functional parameters: severity of airway obstruction, breathing, and feeding difficulties. Care and caution must be exercised when interpreting recommendations for the whole group, because morphologic phenotype, airway physiology, and severity of RS vary.…”

Section: Conclusion and Offeringmentioning

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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“…A wide range of clinical manifestations exists, but the main clinical problems faced by clinicians include upper airway obstruction and feeding difficulties [7]. …”

Section: Introductionmentioning

confidence: 99%

Mandibular distraction in neonates: indications, technique, results

et al. 2012

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BackgroundThe Pierre Robin Sequence features were first described by Robin in 1923 and include micrognathia, glossoptosis and respiratory distress with an incidence estimated as 1:8,500 to 1:20,000 newborns. Upper airway obstruction and feeding difficulties are the main concerns related to the pathology. Mandibular distraction should be considered a treatment option (when other treatments result inadequate).Patiants and methodsTen patients between the ages of 1 month and 2 years with severe micrognathia and airway obstruction were treated with Mandibular Distraction Osteogenesis (MDO).All patients underwent fibroscopic examination of the upper airway and a radiographic imaging and/or computed tomography scans to detect malformations and to confirm that the obstruction was caused by posterior tongue displacement. All patients were evaluated by a multidisciplinary team. Indications for surgery included frequent apneic episodes with severe desaturation (70%). Gavage therapy was employed in all patients since oral feeding was not possible. The two tracheotomy patients were 5 months and 2 years old respectively, and the distraction procedure was performed to remove the tracheotomy tube. All patients were treated with bilateral mandibular distraction: two cases with an external multivector distraction device, six cases with an internal non-resorbable device and two cases with an internal resorbable device. In one case, the patient with Goldenhar's Syndrome, the procedure was repeated.ResultsThe resolution of symptoms was obtained in all patients, and, when present, tracheotomy was removed without complications. Of the two patients with pre-existing tracheotomies, in the younger patient (5 months old) the tracheotomy was removed 7 days postoperatively. In the Goldenhar's syndrome case (2 years old) a Montgomery device was necessary for 6 months due to the presence of tracheotomy-inducted tracheomalacia. Patients were discharged when the endpoint was obtained: symptoms and signs of airway obstruction were resolved, PAS and maxillomandibular relationship improved, and tracheotomy, when present, removed. During the follow-up, no injury to the inferior alveolar nerve was noted and scarring was significant in only the two cases treated with external devices.ConclusionMandibular Distraction Osteogenesis is a good solution in solving respiratory distress when other procedures are failed in paediatric patients with severe micrognatia.

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A New Grading of Pierre Robin Sequence

Abstract: Since implementation, this new classification of babies with PRS into three grades has clarified care pathways and enhanced communication between the Cleft Clinical Nurse Specialists and other health professionals involved with the care of these babies.

Cited by 76 publications

References 7 publications

Robin Sequence

Robin Sequence

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

Mandibular distraction in neonates: indications, technique, results

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