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Year : 2015  |  Volume : 2  |  Issue : 1  |  Page : 30-33

Interdisciplinary management for restoration of function and esthetics in a patient with hereditary amelogenesis imperfecta

Department of Orthodontics and Dentofacial Orthopaedics, A.M.U., Aligarh, Uttar Pradesh, India

Date of Web Publication8-Apr-2015

Correspondence Address:
Saba Khan
Department of Orthodontics and Dentofacial Orthopaedics, A.M.U., Aligarh, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2348-2915.154646

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Amelogenesis imperfecta (AI) is a type of the hereditary disorder which is expressed as a group of conditions causing developmental alterations in the structure of enamel. It is associated with a reduction of oral health-related quality-of-life, has an impact on psychological well-being, and leads to various physiological problems. Children or adults with AI express varying degree of malocclusions either in the form of crowding, impacted teeth, spacing, retained teeth, reduced vertical height due to abnormal tooth structure or undue tooth loss. Orthodontic treatment should precede esthetic rehabilitation. Proper diagnosis of the case is quintessential to provide durable functional and esthetic result to these patients, improving the quality of their lives. We present a case of interdisciplinary management for restoring function and esthetics in a patient with hereditary AI of the hypoplastic type accompanied with tooth impaction and some other dental anomalies.

Keywords: Amelogenesis imperfecta, occlusal rehabilitation, porcelain crown

How to cite this article:
Dhiman S, Khan S, Maheshwari S, Upadhyay JS. Interdisciplinary management for restoration of function and esthetics in a patient with hereditary amelogenesis imperfecta. J Dent Res Rev 2015;2:30-3

How to cite this URL:
Dhiman S, Khan S, Maheshwari S, Upadhyay JS. Interdisciplinary management for restoration of function and esthetics in a patient with hereditary amelogenesis imperfecta. J Dent Res Rev [serial online] 2015 [cited 2023 Mar 27];2:30-3. Available from: https://www.jdrr.org/text.asp?2015/2/1/30/154646

  Introduction Top

Amelogenesis imperfecta (AI) is a group of inherited abnormalities of dental enamel. [1],[2],[3] It may be classified into: Hypoplastic, hypocalcified, and hypomature, depending on the clinical presentation of the defects and the likely stage of enamel formation that is primarily affected. [2] Usually, both deciduous and permanent dentitions are involved. The defect lies in the enamel, the dentin and root form are usually normal. Although these teeth are subjected to more wear and tear, they are more resistant to decaying. The disease may be autosomal dominant, autosomal recessive or X-linked, it is genetically and clinically heterogeneous.

Esthetics, loss of vertical dimension due to occlusal wear resulting in masticatory dysfunction and tooth sensitivity are major clinical concerns in a patient of AI. It is important to restore these enamel defects as early for esthetic and functionally stable outcome, and also for psychological well-being of the patient 4. A tentative treatment plan in such patients must be chalked out according to age and socioeconomic status of the patient as well as type and severity of the disorder and the intraoral findings. [4] It is critical to evaluate, diagnose, and resolve concerns. The aim of this paper was to outline the management of esthetics and function with a multidisciplinary approach in a patient with AI of the hypoplastic type accompanied with tooth impaction and some other dental anomalies in 22-year-old female patient.

  Case Report Top

Diagnosis and etiology

A 22-year-old female patient presented with a chief complaint of discolored and irregular teeth as shown in [Figure 1]. Tooth shades varied from light to dark yellow. Severe attrition of buccal segments in both the arches was present, but the patient had no problem of sensitivity. History revealed that her primary dentition had a similar appearance. The patient's oral hygiene was satisfactory, along with some hypoplastic carious lesions. Vitality tests were done and teeth 24, 25, 45 were found to be nonvital. Rest of the teeth were vital and nontender on percussion. Cusps of some teeth had crumbled appearance. Her maternal grandfather and two of her sisters had the same appearance of teeth. Hence, etiology of her condition was attributed to a hereditary cause, and the patient was diagnosed as a case of AI with X-linked recessive pattern of inheritance.
Figure 1: Intra-oral pretreatment photographs

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All orthodontic records were obtained prior to treatment as shown in [Figure 2]. Patient had impacted 13, 23; retained deciduous canine in right maxillary region and lateral incisor in left maxillary region. Permanent maxillary lateral incisors were missing bilaterally. Third molar on the right side was missing. Mandibular first premolars were rotated mesiolingually. Her 36 was already extracted few years back due to caries and 37 had tipped mesially into the extraction space. The patient had crowding in the mandibular arch and anterior crossbite with respect to maxillary left central incisor.
Figure 2: Pretreatment orthopantomogram

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The enamel of the teeth appeared to have the same radiodensity as dentin. Roots had normal anatomy. Clinically, patient showed a convex facial profile with competent lips and frontal symmetry. Cephalometric reading showed that patient had skeletal class I base with hyperdivergent growth pattern.

Treatment objectives for this patient were (1) Management of grossly carious teeth; prevention of further deterioration of the remaining dentition; (2) alignment of teeth; (3) improvement of esthetics and function; (4) extraction of impacted maxillary canines; (5) management of missing lateral incisors; (6) patient education and motivation, and (7) regular follow-up.

Treatment plan

Included oral prophylaxis, restoration of all decayed teeth, root canal treatment of 24, 25, 45, and their esthetic rehabilitation with metal ceramic crown. To prevent further detrition of molars they would be covered with metal ceramic crown after crown lengthening, prior to commencement of orthodontic treatment.

As impacted canines were not in favorable for orthodontic eruption and surgical extrusion would have increased the treatment duration unnecessarily. Hence, it was planned to go for the extraction of impacted 13, 23.

Orthodontic uprighting of 37, followed by protraction of 35 into the extracted space of 36 to relieve crowding in the anterior region and finally prosthetic replacement of remaining space was planned.

It was also planned to do periodontal correction of gingival contours in the anterior region.

Finally, esthetic rehabilitation with porcelain veneers in maxillary anterior region and metal ceramic crown in rest of the teeth.

Treatment progress

After discussing the treatment plan with the patient, informed consent was obtained. Treatment was done in three phases. In the first phase, root canal treatment of 24, 25, and 45 was done. 1-month after root canal treatment, crowns were prepared. 3 unit Metal ceramic fixed bridge was given with respect to 24, 25 and 26. 37 and 45, 46 were also restored with Metal ceramic crowns.

In phase II, extraction of impacted 13 and 23 was done. Treatment was then began with preadjusted edgewise appliance. Bonding was done including retained deciduous teeth. Banding was done in all teeth that were restored with Metal ceramic crowns. Metal ceramic crown kept dislodging repeatedly, so crown lengthening of 37 was done one more time, and a metal crown was cemented on the tooth with buccal tube directly welded to it. Then uprighting of 37 was done. 35 could not be protracted in the extraction space even after 3 months of the treatment, so extraction of one of her lower central incisor was done to relieve crowding in mandibular anterior region. Mandibular first molars were de-rotated. This phase took near about 1-year. Debonding was done thereafter followed by a retention period of 6 months. Suprcrestal fibrotomy was done with respect to rotated mandibular first premolars.

After alignment and space closure in Phase III Maxillary anterior teeth were restored with Porcelain veneers including first premolars on both sides. Mandibular anteriors were restored with metal ceramic crown. Three unit metal-ceramic fixed partial denture was given with respect to 45, 46, and 47.

  Treatment results Top

Fitting of all restoration was good as shown in [Figure 3]. Altered occlusal table was restored, and her masticatory efficiency was increased.
Figure 3: Intra-oral posttreatment photographs

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  Discussion Top

With AI, there are problems of socialization and impaired function which must be managed by early intervention, with careful treatment planning, in both preventive and restorative aspect. It is associated with a high morbidity for the patients and may present major restorative and sometimes orthodontic challenges for the dental team. Children or adults with AI are not without malocclusions, they can have crowding, impacted teeth, spacings, retained teeth, reduced vertical height due to tooth structure or even due to tooth loss 50% of AI patients with malocclusions have associated skeletal anterior open bites, without the significance of this association being elucidated. [5] Seow showed that people with AI have 6 times the tendency of unaffected people to have impaction of permanent teeth. [6] Orthodontic correction must precede final restorative treatment. A number of studies have been reported about the early or late orthodontic interventions in AI. [7],[8] However, orthodontic treatment with fixed appliances has been disregarded in these cases. In literature, there is only one case report treated by fixed orthodontic appliances. In general, removable appliances are used to correct the possible malocclusions seen in these cases. [7],[8],[9],[10] It is well-known that excellent functional and esthetics outcomes can be obtained only by fixed orthodontic mechanics.

Restorative treatment requires long-term analysis and good oral care practice. Root canal therapy and esthetic crown replacement for decayed teeth should be done. A multi-disciplinary approach consisting of an orthodontist, pediatric dentist and an endodontist, prosthodontist, periodontist, oral surgeon should be planned at an early age.

  Conclusion Top

Interdisciplinary treatment of AI not only improves the overall health and appearance of the dentition, but greatly enhances the patient's emotional well-being and quality-of-life.

  References Top

Rao S, Witkop CJ Jr. Inherited defects in tooth structure. Birth Defects Orig Artic Ser 1971;7:153-84.  Back to cited text no. 1
Weinmann J, Svoboda J, Woods R. Hereditary disturbances of enamel formation and calcification. J Am Dent Assoc 1945;32:397-418.  Back to cited text no. 2
Sundell S, Koch G. Hereditary amelogenesis imperfecta. I. Epidemiology and clinical classification in a Swedish child population. Swed Dent J 1985;9:157-69.  Back to cited text no. 3
Sari T, Usumez A. Restoring function and esthetics in a patient with amelogenesis imperfecta: A clinical report. J Prosthet Dent 2003;90:522-5.  Back to cited text no. 4
Poulsen S, Gjørup H, Haubek D, Haukali G, Hintze H, Løvschall H, et al. Amelogenesis imperfecta - A systematic literature review of associated dental and oro-facial abnormalities and their impact on patients. Acta Odontol Scand 2008;66:193-9.  Back to cited text no. 5
Seow WK. Dental development in amelogenesis imperfecta: A controlled study. Pediatr Dent 1995;17:26-30.  Back to cited text no. 6
Ozturk N, Sari Z, Ozturk B. An interdisciplinary approach for restoring function and esthetics in a patient with amelogenesis imperfecta and malocclusion: A clinical report. J Prosthet Dent 2004;92:112-5.  Back to cited text no. 7
Siadat H, Alikhasi M, Mirfazaelian A. Rehabilitation of a patient with amelogenesis imperfecta using all-ceramic crowns: A clinical report. J Prosthet Dent 2007;98:85-8.  Back to cited text no. 8
Ayers KM, Drummond BK, Harding WJ, Salis SG, Liston PN. Amelogenesis imperfecta - Multidisciplinary management from eruption to adulthood. Review and case report. N Z Dent J 2004;100:101-4.  Back to cited text no. 9
Keles A, Pamukcu B, Isik F, Gemalmaz D, Güzel MZ. Improving quality of life with a team approach: A case report. Int J Adult Orthodon Orthognath Surg 2001;16:293-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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