Amelogenesis imperfecta (AI) is a rare heterogeneous group of condition characterized by inherited enamel defect causing developmental, structural and chemical alteration of enamel. From understanding the molecular basis of this condition, presenting abnormal manifestation as thin, soft, irregular surfaces, rough enamel edges, fragile, pitted and/or badly discolored enamel with poor function and aesthetics. This leads to various problems such as early tooth loss wear, discolored teeth with severe embarrassment due to negative social outcome, discomfort following sensitivity, eating difﬁculties and sometimes pain. AI was ﬁrst diagnosed in early 1980ís, without any exact knowledge of gene theories. Later on in year 1991 the exact cause of AI was known from Mutations in the gene AMELX, encoding an extracellular matrix protein secreted by ameloblasts cells during enamel formation. This disorder has an adverse impact on oral health and the quality of life of the individual causing physiologic and psychological problems. The correction of such severely worn out dentition may require extensive multiple disciplinary restorative treatment to achieve appropriate functions with aesthetic results. It is important to identify the type of AI depending upon manifestations and factors that contribute to the excessive wear and loss of vertical dimension. The correction of the defects has to be done without violating the biologic or mechanical natural principles. Full mouth reconstruction in such patients improves esthetics, function and comfort. The following case report presents a systematic approach in rehabilitating a case of AI hypocalcified type using full ceramic restoration in anterior region using E-MAX crowns and metal reinforced porcelain restorations PFM crowns in posterior region.
Key words: Amelogenesis imperfect AI, Full mouth reconstruction, Restoration, Vertical dimension, Aesthetics E-MAX and PFM crowns.