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Dentinogenesis Imperfecta — NDEB AFK MCQ

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ModerateOral PathologyDentinogenesis ImperfectaNDEB AFK

Which hereditary condition affects both the primary and permanent dentitions with characteristically opalescent teeth, obliterated pulp chambers, and bulbous crowns?

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Correct answer: A — Dentinogenesis imperfecta (Type II, or shields Type II)

Dentinogenesis imperfecta (DI) Type II (hereditary opalescent dentin, Shields Type II) is an autosomal dominant disorder caused by DSPP gene mutations. It affects both primary and permanent teeth with blue-grey to amber-brown opalescent discolouration, short bulbous crowns, constricted cervical areas, obliterated pulp chambers (radiographically), and short roots. The enamel chips easily due to the defective DEJ. DI Type I is associated with osteogenesis imperfecta. DI Type II is the isolated dental form.

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