RESUMEN
BACKGROUND: Amelogenesis imperfecta (AI) represents a group of hereditary conditions which affects enamel formation in the primary and permanent dentitions. Gene mutations alter the quality and/or quantity of enamel. AI often has severe consequences for the patient such as high tooth sensitivity, low aesthetic quality of the dentition, and poor mechanical properties of the dental tissues. This can result in reduced oral health-related quality of life. CASE REPORT: We present the case of a child affected by AI which had been diagnosed at the age of 9 years. Teeth presented many enamel defects. The patient presented thin brown to yellow enamel and the surface was rough and granular. He revealed short clinical crowns, occlusal wear with exposed dentine in posterior areas. He also presented a lateral open bite and lingual lateral interposition due to partial destruction of deciduous molars. Panoramic radiograph showed no differences between enamel and dentine appearance and also coronary destruction of permanent molars. TREATMENT: The initial treatment consisted of adaptation of composite resins on permanent incisors to improve aesthetics. Preformed metal crowns were placed on first permanent molars to prevent their premature destruction. FOLLOW-UP: This was disrupted for a variety of reasons. Fourteen years later, after a dental nomadism, the patient consulted by chance a dentist who identified his genetic pathology and was aware of its consequences. Extensive prosthodontic treatment was needed, but oral hygiene was poor and gingivitis remained. CONCLUSION: Dealing with high loss of motivation has been one of the main challenges because this patient had a lot of psychological problems. He was concerned, as are many patients affected by AI or other enamel abnormalities. This paper highlights the difficulties of long-term care of this dental abnormality. Psychological aspects of the quality of life, which is a common feature in patients suffering from many kinds of enamel anomalies, are very important.