Cleidocranial Dysostosis

Cleidocranial Dysostosis

Question. Write short note on cleidocranial dysostosis.
Answer. It is also called cleidocranial dysplasia or Marie and

Sainton disease.

Cleidocranial Dysostosis Etiology

• Hereditary: True dominant Mendelian characteristic.
• Genetic: There is incomplete penetration of the genetic trait. Defect in CBFA I gene chromosome.

Cleidocranial dysostosis

Cleidocranial Dysostosis Clinical Features 

• The disease affects men and women with equal frequency.
• It primarily affects skull, clavicle and dentition.
• Head is brachycephalic or wide or short.
• Nasal bridge is depressed with broad base.
• In skull, fontanelles remain open or at least exhibit delayed closing.
• Lacrimal and zygotic bone remains underdeveloped.
• Sagittal suture is sunken and gives skull the flat appearance.
• Nasal bridge is depressed with broad base.

Cleidocranial Dysostosis: Symptoms, Causes, and Diagnosis

Marie and Sainton disease

Cleidocranial Dysostosis Oral Manifestations 

• Micrognathia is present.
• Maxilla is underdeveloped and smaller than normal in relation.
• Prolonged retention of primary dentition and delayed eruption of permanent dentition.
• There is complete absence of cementum.
• Presence of supernumerary teeth in anterior region.
• Roots of teeth are often short and thinner than normal.
• There is hypoplasia of enamel.
• High arch palate and cleft palate can be present.

Cleidocranial Dysostosis Radiographic Features 

• Skull film reveals the presence of open sutures, wormian bones, a wide cranium, and delayed ossification of fontanelles.
• Evaluation of the chest radiograph reveals the absence of clavicles.
• Jaws are flattened, and there is overgrowth of the cranial base anteroposteriorly.
• Jaw examination reveals prolonged retention of primary dentition, multiple superanumerary teeth, and a small, underdeveloped jaw.
• Roots of the teeth are short and thin.

Cleidocranial Dysostosis: Clinical Features and Pathophysiology

Inherited skeletal dysplasias

Cleidocranial Dysostosis Management 

• Not specific.
• The patient consists of supernumerary teeth, which can be extracted, and prosthetic replacement of teeth can be done.
• An unerupted tooth can be extracted orthodontically.