To investigate the frequency of developmental defects of enamel (DDE) in children with intellectual disability. Children aged 5-18 years (72 children with intellectual disabilities and 72 controls) were included in the study. All the teeth were screened for developmental defects of enamel using the modified Developmental defects of enamel (mDDE) index. Out of the 72 children with intellectual disabilities in this study, 20 (27.78%) presented dental defects of enamel, compared with 8 (11.11%) of those in the control group, which was considered statistically significant (p = 0.021). The majority of children in both groups had white demarcated opacities. Children in both groups were more likely to have maxillary teeth affected than the mandibular teeth and the asymmetrical demarcated enamel defects were more common than the symmetric ones. Majority of opacities in children in both groups were on the maxillary incisors. Children with intellectual disabilities have more developmental defects of enamel than children in the control group. Enamel defects increase caries risk and cause reduction in enamel mechanical properties leading to restoration failures.
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