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A UK-based consensus on clinical decision flowcharts for managing childhood amelogenesis imperfecta in the permanent
S Lakhani1,2, J Monteiro3,4, M Agel3
1Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom. shrita.lakhani@nhs.net.
Purpose:
Amelogenesis imperfecta (AI) is a rare genetic condition characterised by defective enamel formation, with variable presentations. Diagnosis traditionally involves family history, clinical presentation, with genetic testing being increasingly integrated into clinical practice. Children and young people (CYP) with AI face clinical and psychosocial challenges over extended periods. Given the absence of specific guidelines for AI management in CYP, this project aimed to develop flowcharts to guide dental practitioners in managing the three primary AI phenotypes: hypoplastic, hypocalcified, and hypomaturation. The flowcharts focus on the permanent dentition and provide a structured, yet flexible treatment approach, addressing key clinical issues such as hypersensitivity management, and aesthetic restoration.
Methods:
The flowcharts were developed collaboratively by the UK Amelogenesis Imperfecta/Dentinogenesis Imperfecta National Clinical Excellence Network (AI/DI CEN). Expert opinion was sought over a series of meetings with specialists who treat children with amelogenesis imperfecta. Initial drafts of the flowcharts were discussed in two discussion forums until consensus was achieved.
Results:
The flowcharts prioritise prevention, minimally invasive treatment, and shared decision-making, adapting interventions to phenotype type and severity as well as individual patient needs.
Conclusion:
This guidance is presented to assist dental professionals in delivering comprehensive, empathetic, and effective care for CYP with AI.
