Crouzon syndrome

Overview

Board takeaway: Assess obstruction and sleep apnea before sedation, protect exposed eyes, and coordinate orthodontics with midface surgery.

Craniosynostosis and maxillary hypoplasia with proptosis; airway and skeletal occlusion need coordinated care.

How does Crouzon syndrome affect dental management?

• Airway: Assess airway obstruction and sleep history before sedation; coordinate with anesthesia
• Craniofacial coordination: midface advancement may occur in childhood for airway or eye protection; definitive orthognathic surgery follows growth, with orthodontics before and after.
• Prevention and eye protection: Tailor prevention to crowding and hygiene access; protect exposed corneas from drying and accidental contact with instruments

Exam pearl

What is Crouzon syndrome?

A genetic craniosynostosis syndrome causing abnormal skull growth, shallow eye sockets, and underdevelopment of the midface.

What are the main systemic features of Crouzon syndrome?

• Genetics: Craniofacial dysostosis; FGFR2 variant, autosomal dominant with variable expression (about half inherited)
• Craniofacial features: Coronal craniosynostosis, shallow orbits with proptosis, hypertelorism, beaked nose, midface hypoplasia
• Hands, feet and development: Hands and feet are normal; intelligence is usually typical
• Medical risks: Conductive hearing loss, raised intracranial pressure, upper-airway obstruction and OSA

What oral findings occur in Crouzon syndrome?

• Occlusion: Maxillary hypoplasia with relative mandibular prognathism: Class III, anterior open bite, posterior crossbite
• Arch and eruption: V-shaped maxillary arch, narrow high palate, crowding and ectopic eruption
• Clefting: Cleft lip or palate is uncommon

For Crouzon syndrome, what do you check before sedation?

• Upper-airway obstruction/OSA from midface hypoplasia
• Raised intracranial pressure history and prior craniofacial surgery
• Anesthesia coordination: match airway findings, procedure length, and recovery needs to the team and setting; the syndrome name alone does not select the anesthetic technique

For Crouzon syndrome, how do you distinguish Crouzon from Apert syndrome?

• Shared findings: Both are FGFR2 craniosynostoses with midface hypoplasia, shallow orbits and proptosis.
• Distinction: Crouzon has normal hands and feet and usually typical intelligence; Apert has symmetric syndactyly.

Sources

• Supplemental study guide: Pediatric Dental Study Guide: Crouzon syndrome
• Policy on the Management of Patients with Cleft Lip/Palate and Other Craniofacial Anomalies
• Management of Dental Patients with Special Health Care Needs
• Management of the Developing Dentition and Occlusion in Pediatric Dentistry
• Pediatric Airway Assessment
• Supplemental genetics: MedlinePlus Genetics: crouzon syndrome