European Archives of Paediatric Dentistry

, Volume 12, Issue 4, pp 219–223 | Cite as

Case report: Unusual dental morphology in a child with ankyioblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome

  • K. FitzGeraldEmail author
  • S. A. Lynch
  • E. McKiernan


BACKGROUND; Anomalies of dental anatomy are common in the ectodermal dysplasia syndromes. These anomalies, when found in combination with dental caries, can pose a restorative challenge for the paediatric dentist. Modification of traditional techniques and approaches may help the practitioner provide a successful treatment outcome. CASE REPORT: A 3 years and 11 months old girl with a diagnosis of ankyioblepharon-ectodermal dysplasia-cleft lip/palate (AEC) syndrome was referred for treatment to a specialist paediatric dental service. Her abnormal dental anatomy, hypodontia and dental caries formed a triad of challenges for the team. Under general anaesthesia, her dentition was restored using a combination of restorative approaches and techniques, including the placement of both composite resin and preformed metal crown restorations. FOLLOW-UP: At 18-month follow-up, the family had successfully implemented good home care and dietary practices, and the local dental service had instituted a preventive programe consisting of regular examination, advice and fluoride varnish placement. The restorations remained intact and no further caries was detected. At 24-month follow-up, the first permanent molars were partially erupted, and displayed unusually deep fissures. There was also a degree of ectopic eruption of the first permanent molars, and possibly of one of the maxilary permanent incisors. CONCLUSION: Dental care for children with AEC syndrome is optimised by early intervention, good home care and regular professional review. Dental care providers should be aware of the possibility of complex dental anatomy, and bear this in mind should it become necessary to formulate a restorative treatment plan.


Ectodermal dysplasia dental care for children anodontia dental caries dental caries/therapy 


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Copyright information

© European Archives of Paediatric Dentistry 2011

Authors and Affiliations

  1. 1.Department of Paediatric DentistryOur Lady’s Children’s HospitalCrumlin, Dublin 12Ireland
  2. 2.National Centre for Medical GeneticsOur Lady’s Children’s HospitalCrumlin, Dublin 12Ireland
  3. 3.National Maxillofacial UnitSt. James’s HospitalDublin 8Ireland

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