scholarly journals Prenatal Identification of Pierre Robin Sequence: A Review of the Literature and Look towards the Future

2015 ◽  
Vol 39 (2) ◽  
pp. 81-89 ◽  
Author(s):  
Matthew G. Kaufman ◽  
Christopher I. Cassady ◽  
Charles H. Hyman ◽  
Wesley Lee ◽  
Mehernoor F. Watcha ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
Medical Literature ◽  
Medical Team ◽  
Pierre Robin Sequence ◽  
Review Of The Literature ◽  
Respiratory Compromise ◽  
Fetal Ultrasonography ◽  
Diagnostic Pathway ◽  
Robin Sequence ◽  
Pierre Robin

Fetal ultrasonography is an important tool used to prenatally diagnose many craniofacial conditions. Pierre Robin sequence (PRS) is a rare congenital deformation characterized by micrognathia, glossoptosis, and airway obstruction. PRS can present as a perinatal emergency when the retropositioned tongue obstructs the airway leading to respiratory compromise. More predictable and reliable diagnostic studies could help the treating medical team as well as families prepare for these early airway emergencies. The medical literature was reviewed for different techniques used to prenatally diagnose PRS radiologically. We have reviewed these techniques and suggested a possible diagnostic pathway to consistently identify patients with PRS prenatally.

Cleft Palate, Cleft Lip, and Pierre Robin Sequence

2018 ◽  
Author(s):  
Michelle Keese Harvey ◽  
Ihab Ayad
Keyword(s):  
Cleft Palate ◽  
Airway Obstruction ◽  
Cleft Lip ◽  
Anesthetic Management ◽  
Treatment Options ◽  
Pierre Robin Sequence ◽  
Respiratory Compromise ◽  
Feeding Difficulties ◽  
Robin Sequence ◽  
Pierre Robin

Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and airway obstruction. Often associated with cleft palate, PRS is usually an isolated finding but is associated with a syndrome one-third of the time. The micrognathia and glossoptosis lead to airway obstruction, respiratory compromise, and feeding difficulties. Severe cases and prolonged and repeated airway obstruction and respiratory distress can lead to failure to thrive, hypoxemia, pulmonary hypertension, cardiac arrest, and death. Treatment options for the management of airway obstruction in PRS depends on the degree of obstruction and any associated comorbidities and range from noninvasive respiratory support to surgical correction of the physical defect. Patients with PRS should be considered as challenging to ventilate and intubate, and the practitioner should be well prepared for the possibility of difficulty with airway management. Anesthetic management is tailored to minimize postoperative upper airway obstruction and avoid disruption of the integrity of the surgical repair.

A Systematic Review of Mandibular Distraction Osteogenesis Versus Orthodontic Airway Plate for Airway Obstruction Treatment in Pierre Robin Sequence

2021 ◽  
pp. 105566562110118
Author(s):  
Darren B. Abbas ◽  
Christopher Lavin ◽  
Evan J. Fahy ◽  
HyeRan Choo ◽  
Mai Thy Truong ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
Distraction Osteogenesis ◽  
Literature Search ◽  
Oral Feeding ◽  
Treatment Modalities ◽  
Pierre Robin Sequence ◽  
Mandibular Distraction ◽  
Robin Sequence ◽  
Mandibular Distraction Osteogenesis ◽  
Pierre Robin

Objective: Mandibular distraction osteogenesis (MDO) is frequently performed to address airway obstruction in patients with Pierre Robin sequence (PRS), though more recently the technique of orthodontic airway plating (OAP) has gained traction. We aimed to evaluate OAP compared to MDO for airway obstruction in PRS. Design: A systematic literature search across PubMed, Embase, and Google Scholar identified all studies published in English, which involved MDO or any form of OAP as treatments for PRS. All relevant articles were reviewed in detail and reported on, adhering to PRISMA guidelines. Main Outcome Measures: Airway (tracheostomy avoidance, decannulation rate), feeding (full oral feeding tolerance). Results: Literature search identified 970 articles, of which 42 MDO studies and 9 OAP studies met criteria for review. A total of 1159 individuals were treated with MDO, and 322 individuals were treated with OAP. Primary outcomes appear similar for MDO and OAP at face value; however, this must be interpreted with different pretreatment contexts in mind. Conclusions: Orthodontic airway plating may be considered for airway obstruction in PRS, as some airway-related and feeding-related outcomes appear similar with MDO, per existing evidence in the literature. However, since PRS severity differed between studies, OAP cannot be uniformly considered a replacement for MDO. Further research is required to more comprehensively assess these treatment modalities inclusive of metrics that allow for direct comparison.

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

2020 ◽  
Vol 57 (8) ◽  
pp. 1032-1040 ◽  
Author(s):  
Angela S. Volk ◽  
Matthew J. Davis ◽  
Amit M. Narawane ◽  
Amjed Abu-Ghname ◽  
Robert F. Dempsey ◽  
...  
Keyword(s):  
Airway Management ◽  
Airway Obstruction ◽  
Distraction Osteogenesis ◽  
Pierre Robin Sequence ◽  
Management Decisions ◽  
Mandibular Distraction ◽  
Robin Sequence ◽  
Mandibular Distraction Osteogenesis ◽  
Mandibular Body ◽  
Pierre Robin

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.

scholarly journals Management of Airway Obstruction in Infants With Pierre Robin Sequence

2018 ◽  
Vol 6 (5) ◽  
pp. e1688 ◽  
Author(s):  
Christopher M. Runyan ◽  
Armando Uribe-Rivera ◽  
Shahryar Tork ◽  
Tasneem A. Shikary ◽  
Zarmina Ehsan ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Pierre Robin

Pediatric Feeding in Infants With Pierre Robin Sequence Following Mandibular Distraction Osteogenesis

2016 ◽  
Vol 1 (13) ◽  
pp. 5-9
Author(s):  
Kelly Mabry
Keyword(s):  
Cleft Palate ◽  
Airway Obstruction ◽  
Distraction Osteogenesis ◽  
Pierre Robin Sequence ◽  
Mandibular Distraction ◽  
Post Surgery ◽  
Feeding Experience ◽  
Robin Sequence ◽  
Mandibular Distraction Osteogenesis ◽  
Pierre Robin

Pierre Robin sequence (PRS) causes anomalies that can include micrognathia, cleft palate, and glossoptosis. At birth, infants typically present with a small mandible (micrognathia) which displaces the tongue posteriorly and causes breathing difficulties due to airway obstruction. A large, U-shaped cleft palate is often associated with this condition and the combined phenotype can be seen in isolation or in conjunction with a syndrome. In the neonatal period, management of PRS focuses on airway obstruction and feeding through conservative positioning techniques that are often successful. However, infants that do not respond to conservative measures require surgical intervention such as tongue-lip adhesion, tracheostomy, or mandibular distraction osteogenesis (MDO) to manage their airway. While each of these surgical procedures have been used effectively, the process of MDO has become the definitive technique to surgically correct the airway obstruction in PRS by lengthening the mandible, which also brings the tongue forward and out of the airway. Feeding intervention is directly related to the resolution of micrognathia which in turn advances the tongue base anteriorly, resolving the glossoptosis and airway obstruction. The infant's feeding experience, including pre-surgery and post-surgery considerations, are discussed.

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