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Orphanet: Syndrome de brachyolmie amelogenese imparfaite

Amelogenesis imperfecta AI is a hereditary disorder that causes developmental alterations in the structure of enamel. In addition, tooth sensitivity, missing or impacted teeth, taurodontism, altered dental esthetics and anterior open bite can also iimparfaite associated with AI. This clinical report presents the diagnosis, treatment planning and prosthetic rehabilitation of a year-old female patient with AI associated with a group of dental anomalies.

Following clinical and radiographic examination, histologic imparfaiye of the teeth confirmed the diagnosis of rough pattern hypoplastic AI. The patient was rehabilitated with full-mouth zirconium oxide ceramic fixed bridges. Adaptation of the temporomandibular joints and masticatory muscles to the bridges was carefully observed over 3 years.

At the end of this follow-up period, the patient was satisfied with the esthetics, function and phonation of her prostheses. Amelogenesis imperfecta AI is a hereditary disorder, typically characterized by generalized enamel defects in both primary and permanent dentition.

AI may be an isolated finding or it may be part of a malformative syndrome, such as amelo-onycho-hypohidrotic syndrome, Morquio syndrome, Kohlschutter syndrome, imparfaiet syndrome, AI with taurodontism syndrome, oculo-dento-osseous dysplasia or epidermolysis bullosa hereditaria. Although the molecular basis of AI is not fully understood, in the past decade several mutations in the amelogenin AMEL X gene and enamelin gene have been identified in amelogenees with X-linked and autosomal forms of AI.

No cases of mutation in the Y-chromosome amelogenin gene have been reported. The main types of AI are correlated with defects in the enamel synthesis process and have been classified into four broad categories based primarily on phenotype.

Orphanet: Epilepsie demence amelogenese imparfaite

AI has been associated with several other dental anomalies, including dental or skeletal open bite, disturbances in eruption, congenitally missing teeth, pulpal calcification, hypercementosis, pathologic root and crown resorption, tooth sensitivity, poor dental esthetics, decreased vertical dimension and taurodontism.

The multidisciplinary team should be in close collaboration in terms of planning the immediate, transitory and long-term phases of treatment. The orthodontist and pediatric dentist play important roles with regard to mixed dentition. They help guide the prosthodontist and oral surgeon, who might be needed to treat the permanent dentition.

In addition, the role of the periodontist includes maintaining the health and structure of the masticatory system during and after AI treatment. The speech therapist plays an important role in restoring the system and in long-term management. The following clinical report describes the therapeutic planning and rehabilitative procedures experienced by a patient affected by AI.

Detailed dental, medical and social histories were obtained from the patient. Her general medical history was nonsignificant. She was born normally at term after an uneventful pregnancy. She was healthy and her general appearance was normal. A renal ultrasound scan was normal and showed no evidence of nephrocalcinosis. Laboratory findings, including serum electrolytes, calcium, phosphate, urea, creatinine, alkaline phosphatase and parathormone levels, were all normal; blood pressure was also normal.

However, plasma calcium levels in her mother and father were slightly increased. Clinical and radiographic examination of the patient revealed short crowns, occlusal wear with exposed dentin in the posterior areas and asymmetry of the gingival contours in the anterior maxillary teeth.

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Intraoral examination revealed yellow to yellowish-brown teeth with rough surfaces, conspicuous and irregular defects and a lack of contact points Fig. A deficiency in the mineral content of the enamel was evidenced by lack of radiographic opacity and a pathologic loss of enamel through wear and fracturing on both upper and lower teeth.

On amelogenee panoramic radiograph, the thin enamel layer ame,ogenese not be distinguished from the underlying imparfxite Fig. The roots were normal in length and form and pulp chambers were regular in size.

Root canal therapy and fillings were evident. In the left maxillary anterior region, the bite was open up to and including the first premolar. Oral examination showed soft tissue to be within normal limits, and no periodontal pockets were detected. Gingival tissue was enlarged in the molar region. Based on these clinical findings, the diagnosis was generalized hypoplastic AI, with almost normal crown contours suggestive of mild, localized hypoplasia.

Intraoral views before treatment show a discoloured teeth with rough surfaces and irregular defects, b class I molar occlusion on the right and c class III molar occlusion on the left. In consultation with the patient, full maxillary and mandibular rehabilitation with zirconium oxide ceramic crowns extending to the second molars was considered to be the best therapeutic option.

A diagnostic setup was prepared and informed consent was obtained from the patient. Under local anesthesia, teeth were prepared with circumferential shoulder margins of 1—1. Provisional restorations were fabricated by the dental technician and cemented with noneugenol zinc oxide amelogeneee TempBond NE, Kerr Corp, Orange, Calif. Panoramic radiography before treatment reveals a very thin enamel layer, normal roots and pulp chambers, as well as earlier root canal therapy and fillings.

Intraoral view after preparation of the teeth. Definitive impressions of the maxillary and mandibular teeth and abutments were made with a polyether impression material Impregum, 3M ESPE, St. The die model was scanned and the data were transmitted to a milling facility, where the frameworks were designed using White CAM 5. KG were manufactured with a thickness of 0. Occlusion was evaluated and adjusted intraorally to provide canine-guided consistent and regular contact between the crowns Fig.

The patient was instructed in the maintenance of interproximal gingival health using dental floss Super Floss, Oral-B, Boston, Mass. Routine panoramic radiographs were taken after treatment and annually during follow-up for 3 years Fig.

Zirconium oxide copings on the maxillary a and mandibular b master models. Intraoral impxrfaite during test positioning of the copings. Post-treatment intraoral a and radiographic b views showing restored teeth. The patient was very satisfied with the results in terms of both esthetics and function. Photographs taken after treatment: The patient was followed at 3, 6 and 12 months and then annually with visual and radiographic examinations. During the first year, hygiene and long-term outcome were assessed.

The patient acknowledged improved function and esthetics, and was pleased with the results Fig. The maxillary and mandibular left third molars, which were decayed, were extracted after rehabilitation of the patient.

The analysis showed that there was no enamel layer. Although, amelogeneee abnormality was present in the dentin, a thick primary dentin production had occurred on the coronal pulp chamber Fig.

While there were areas where the enamel had chipped off, a thin enamel layer with rough and nonuniform prismatic architecture was seen in some regions Fig.

Scanning electron micrograph of the same tooth shows areas with irregular and thin enamel layer white arrow and areas without enamel black arrow. Zirconium oxide-based restorative materials have excellent mechanical properties and low bacterial adhesion and they are biocompatible.

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This allows several applications in restorative dentistry, one of which is as a core material for ceramic crowns and fixed partial dentures. The maxillary and mandibular right and left third molars were fully erupted; therefore, the permanent restorative material, zirconium oxide ceramic, was selected as a suitable replacement for the imparcaite structures because of the attractive mechanical properties described above.

Previous studies have reported several methods for determining type of AI using combinations of clinical, radiographic, histologic and genetic criteria.

Although, SEM revealed a thin enamel layer with irregular structure, a thick layer of primary dentin had formed around the coronal pulp to resist traumatic forces. Patients with this anomaly often seek treatment because of an unpleasant appearance, impaired mastication and social embarrasment. In the present case, the main complaints were tooth discolouration and generalized sensitivity. The patient was dissatisfied with her dental appearance and concerned about the long-term condition of her teeth.

The aim was to satisfy her desire for esthetic improvement and not primarily to change her occlusal scheme. Therefore, full-mouth zirconium oxide ceramic rehabilitation of the patient was provided without changing the occlusal vertical dimension. After prosthetic rehabilitation, impartaite facial appearance and occlusion were improved. Long-term dental management consisted of regular clinical and radiographic reviews at 3, 6, 12, 24 and 36 months.

Amélogenèse imparfaite (AI)

Function, phonation and esthetic expectations of the patient were met. In the radiologic and clinical examination, no problem was seen in soft tissue or in maintenance of the restorations. Function, parafunction, aging and stress fatigue affect the longevity of restorations in the oral environment. In the presented case report, esthetic and functional rehabilitation of hypoplastic AI was performed with the use of zirconium oxide ceramic restorations.

Ergun is professor, department of prosthodontics, faculty of dentistry, Gazi University, Ankara, Turkey. Kaya is research assistant, department of prosthodontics, faculty of dentistry, Gazi University, Ankara, Turkey. Egilmez is research associate, department of prosthodontics, faculty of dentistry, Gazi University, Ankara, Turkey.

Cekic-Nagas is research associate, department of prosthodontics, faculty of dentistry, Gazi University, Ankara, Turkey. Abstract Amelogenesis imperfecta AI is a hereditary disorder that causes developmental alterations in the structure of enamel. This article has been peer reviewed. Interdisciplinary treatment for a patient with open-bite malocclusion and amelogenesis imperfecta.

Am J Orthod Dentofacial Orthop. Hypomaturation amelogenesis imperfecta due to WDR72 mutations: Amelogenesis imperfecta and nephrocalcinosis syndrome: Exclusion of known gene for enamel development in two Brazilian families with amelogenesis imperfecta. Case report of a rare syndrome associating amelogenesis imperfecta and nephrocalcinosis in a consanguineous family.

Functional and Esthetic Rehabilitation of a Patient with Amelogenesis Imperfecta

Novel ENAM mutation responsible for autosomal recessive amelogenesis imperfecta and localised enamel defects. Defining a new candidate gene for amelogenesis imperfecta: Amelogenesis imperfecta due to a mutation of the enamelin gene: A multidisciplinary approach to the functional and esthetic rehabilitation of amelogenesis imperfecta and open bite deformity: The efficacy of posterior three-unit zirconium-oxide-based ceramic fixed partial dental prostheses: Retention of zirconium oxide ceramic crowns with three types of cement.

Five-year prospective clinical study of posterior three-unit zirconia-based fixed dental prostheses. A prospective evaluation of zirconia posterior fixed dental prostheses: Rehabilitation of amelogenesis imperfecta using a reorganized approach: