Cleidocranial dysplasia

Overview

Board takeaway: Plan staged care for failed eruption and multiple supernumerary teeth. An autosomal dominant RUNX2 defect also causes absent or hypoplastic clavicles and open fontanelles.

How does Cleidocranial dysplasia affect dental management?

• Diagnosis: Early diagnosis with panoramic imaging
• Staged multidisciplinary care (Toronto-Melbourne or Belfast-Hamburg approaches): remove primary and supernumerary teeth, surgically expose and bond permanent teeth, then use orthodontic traction.
• Later care: Orthognathic surgery after growth; prosthetics where teeth fail to erupt

Exam pearl

What is Cleidocranial dysplasia?

A genetic skeletal disorder, usually autosomal dominant and associated with RUNX2 mutations, characterized by underdeveloped or absent clavicles, delayed skull-suture closure, and abnormalities of tooth number and eruption.

What are the main systemic features of Cleidocranial dysplasia?

• Genetics: RUNX2 mutation; autosomal dominant with variable expression
• Skeleton: Hypoplastic or absent clavicles; delayed fontanelle closure; Wormian bones; frontal bossing; short stature
• Face: Hypertelorism, broad nasal bridge, brachycephaly

What oral findings occur in Cleidocranial dysplasia?

• Prolonged retention of primary teeth; delayed or failed eruption of permanent teeth
• Multiple supernumerary teeth, especially in the premolar and incisor regions; dentigerous cysts
• Maxillary hypoplasia with relative mandibular prognathism; high-arched or cleft palate

For Cleidocranial dysplasia, why do permanent teeth fail to erupt?

• Abnormal bone and tooth development related to RUNX2, together with retained primary teeth and supernumerary obstruction, can delay eruption.
• Plan from the individual anatomy: image the permanent and supernumerary teeth, assess root development and available space, and coordinate staged surgery and orthodontics. A single presumed cementum defect does not explain or determine every treatment plan.

A 10-year-old has many retained primary teeth and no permanent teeth erupted. The panoramic radiograph shows many unerupted and supernumerary teeth. What else do you examine?

Examine the shoulders and skull:

• Hypoplastic or absent clavicles; the child can bring the shoulders together
• Open fontanelles and sutures, frontal bossing, and Wormian bones

These findings suggest cleidocranial dysplasia (RUNX2, autosomal dominant).

Sources

• Supplemental study guide: Pediatric Dental Study Guide: Cleidocranial dysostosis
• Management of the Developing Dentition and Occlusion in Pediatric Dentistry
• Management Considerations for Pediatric Oral Surgery
• Supplemental genetics: MedlinePlus Genetics: cleidocranial dysplasia
• AAPD best practice: AAPD: Management of Dental Patients with Special Health Care Needs (2026)