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Cleidocranial Dysplasia — NDEB AFK MCQ

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ModerateOral PathologyCleidocranial DysplasiaNDEB AFK

Which inherited condition is characterised by supernumerary teeth, delayed exfoliation of primary teeth, absent or hypoplastic clavicles, and patent fontanelles?

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Correct answer: E — Cleidocranial dysplasia

Cleidocranial dysplasia (CCD) is an autosomal dominant skeletal disorder caused by mutations in the RUNX2 gene (a transcription factor essential for osteoblast differentiation). Key features include hypoplastic or absent clavicles (patients can approximate shoulders anteriorly), multiple supernumerary teeth, delayed eruption and exfoliation, open fontanelles and sutures, short stature, and brachycephalic skull. Dental management involves serial extractions and prosthetic rehabilitation.

Reference: Neville – Oral and Maxillofacial Pathology, 4th ed., 2016