Cleidocranial dysplasia is a genetic condition characterized by incomplete penetrance that primarily affects the skull, clavicles, and dentition. Key features include a brachycephalic skull with sunken sutures and depressed nasal bridge, absence or malformation of clavicles causing shoulders to meet in the midline, retained primary teeth and delayed permanent tooth eruption, and supernumerary teeth. Radiographs show open skull sutures, absence or malformation of clavicles, and dental anomalies. Diagnosis is based on the shoulder meeting in the midline and dental and skeletal features. Management focuses on dental care including extraction of supernumerary teeth while skull and clavicle anomalies require no

1. Cleidocranial dysplasia

2. Aetiology
• It appears as a true dominant Mendelian
characteristic
• incomplete penetrance of genetic trait

3. Clinical features
• Primarily affects skull, clavicle and dentition
• shoulder meet in midline
• head is brachycephalic or wide and short
• lacrimal and zygomatic bone are also
underdeveloped
• sagittal suture is characteristically sunken,
giving skull flat appearance
• nasal bridge is depressed with a broad base

4. Oral manifestation
• Micrognathia
• maxilla is underdeveloped and is smaller than
normal, in relation to mandible
• prognathism
• prolonged retention of primary dentition and
delayed eruption of permanent dentition
• high narrow arched palate and cleft palate may
be common
• Crown may be pitted because of enamel
hypoplasia

5. Radiographic features
• Skull film reveals open sutures
• malformation and absence of clavicles
• prolonged retention of primary teeth
• presence of supernumerary teeth usually in
anterior region
• small underdeveloped jaw
• roots of teeth are short and thinner than
normal

6. Diagnosis
• Meeting shoulder in the midline is typical
feature of cleidocranial dysplasia
• supernumerary teeth, open sutures and
absence of clavicle

7. Management
• Management of skull and clavicle anomalies –
there is no treatment is required for this
anomaly
• dental care – supernumerary teeth, they
should be extracted