Pathology · patient case question

What is the diagnosis?

Patient case: Faint, poorly formed teeth in one quadrant on a panoramic film

Patient: Male, 18 years old

Chief Complaint: “Two of my top teeth on the right never came in properly and they crumble.”

Background: No systemic disease, no medications, no family history of dental anomaly. No history of trauma or infection in childhood.

Vital Signs: BP 118/72 mmHg · HR 70 bpm

Findings: Panoramic radiograph shows two adjacent maxillary right teeth with markedly THIN enamel and dentin, very wide pulp chambers, short roots and open apices, giving a faint, wispy outline that barely contrasts with the surrounding bone. The adjacent and contralateral teeth are entirely normal. Clinically the affected teeth are small, yellow-brown, rough and partially unerupted, with soft-tissue enlargement over the ridge.

  1. Dentinogenesis imperfecta
  2. Dentin dysplasia type I
  3. Regional odontodysplasia
  4. Amelogenesis imperfecta
Show answer and explanation

Correct answer: C. Regional odontodysplasia

  • A. Dentinogenesis imperfecta is hereditary and generalised, giving bulbous crowns with cervical constriction and pulp chambers that OBLITERATE rather than enlarge. The opposite radiographic picture to this one.
  • B. Dentin dysplasia type I, the 'rootless teeth', gives normal-looking crowns with extremely short or absent roots and crescent-shaped pulpal remnants, generalised across the dentition rather than confined to a quadrant.
  • C. CORRECT. REGIONAL ODONTODYSPLASIA — the 'GHOST TEETH' of the older literature — is defined by its LOCALISED, segmental distribution. Both enamel and dentin are hypoplastic and hypocalcified in a group of ADJACENT teeth, usually in one quadrant, with a sharp cut-off at the midline. The radiographic hallmark is exactly what is described: a faint, wispy outline with almost no contrast against bone, thin hard tissue shells, enormous pulp chambers and short open-apex roots. It is non-hereditary and sporadic, favours the MAXILLA and the anterior segment, affects both dentitions, and the teeth frequently fail to erupt or become infected.
  • D. Amelogenesis imperfecta is a hereditary defect of ENAMEL ONLY, and it is GENERALISED — every tooth in both dentitions. The dentin, pulp chambers and root form are normal, and there is no segmental distribution.

Note: Distribution is the whole diagnosis. GENERALISED and hereditary points to amelogenesis or dentinogenesis imperfecta; a LOCALISED segment with both enamel and dentin affected and a ghostly radiographic appearance is regional odontodysplasia. Management is usually extraction of the affected teeth with prosthetic replacement, since they are unrestorable and prone to abscess; some clinicians retain them for space maintenance where they are asymptomatic.

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