Developmental Disorders

Amelogenesis imperfecta (AI) is a hereditary defect of enamel affecting both the primary and permanent dentition. By definition, AI includes only those cases where enamel defects occur in the absence of other syndromes or metabolic disorders. Although AI affects only the enamel formation, it has significant consequences for the affected patients. Often AI patients experience difficulty in maintaining oral hygiene, decreased masticatory function, and lower self-esteem, affecting their overall quality of life.

The severity of clinical problems varies with each type of AI. Unfortunately, restorative treatment for patients with AI is not often provided at an early age due to issues related to tooth sensitivity, difficulty in managing extensive treatment needs, and even cost. There is a tendency to adopt a “wait-and-see” policy, often resulting in the development of deep overbite and deleterious structural lost.

Full mouth rehabilitation combined with a multidisciplinary approach may be advantageous. Prosthodontics, periodontics, orthodontics, and endodontics may be necessary. Treatment could also include orthognathic surgery. Crown lengthening and gingival recontouring may be indicated in the case of short clinical crowns and gingival hyperplasia. Orthodontic treatments may be used to close interdental spaces prior to restoration and correct the anterior open bite malocclusion. Root canal therapy is indicated when pulp exposures are caused by severe attrition or tooth reduction. Orthognathic surgery may be indicated in case of severe malocclusion. Consultation with the appropriate specialists may help in developing a comprehensive treatment plan for each individual.

With the advance in techniques and increase in the availability of various dental materials, many studies have shown the use of glass ionomer cements, composite resin veneers, porcelain veneers, stainless steel crowns, lab-fabricated crowns, and/or overdentures can restore the affected teeth. The treatment approach should consider the specific AI type and underlying defect. In patients with hypoplastic AI, the enamel is usually sufficient for bonding so composite resin restoration may be successful masking discoloration and improving crown morphology. In patients with hypocalcified AI, there is insufficient enamel for bonding. Glass ionomer cements and composite resin restorations might initially be successful in these cases, but the long term-prognosis is guarded as the hypocalcified enamel may fracture, causing defective margins and broken restorations. Full coverage restorations are commonly recommended for hypocalcified AI. In the case of hypo maturation AI, the defective enamel contains excessive organic matter that over time becomes porous and stained; the defective enamel should be removed before placement of restorations.

Amelogenesis imperfecta is a group of inherited disorders that pose diagnostic and restorative treatment challenges for dental care providers. There is currently no standard of care established for managing patients with AI although a multidisciplinary approach may be advantageous. The cumulated evidence on outcomes of alternative restorations for each type of AI is critically needed. With such evidence, clinicians may then select more favorable approaches to treat individual AI patients and to optimize their patient’s oral health and long-term prognosis.