Background: Developmental defects of the enamel [DDE] are a widespread problem among children with cleft lip/palate.
Objective: Our aim was to conduct a systematic literature review regarding the prevalence of DDE in children with cleft lip/
palate.
Materials and Methods: A systematic literature search was conducted of PubMed for the years between 1961 and 2018. The key search terms were ‘children with cleft lip palate’ and ‘developmental defect of enamel’, or ‘enamel defect’. Two trained reviewers conducted a risk of bias assessment using a nine-item checklist for prevalence studies.
Results: From the 7 selected full articles, the analysis of pooled prevalence of DDE in primary teeth was 53.3% vs. 32.4% in permanent teeth. Teeth adjacent to the cleft side had a higher occurrence of DDE. The risk of bias assessment revealed that
most full articles were about the low-risk category.
Conclusion: The present study revealed that the prevalence of DDE in primary teeth was 53.3% vs.32.4%in permanent teeth. The prevalence of DDE in children with cleft lip/palate was high.Early detection of DDE, its effective preventive care,
and tooth monitoring are appropriate management of enamel defects in these children.

Keywords: Developmental defect of enamel, Cleft lip, Cleft palate