Related Experiment Video

Updated: Sep 16, 2025

Drug-Induced Sleep Endoscopy DISE with Target Controlled Infusion TCI and Bispectral Analysis in Obstructive Sleep Apnea
07:54

Drug-Induced Sleep Endoscopy DISE with Target Controlled Infusion TCI and Bispectral Analysis in Obstructive Sleep Apnea

Published on: December 6, 2016

20.0K

Differential impact of Crouzon and Apert syndromes on upper airways morphology: implications for Obstructive Sleep

Floriane Remy1, Maxime Taverne2, Roman Hossein Khonsari3

  • 1Univ. Bordeaux, CNRS, Ministère de la Culture, PACEA, UMR 5199, 33615, Pessac, France.

Journal of Cranio-Maxillo-Facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery
|July 9, 2025
PubMed

Insights

Children with syndromic craniosynostoses have distinct airway differences, with Crouzon syndrome affecting the oropharynx and Apert syndrome altering the nasopharynx. These findings aid in personalized respiratory diagnoses and treatments.

Area of Science:

  • Craniofacial biology and pediatric respiratory medicine.

Background:

  • Children with syndromic craniosynostoses have a high risk of obstructive sleep apnoea (OSA).
  • The specific mechanisms linking craniofacial abnormalities to OSA in these conditions are not fully understood.

Purpose of the Study:

  • To investigate how Crouzon and Apert syndromes impact upper airway morphology in children.
  • To examine the relationship between airway shape, craniofacial structures, and obstructive sleep apnoea (OSA).
  • To analyze the covariation between upper airway and craniofacial regions.

Main Methods:

  • Geometric morphometric analysis of 3D upper airway models from 37 patients with syndromic craniosynostoses and 53 controls (0-49 months).
  • Analysis of 52 landmarks on 3D airway models and apnoea-hypopnoea index (AHI) data.
  • Controlling for age, mouth openness, and craniocervical posture in analyses.

Main Results:

  • Age, mouth openness, and posture significantly influenced airway shape, necessitating standardized imaging.
  • Crouzon syndrome was associated with oropharynx changes, while Apert syndrome affected the nasopharynx.
  • No direct correlation was found between airway shape and OSA severity; upper airway growth and craniofacial covariation were not impacted by the syndromes.

Conclusions:

  • Syndrome-specific differences in airway morphology exist, linked to FGFR mutations affecting bone and soft tissues.
  • Findings support tailored respiratory diagnoses and personalized treatment strategies for children with syndromic craniosynostoses.

Keywords:
FGFRNasal airwayObstructive respiratory disordersPharynxSyndromic craniosynostosis

More Related Videos

Author Spotlight: Traditional Chinese Medicine for Sleep Disorders in Acute COPD — A Safe, Cost-Effective Approach04:53

Author Spotlight: Traditional Chinese Medicine for Sleep Disorders in Acute COPD — A Safe, Cost-Effective Approach

Published on: October 18, 2024

913
Author Spotlight: Utilizing Traditional Chinese Acupuncture of the Ear to Improve Sleep Disorders05:34

Author Spotlight: Utilizing Traditional Chinese Acupuncture of the Ear to Improve Sleep Disorders

Published on: August 18, 2023

2.5K

Related Concept Videos

Sleep Apnea01:21

Sleep Apnea

Sleep apnea is a condition where breathing stops intermittently during sleep, often leading to significant health issues. Each episode can last from 10 to 20 seconds or more and is frequently accompanied by a brief arousal from sleep. This disturbance, largely unnoticed by the individual, can lead to severe daytime fatigue. Commonly, individuals seek help after being informed by their partners about loud snoring and noticeable breathing pauses during sleep.
The condition is more prevalent among...
225
Chronic Obstructive Pulmonary Disease-II: Pathophysiology01:20

Chronic Obstructive Pulmonary Disease-II: Pathophysiology

Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
3.1K
Other Pulmonary Disorders01:17

Other Pulmonary Disorders

Respiratory disorders encompass a range of conditions with varying levels of severity. Asthma, marked by chronic airway inflammation and hypersensitivity, is one such condition. It can lead to airway obstruction due to factors like bronchial spasms, mucosal edema, increased mucus secretion, or epithelial damage. Asthma triggers are diverse, ranging from allergens to emotional upset, and treatment focuses on both immediate relief through bronchodilators and long-term inflammation suppression.
913
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
369
COPD: Pathogenesis and Clinical Features01:20

COPD: Pathogenesis and Clinical Features

Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
533
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies01:27

Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies

Assessing and diagnosing Chronic Obstructive Pulmonary Disease (COPD) involves a detailed approach that includes a comprehensive review of medical history, physical examination, and a variety of diagnostic tests. This thorough evaluation is essential to ensure an accurate diagnosis and guide effective management strategies.
Medical History
2.6K