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Generalised hypomineralisation of enamel in oculodentodigital dysplasia: comprehensive dental management of a case
  1. Emilija Daina Jensen1,2
  1. 1Discipline of Paediatric Dentistry, The University of Adelaide, Adelaide, South Australia, Australia
  2. 2Department of Paediatric Dentistry, Women's and Children's Hospital Adelaide, North Adelaide, South Australia, Australia
  1. Correspondence to Dr Emilija Daina Jensen; emilija.jensen{at}


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.

  • dentistry and oral medicine
  • genetics
  • mouth

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  • Contributors EDJ obtained consent, performed the literature review and wrote the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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