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Cleidocranial Dysplasia — MFDS Part 1 MCQ

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ModerateHuman DiseaseCleidocranial DysplasiaMFDS Part 1

Which dental pattern is most characteristic of cleidocranial dysplasia?

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Reveal the answer and explanation

Correct answer: DMultiple supernumerary teeth with delayed or arrested eruption of permanent teeth

Explanation lettering: E = shown as A · D = shown as B · A = shown as D · B = shown as E

A is correct. Cleidocranial dysplasia is a RUNX2-related skeletal dysplasia whose characteristic dental phenotype includes multiple supernumerary permanent teeth, retained primary teeth, and delayed or arrested eruption of the permanent dentition. Option A combines the two most discriminating dental findings. Generalised hypodontia in B is the opposite numerical abnormality. Generalised enamel hypoplasia in C suggests a primary enamel-development disorder rather than cleidocranial dysplasia. Macrodontia and delayed primary-tooth eruption in D are not characteristic; primary-tooth formation and eruption are generally normal. Generalised primary-tooth ankylosis and premature permanent-tooth loss in E are also not defining features.

Reference: Kreiborg S, Jensen BL. Tooth formation and eruption—lessons learnt from cleidocranial dysplasia. European Journal of Oral Sciences. 2018;126(Suppl 1):72-80. https://pubmed.ncbi.nlm.nih.gov/30178560/