Functional and Esthetic Rehabilitation of a Patient with Amelogenesis Imperfecta - A Case Report

INTRODUCTION

Amelogenesis Imperfecta (AI) is a hereditary condition where enamel formation is disturbed resulting in defects in mineralization and matrix formation. AI results in poor development or complete absence of the enamel of the teeth caused by improper differentiation of ameloblasts¹ .

The term amelogenesis imperfecta has been defined to include a variety of genetically determined disorders that primarily affect the enamel of all or nearly all the teeth without causing detectable alteration elsewhere in the body² . Thus amelogenesis imperfecta represents an inherited group of anomalies of enamel formation with an incidence of 1:718 to 1:14,000³ .

On the clinical and radiographic basis, three broad types exist, .The hypoplastic type of amelogesisis imperfecta is characterized by a deficiency in the quantity of enamel, which can be expressed clinically through fine enamel, or with grooves and pits on its surface. The hypocalcified types show enamel that has low mineralization, manifested clinically by pigmented, softened and easily detachable enamel. The hypomaturation types are associated with anomalies of the maturation stage during the formation of the enamel, resulting in an opaque and porous enamel^4,5.

AI does not directly increase the risk for the development of caries in the affected teeth, however, the absence of normal enamel morphology invariably results in diminished occlusal function, and often in severely compromised esthetics^{6, 7} .

Most reports of the treatment of AI in the dental literature have involved children and young adolescents. There are few reports regarding rehabilitation of older individuals. Treatment objectives for the adult patient include the relief of pain and the improvement of facial esthetics and function⁸ . The present article describes the full mouth rehabilitation of an adult diagnosed with amelogenesis imperfecta of hypo calcified type.

CASE REPORT

A 34 year old male patient reported to the Department of Conservative dentistry and Endodontics, A. B. Shetty Memorial Institute of Dental Sciences with the chief complaint of badly broken down teeth and unsatisfactory appearance. Patient gave history of chipping of teeth and eventual fracture of the anterior teeth. Since the esthetics was severely compromised, and the patient was unaware of the different treatment modalities, he wanted to undergo total extraction followed by complete denture.

His medical condition was good and his family history did not reveal anything significant. Patient gave the history of extraction of few teeth as they were grossly destructed.

On clinical examination, patient was normal built without any abnormalities. Oral examination revealed missing 16, 31, 32, 35, 36, 41, 42, 43, 44, 45, 46.Calculus deposits due to poor oral hygiene was observed. Enamel was thin on most areas and was absent on the occlusal surfaces due to attrition. (Figure 1). Soft tissue examination showed mild gingivitis.

Radiographically no enamel could be appreciated in most of the teeth that were present.(Figure 2). Patient exhibited no parafunctional habits. After thorough examination, the condition was diagnosed as amelogenesis imperfecta of hypocalcified type.

The main objective of the treatment was improvement of esthetics and masticatory function. Using diagnostic models, along with clinical and radiographic findings, the treatment plan was formulated. A systematic treatment plan was formulated which included endodontic, restorative and prosthetic approach. The patient was informed about the endodontic and restorative procedures, and also the prosthetic treatment procedures that were planned.

Treatment plan

1. Oral prophylaxis

2. Root canal treatment for all the mandibular teeth that were present and the maxillary anterior teeth and the premolars.

3. Custom made post and core for 33, 34, and the maxillary anterior teeth and maxillary right first premolar followed by ceramic crowns

4. Ceramic crowns for all the root canal treated teeth in the maxillary arch including bridge for the missing first molar.

5. Over denture for the mandible.

Treatment 

Oral prophylaxis was done and oral hygiene instructions and dietary advice was given. This was followed by root canal treatment of the maxillary anterior teeth and premolars and for the mandibular second molars and mandibular left canine and first premolar. RCT was done for maxillary premolars and mandibular molars as there was insufficient tooth structure and further reduction of tooth for crown placement would have resulted in pulpal exposure.

Since teeth 11 to 14 and 21 to 23 were badly broken down, RCT was followed by custom made post and core.( Figure 3). Crown lengthening procedure was performed on maxillary anterior teeth to increase the clinical crown length. Gold alloy was used to fabricate the posts for all the teeth. Post was placed on the buccal root of premolar. Provisional crown was cemented at the predetermined vertical dimension. Composite restoration was done for maxillary molars which had proximal caries.

Patient was further referred to the department of prosthetic dentistry where jaw relation was recorded and vertical dimension was increased. Following this, ceramic crowns were fabricated and cemented for maxillary teeth and over denture was fabricated for mandibular arch.( Figure 4).

DISCUSSION

Amelogenesis imperfecta is a condition that seriously compromises the oral and psychological health of the patient and it requires early recognition and action⁹ . Nearly all patients affected with amelogenesis imperfecta assessed themselves to be aesthetically disturbed by their pretreatment condition. The condition negatively affected the relationship with other people and their self-esteem. Prosthetic management had a positive influence & judged that the aesthetic rehablitation is the most important factor in treatment¹⁰ .

The different materials and methods for restorative procedures currently available have made it both exciting and confusing for dental practitioners. It should be pointed out limitations exist, and the application of techniques are not universal. It has been reported adhesive restorative techniques, over dentures, porcelain fused- to metal crowns, fixed partial dentures, full porcelain crowns, and inlay/only restorations are all used for the treatment of Amelogenesis Imperfecta¹¹.

Historically, patients with AI have been treated with extractions followed by construction of complete removable dentures. These options are psychologically harsh when the problem must be addressed in adolescent patients.^{11, 12}

In the present case the patient wanted to undergo total extraction and had already extracted most of his mandibular teeth. The patient was not aware of the condition he had and had opted for total extraction since he was not aware of the treatment modalities available to save his teeth. The patient was educated about the condition and was motivated to save the remaining teeth. The goal of the treatment was to improve oral health, protect and preserve the tooth structure and also to establish an aesthetic appearance and efficient masticatory function.

In the present situation, a porcelain fused to metal alloy approach was utilized for the restoration of the posterior as well as for the anterior teeth. Both the marginal fit and the color acceptability of the restorations were satisfactory.

Most of the cases of Amelogenesis Imperfecta may go undiagnosed and the patients go for total extraction. It is very essential to diagnose the condition properly and educate the patient about the condition and the various treatment options available to save teeth. Avoiding total extraction and going for endodontic, restorative and prosthetic approach to save teeth not only improves the aesthetic, functional and general oral condition but also have a positive psychological impact on the patient.

SUMMARY

The technical difficulties of treating patients with amelogenesis imperfecta are far outweighed by the psychosocial benefits to the affected individual. The treatment of patients should start with early and proper diagnosis and intervention to prevent restorative problems. Appropriate treatment plan with patient motivation and education is a must for rehabilitation of rare dental disorders. This case report describes the functional and esthetic rehabilitation of amelogenesis imperfecta with endodontic treatment followed by custom made post and core, PFM crowns, bridges and over denture.