Management of Airway Obstruction in Infants With Pierre Robin Sequence

Runyan, Christopher M.; Uribe-Rivera, Armando; Tork, Shahryar; Shikary, Tasneem; Ehsan, Zarmina; Weaver, K. Nicole; Hossain, Monir; Gordon, Christopher B.; Pan, Brian S.

doi:10.1097/gox.0000000000001688

Cited by 29 publications

(37 citation statements)

References 33 publications

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“…Since the publication of a seminal paper by Sher in 1992, it has been understood that although micrognathia-associated glossoptosis underlies the airway obstruction in all patients with PRS to some extent, clinical airway obstruction is determined by a number of causes, including neurologic dysfunction, rate of neuromuscular maturation, and laryngomalacia (Randall et al, 1965; Sher, 1992; Morice et al, 2018). Additionally, Runyan et al (2018) published a retrospective analysis of 171 patients with PRS and found that the most significant factor predictive of surgical intervention was a high obstructive index on preintervention sleep study; the authors noted no differences in micrognathia between patients managed conservatively and those managed surgically ( P = 1.000) (Runyan et al, 2018). Morice et al (2018) have also reported that grades of retrognathia and glossoptosis are not predictive of respiratory and feeding disorder severity in both patients with syndromic and nonsyndromic PRS (Morice et al, 2018).…”

Section: Discussionmentioning

confidence: 99%

Quantification of Mandibular Morphology in Pierre Robin Sequence to Optimize Mandibular Distraction Osteogenesis

Volk

Davis

Narawane

et al. 2020

The Cleft Palate Craniofacial Journal

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Background: Mandibular distraction osteogenesis (MDO) is the primary surgical intervention to treat airway obstruction in Pierre Robin sequence (PRS). Current morphologic studies of PRS mandibles do not translate into providing airway management decisions. We compare mandibles of infants with nonsyndromic PRS to controls characterizing morphological variances relevant to distraction. We also examine how morphologic measurements and airway grades correlate with airway management. Methods: Patients with PRS under 2 months old were age and sex matched to controls. Demographic and perioperative data, and Cormack-Lehane airway grades were recorded. Computed tomography scans were used to generate mandibular models. Bilateral condylions, gonions, and the menton were identified. Linear and angular measurements were made. Wilcoxon rank sum and 2-sample t tests were performed. Results: Twenty-four patients with PRS and 24 controls were included. Seventeen patients with PRS required MDO. PRS patients had shorter ramus heights (16.7 vs 17.3 mm; P = .346) and mandibular body lengths (35.3 vs 39.3 mm; P < .001), more acute gonial angles (125.3° vs 131.3°; P < .001), and more obtuse intergonial angles (94.2° vs 80.4°; P < .001) compared to controls. No significant differences were found among patients requiring MDO versus conservative management nor among distracted patients with high versus low airway grades. Conclusion: Our study examines the largest and youngest PRS population to date regarding management of early airway obstruction with MDO. Our findings indicate that univector mandibular body distraction allows for normalization in nonsyndromic patients with PRS, and airway obstruction management decisions should remain clinical.

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Section: Discussionmentioning

confidence: 99%

Quantification of Mandibular Morphology in Pierre Robin Sequence to Optimize Mandibular Distraction Osteogenesis

Volk

Davis

Narawane

et al. 2020

The Cleft Palate Craniofacial Journal

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“…More recently published studies about RS tend not to require CP for inclusion, likely reflecting expert consensus guidelines emphasizing this point (Breugem et al, 2016). In cohorts ascertained according to the 2016 consensus criteria, prevalence of CP ranges from 43% to 92% (Basart et al, 2015; Daniel et al, 2013; Flores et al, 2015; Runyan et al, 2018). Although intact palate was identified in one study as a risk factor for unsuccessful treatment with mandibular distraction osteoegenesis, there has overall been minimal investigation into whether there are differences in outcomes or etiologies between children with RS based on presence of absence of CP (Flores et al, 2015).…”

Section: Introductionmentioning

confidence: 99%

“…Higher prevalence of CP is observed in RS cohorts ascertained without requiring uniform, objective evaluation for airway obstruction such as polysomnography (Daniel et al, 2013; Flores et al, 2015; Holder‐Espinasse et al, 2001; Kam et al, 2015; Runyan et al, 2018; Thouvenin et al, 2013). We speculate that the presence of CP in a micrognathic child represents an obvious reminder to clinicians to consider the possibility of RS.…”

Section: Introductionmentioning

confidence: 99%

Robin sequence without cleft palate: Genetic diagnoses and management implications

Weaver

Sullivan

Balow

et al. 2021

American J of Med Genetics Pt A

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Robin sequence (RS), the triad of micrognathia, glossoptosis, and airway obstruction, is a major cause of respiratory distress and feeding difficulties in neonates. Robin sequence can be associated with other medical or developmental comorbidities in ~50% of cases (“syndromic” RS). As well, RS is variably associated with cleft palate (CP). Previous studies have not investigated differences in clinical characteristics of children with RS based on presence or absence of CP. We retrospectively reviewed 175 children with RS and compared genetic diagnoses, medical and developmental comorbidities, severity of airway obstruction, and feeding outcomes between those with and without CP. Strikingly, 45 of 45 (100%) children with RS without CP were classified as syndromic due to presence of comorbidities unrelated to RS, while 83 of 130 (64%) children with RS with CP were classified as syndromic. Among 128 children with syndromic RS, there were no differences in severity of airway obstruction, surgical intervention rate or type, or feeding outcome at 12 months based on CP status. Our findings support the conclusion that the pathogenesis of RS without CP is distinct from RS with CP and more likely to cause additional medical or developmental problems. Alternatively, children with RS without CP and without additional anomalies present may be under recognized.

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“…Infants treated by conservative technique and with MDO had developed long‐term feeding and airway obstruction results in comparison with the tracheostomy group. 20 …”

Section: Discussionmentioning

confidence: 99%

Effective intubation technique to manage the airway obstruction in A case of Pierre Robin sequence: A case report

Mehrpisheh

Farhadi

2022

Clinical Case Reports

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Management of Airway Obstruction in Infants With Pierre Robin Sequence

Abstract: Supplemental Digital Content is available in the text.

Cited by 29 publications

References 33 publications

Quantification of Mandibular Morphology in Pierre Robin Sequence to Optimize Mandibular Distraction Osteogenesis

Quantification of Mandibular Morphology in Pierre Robin Sequence to Optimize Mandibular Distraction Osteogenesis

Robin sequence without cleft palate: Genetic diagnoses and management implications

Effective intubation technique to manage the airway obstruction in A case of Pierre Robin sequence: A case report

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