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Generalised hypomineralisation of enamel in oculodentodigital dysplasia: comprehensive dental management of a case.

ABSTRACT: Oculodentodigital dysplasia (ODDD) is a rare congenital disorder characterised by developmental abnormalities of the eye, dentition and digits of the hands and feet, with neurological symptoms reported in 30% of individuals. Dental anomalies associated with ODDD include enamel hypoplasia and subsequent caries, microdontia, missing teeth, amelogenesis imperfecta, pulp stones and delayed tooth development. Here, we describe the comprehensive dental management of a 3-year-old girl who presented with rapid deterioration of the primary dentition due to generalised enamel hypomineralisation. Conservative, comprehensive restorative management was performed under general anaesthesia. Within 6 months, further breakdown of the remaining unrestored enamel was noted. This case documents the challenges of conservative management in dental anomalies that are not well documented due to the extreme rarity of the disorder.

SUBMITTER: Jensen ED

PROVIDER: S-EPMC7802714 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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Generalised hypomineralisation of enamel in oculodentodigital dysplasia: comprehensive dental management of a case.

Jensen Emilija Daina ED

BMJ case reports 20210111 1

Oculodentodigital dysplasia (ODDD) is a rare congenital disorder characterised by developmental abnormalities of the eye, dentition and digits of the hands and feet, with neurological symptoms reported in 30% of individuals. Dental anomalies associated with ODDD include enamel hypoplasia and subsequent caries, microdontia, missing teeth, amelogenesis imperfecta, pulp stones and delayed tooth development. Here, we describe the comprehensive dental management of a 3-year-old girl who presented wit ...[more]

PMID: 33431460

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