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Cleidocranial dysplasia
Aetiology
• It appears as a true dominant Mendelian
characteristic
• incomplete penetrance of genetic trait
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
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
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
Diagnosis
• Meeting shoulder in the midline is typical
feature of cleidocranial dysplasia
• supernumerary teeth, open sutures and
absence of clavicle
Management
• Management of skull and clavicle anomalies –
there is no treatment is required for this
anomaly
• dental care – supernumerary teeth, they
should be extracted

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Cleidocranial Dysplasia: Rare Genetic Disorder Affecting Skull, Clavicle, Teeth

  • 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