Related Experiment Video

Updated: Sep 20, 2025

Minimally Invasive Murine Laryngoscopy for Close-Up Imaging of Laryngeal Motion During Breathing and Swallowing
07:22

Minimally Invasive Murine Laryngoscopy for Close-Up Imaging of Laryngeal Motion During Breathing and Swallowing

Published on: December 1, 2023

674

Prevalence and Management of Laryngomalacia in Patients With Pierre Robin Sequence

Anna E Bakeman1,2, Amber D Shaffer1, Allison B J Tobey1,2

  • 1UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

The Cleft Palate-Craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association
|June 7, 2022
PubMed

Insights

Laryngomalacia affects over half of Pierre Robin Sequence patients and is linked to aspiration. Supraglottoplasty did not significantly improve airway obstruction, but mandibular distraction did.

Area of Science:

  • Pediatric Otolaryngology
  • Craniofacial Anomalies
  • Airway Management

Background:

  • Pierre Robin Sequence (PRS) is a complex congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
  • Laryngomalacia is a common cause of stridor in infants, and its prevalence and impact in PRS patients require further characterization.
  • Understanding the interplay between PRS, laryngomalacia, and treatment efficacy is crucial for optimizing patient outcomes.

Purpose of the Study:

  • To determine the prevalence and clinical presentation of laryngomalacia in a cohort of patients with Pierre Robin Sequence.
  • To evaluate the efficacy of supraglottoplasty (SGP) and mandibular distraction osteogenesis (MDO) in managing airway obstruction in PRS patients with laryngomalacia.

Main Methods:

  • A retrospective cohort study was conducted at a tertiary-care children's hospital.
  • Data from 126 consecutive PRS patients (born 2010-2018) were reviewed, including demographics, comorbidities, and surgical interventions.
  • Airway obstruction was assessed using sleep study data (apnea-hypopnea index [AHI] and obstructive AHI [OAHI]) and modified barium swallow studies.

Main Results:

  • Laryngomalacia was present in 51% of the PRS cohort.
  • Patients with concurrent PRS and laryngomalacia were more likely to exhibit submucous cleft palate and aspiration with cough.
  • Mandibular distraction osteogenesis significantly reduced AHI and OAHI (median improvement of 22.3 and 19.8, respectively; P<.002).
  • Supraglottoplasty alone did not yield significant improvements in AHI or OAHI (P>.06).

Conclusions:

  • Laryngomalacia is highly prevalent in Pierre Robin Sequence patients and associated with increased aspiration risk.
  • Mandibular distraction osteogenesis is an effective treatment for improving airway obstruction in PRS patients with laryngomalacia.
  • Laryngomalacia should not be considered a contraindication for pursuing mandibular distraction osteogenesis in this population.

Keywords:
Pierre Robin Sequenceairwaylaryngomalaciasupraglottoplasty

More Related Videos

Laryngeal Mask Airway LMA Placement in a Neonatal Patient Simulator Using a Non-Inflatable Supraglottic Airway SGA04:56

Laryngeal Mask Airway LMA Placement in a Neonatal Patient Simulator Using a Non-Inflatable Supraglottic Airway SGA

Published on: July 14, 2023

3.2K
Learning Modern Laryngeal Surgery in a Dissection Laboratory07:30

Learning Modern Laryngeal Surgery in a Dissection Laboratory

Published on: March 18, 2020

8.2K

Related Concept Videos

Cardiopulmonary Resuscitation II: ACLS Airway Management01:22

Cardiopulmonary Resuscitation II: ACLS Airway Management

Airway management is a key skill in emergency and critical care settings, as maintaining a clear airway is essential for adequate oxygenation and ventilation.Head Tilt-Chin Lift TechniqueThe head tilt-chin lift maneuver is an essential technique primarily used in patients without suspected cervical spine injuries. To perform this maneuver, one hand is placed on the patient’s forehead, and gentle pressure is applied backward to tilt the head. The fingertips of the other hand are positioned...
123
Cardiopulmonary Resuscitation V: Advanced Airway Management Techniques01:30

Cardiopulmonary Resuscitation V: Advanced Airway Management Techniques

Airway management is essential in emergency and surgical medicine, ensuring ventilation and oxygenation in patients who cannot maintain their own airway. Clinicians use a range of techniques and devices to secure the airway, depending on the patient’s condition and the clinical context. Key methods include endotracheal intubation, rapid sequence intubation (RSI), supraglottic airway devices, and advanced visualization aids. In cases where these approaches fail, surgical airway...
108
Esophageal Strictures-II: Clinical Features and Management01:26

Esophageal Strictures-II: Clinical Features and Management

Patients with esophageal strictures often experience a range of symptoms. Initially, they may have difficulty swallowing solid foods, which can progress to include liquids. Additional symptoms may involve chest pain or discomfort, regurgitating food and fluids, heartburn, unintentional weight loss, coughing or choking during meals, and hoarseness.
Healthcare providers should gather a comprehensive medical history and conduct a physical examination for diagnosis. If esophageal stricture is...
170
Mitral Valve Prolapse III: Nursing Management01:19

Mitral Valve Prolapse III: Nursing Management

The nursing management of Mitral Valve Prolapse, or MVP, centers around patient education, symptom monitoring, and lifestyle modifications.Patient Education on MVP Diagnosis and Heredity: Nurses should provide comprehensive education about MVP, a condition where the mitral valve does not close appropriately during heartbeats. This education often includes the condition's pathophysiology, symptoms, and potential complications, like arrhythmias or mitral regurgitation. Though not fully...
42
Mitral Stenosis III: Medical Management01:26

Mitral Stenosis III: Medical Management

Mitral stenosis, a condition marked by the narrowing of the mitral valve, necessitates an integrated approach for effective management. This approach includes preventative measures, medical therapy, and surgical interventions to reduce symptoms and prevent complications.PreventionPrevention of mitral stenosis primarily focuses on reducing the incidence of bacterial infections, particularly streptococcal infections, which can lead to rheumatic fever and subsequent valvular damage. Timely...
31
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
229