Amelogenesis Imperfecta (AI)
General Considerations and Principles of Management
A primary goal for treatment is to address each concern as it presents but with an overall comprehensive plan that outlines anticipated future treatment needs. Clinicians treating children and adolescents with AI must address the clinical and emotional demands of these disorders with sensitivity. It is important to establish good rapport with the child and family early. Timely intervention is critical to spare the patient from the psychosocial consequences of these potentially disfiguring conditions. A comprehensive and timely approach is reassuring to the patient and family and may help decrease their anxiety.
Preventive Care
Early identification and preventive interventions are critical for infants and children with AI in order to avoid the negative social and functional consequences of the disorder. Regular periodic examinations can identify teeth needing care as they erupt. Meticulous oral hygiene, calculus removal, and oral rinses can improve periodontal health. Fluoride applications and desensitizing agents may diminish tooth sensitivity.
Restorative Care
The appearance, quality, and amount of affected enamel and dentin will dictate the type of restorations necessary to achieve esthetic, masticatory, and functional health. When the enamel is intact but discolored, bleaching and/or microabrasion may be used to enhance the appearance. If the enamel is hypocalcified, composite resin or porcelain veneers may be able to be retained with bonding. If the enamel or dentin cannot be bonded, full coverage restorations will be required. In order to facilitate veneer or crown placement, periodontal therapy may be necessary when acute/chronic marginal gingivitis along with hyperplastic tissue exists.
During the primary dentition, it is important to restore the teeth for adequate function and to maintain adequate arch parameters. Primary teeth may require composite or veneered anterior crowns with posterior full coverage steel or veneered crowns.
The permanent dentition usually involves a complex treatment plan with specialists from multiple disciplines. Periodontics, endodontics, and orthodontics may be necessary and treatment could include orthognathic surgery. The prosthetic treatment may require veneers, full coverage crowns, implants, and fixed or removable prostheses. The fabrication of an occlusal splint is advocated to reestablish vertical dimension when full mouth rehabilitation is necessary. Therapy will need to be planned carefully in phases as teeth erupt and the need arises.
Behavior guidance, as well as the psychological health of the patient, will need to be addressed in each phase. Counseling for the child or adolescent and his/her family should be recommended when negative psychosocial consequences of the disorder are recognized. Due to extensive treatment needs, a patient may require sedation or general anesthesia for restorative care.
Dentinogenesis Imperfecta (DI)
General Considerations and Principles of Management
Providing optimal oral health treatment for DI frequently includes preventing severe attrition associated with enamel loss and rapid wear of the poorly mineralized dentin, rehabilitating dentitions that have undergone severe wear, optimizing esthetics, and preventing caries and periodontal disease. The dental approach for managing DI will vary depending on the severity of the clinical expression.
The clinician must be cautious in treating individuals with OI if performing surgical procedures or other treatment that could transmit forces to the jaws, increasing the risk of bone fracture. Some types of protective stabilization may be contraindicated in the patients with OI.
Preventive Care
Early identification and preventive interventions are critical for individuals with DI in order to avoid the negative social and functional consequences of the disorder. Regular periodic examinations can identify teeth needing care as they erupt. Meticulous oral hygiene, calculus removal, and oral rinses can improve periodontal health. Fluoride applications and desensitizing agents may diminish tooth sensitivity.
Restorative Care
Routine restorative techniques often can be used effectively to treat mild to moderate DI. These treatments more commonly are applied to the permanent teeth, as the permanent dentition frequently is less severely affected than the primary dentition. In more severe cases with significant enamel fracturing and rapid dental wear, the treatment of choice is full coverage restorations in both the primary and permanent dentitions. The success of full coverage is greatest in teeth with crowns and roots that exhibit close to a normal shape and size, minimizing the risk of cervical fracture.
Ideally, restorative stabilization of the dentition will be completed before excessive wear and loss of vertical dimension occur. Cases with significant loss of vertical dimension will benefit from reestablishing a more normal vertical dimension during dental rehabilitation. Cases having severe loss of coronal tooth structure and vertical dimension maybe considered candidates for overdenture therapy. Overlay dentures placed on teeth that are covered with fluoride-releasing glass ionomer cement have been used with success.
Bleaching has been reported to lighten the color of DI teeth with some success; however, because the discoloration is caused primarily by the underlying yellow-brown dentin, bleaching alone is unlikely to produce normal appearance in cases of significant discoloration. Different types of veneers can be used to improve the esthetics and mask the opalescent blue-gray discoloration of the anterior teeth.
Endodontic Considerations
Some patients with dentinogenesis imperfecta will suffer from multiple periapical abscesses apparently resulting from pulpal strangulation secondary to pulpal obliteration or from pulp exposure due to extensive coronal wear. The potential for periapical abscesses is an indication for periodic radiographic surveys on individuals with DI. Because of pulpal obliteration, apical surgery may be required to maintain the abscessed teeth. Attempting to negotiate and instrument obliterated canals in DI teeth can result in lateral perforation due to the poorly mineralized dentin.
Occlusion
Class III malocclusion with high incidences of posterior crossbites and openbites occur in DI Type I and should be evaluated. Multidisciplinary approaches are essential in addressing the complex needs of the individuals affected with DI.
Dentin Dysplasia (DD)
General Considerations and Principles of Management
The goal of treatment is to retain the teeth for as long as possible. However, due to shortened roots and periapical lesions, the prognosis for prolonged tooth retention is poor. Prosthetic replacement including dentures, overdentures, partial dentures, and/or dental implants may be required.
Preventive Care
Preventive care is of foremost importance. Meticulous oral hygiene must be established and maintained. As a result of shortened roots with DD Type I, early tooth loss from periodontitis is frequent.
Restorative Care
Teeth with DD Type I have such poor crown to root ratios that prosthetic replacement including dentures, overdentures, partial dentures, and/or dental implants are the only practical courses for dental rehabilitation. Teeth with DD Type II that are of normal shape, size, and support can be restored with full coverage restorations if necessary. For esthetics, discolored anterior teeth can be improved with resin bonding, veneering, or full coverage esthetic restorations.
Clinicians should be aware that even shallow occlusal restorations may result in pulpal necrosis due to the pulpal vascular channels that extend close to the dentin-enamel junction. If periapical inflammatory lesions develop, the treatment plan is guided by the root length.
Endodontic Considerations
Endodontic therapy, negotiating around pulp stones and through whorls of tubular dentin, has been successful in teeth without extremely short roots. Periapical curettage and retrograde amalgam seals have demonstrated short term success in teeth with short roots.
