Abstract

Abstract Background Regional odontodysplasia (ROD) is a rare localised developmental disorder affecting the dental tissues. It presents with specific clinical, radiographic, and histopathological features. There have been less than 180 reported cases of ROD in the literature. Case Report A 10-year-old boy was seen in the paediatric dental department with a complaint of missing teeth and pain in the left maxilla. Examination revealed retained fragments of primary teeth and a partially erupted upper left first permanent molar (UL6) with an unusual morphology, cavitation, and associated infection. The dental development in the rest of the mouth appeared to be normal. CT scanning of the left maxilla revealed all unerupted permanent teeth were present but had a characteristic "ghost-teeth" appearance suggestive of regional odontodysplasia. The patient underwent surgical exploration of the left maxilla and removal of the UL6. The remaining unerupted teeth in the area showed no signs of infection. Although they have a poor long-term prognosis, they were left in situ in order to preserve alveolar bone whilst the patient is growing. Histological examination showed thin disorganised dental tissues with large pulp chambers, confirming the diagnosis of regional odontodysplasia. Discussion ROD presents management challenges, particularly in children. This case highlights the importance of being aware of the clinical and radiographic features of rare disorders in order to aid diagnosis and treatment planning. This case also demonstrates the importance of targeted intervention and monitoring in order to improve outcomes for placement of dental prostheses in adulthood once growth is complete.

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