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Year : 2013  |  Volume : 3  |  Issue : 1  |  Page : 20-22

Restoration of function and esthetics in a patient with amelogenesis imperfecta

1 Department of Pedodontics and Preventive Dentistry, H.P. Government Dental College and Hospital, Shimla, India
2 Department of Community Medicine, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Web Publication21-Jun-2013

Correspondence Address:
Deepak Chauhan
Department of Pedodontics and Preventive Dentistry, H.P. Government Dental College, Shimla - 171 001, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2230-7095.113823

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Amelogenesis imperfecta (AI) is a rare genetic disorder inherited as either autosomal dominant, autosomal recessive, or X- linked modes. AI is characterized by the defect in enamel formation, resulting in defective mineralization or formation of the matrix. Restoration of the dentition poses great challenges, especially when teeth are severely affected. Treatment aims to relieve pain or sensitivity of teeth and to preserve as much tooth structure as possible while preventing further loss of teeth, to maintain masticatory function, and last but not the least, to improve the aesthetic appearance and quality of life as it has a great psychological impact on the self-esteem of the patient. This case report describes the treatment of a young boy who presented with a severe form of AIimperfect (AI) with a minimally invasive, relatively simple, and cost-effective treatment with composite restorations.

Keywords: Amelogenesis imperfecta, hereditary composite restorations, enamel

How to cite this article:
Chauhan D, Sharma KR, Chauhan T. Restoration of function and esthetics in a patient with amelogenesis imperfecta. Int J Stud Res 2013;3:20-2

How to cite this URL:
Chauhan D, Sharma KR, Chauhan T. Restoration of function and esthetics in a patient with amelogenesis imperfecta. Int J Stud Res [serial online] 2013 [cited 2023 Jan 31];3:20-2. Available from: http://www.ijsronline.net/text.asp?2013/3/1/20/113823

  Introduction Top

Amelogenesis imperfecta (AI) is used to describe a number of conditions resulting from defective formation of the enamel. Many cases are inherited, either as autosomal dominant, recessive, or as X-linked traits. There have been a number of classifications, based primarily on the phenotype. [1],[2],[3] Tooth enamel is the most highly mineralized structure in the human body, with 85% of its volume occupied by hydroxyapatite crystals. [4],[5] The physical properties of enamel are directly related to the composition, orientation, disposition, and morphology of the mineral components within the tissue. [6] During organogenesis, the enamel transitions from a soft and pliable tissue to its final form, which is almost entirely devoid of protein. [4] However, a recent classification based on the molecular basis for the observed enamel phenotype may help clarify the commonly seen overlap in phenotypes. According to this classification, AI is a group of conditions, genetic in origin, which affect the structure and clinical appearance of the enamel of all, or nearly all the teeth, and which may be associated with morphologic or biochemical changes elsewhere in the body. [7] This anomaly of the enamel affects both primary and permanent dentition. [7],[8],[9],[10]

The appearance depends on the type of AI, varying from the mild hypo mature 'snow-capped' enamel to the more severe hereditary hypoplasia with thin enamel which has a yellow- brown appearance, resulting in dental problems like: sensitive teeth, poor aesthetic appearance and reduced masticatory function. Patient may develop gingivitis and periodontitis later because of cleaning difficulties arising due to roughness of enamel surface and pulpal involvement may also occur in severe cases. As AI affects both primary and permanent dentition, prevention of future dental problems must start from the beginning by educating the patient or parents regarding oral hygiene and reviewing at regular intervals. Attention must also be paid to the psychological health of the patient. [11]

  Case Report Top

An 11-year-old boy reported to the Department of Pedodontics and Preventive Dentistry of H.P. Government Dental College and Hospital; Shimla, Himachal Pradesh with the chief complaint of discolored teeth since childhood. Apart from this his past medical and family history was nonsignificant. On intra-oral examination, it was found that he had a normal complement of teeth. The thickness of enamel was reduced to a great extent and was almost completely chipped off from some teeth, exposing the dentin. The surfaces of the teeth had a rough texture exhibiting a yellowish brown discoloration, with diffuse pitting present on the exposed surfaces of the teeth, more prominent on the labial and buccal aspects. The pattern of emergence and timing of shedding of teeth seemed to be delayed than for the normal range. An open bite was present. Examination of the periodontium revealed the presence of chronic, generalized, marginal, and papillary gingivitis, with the deposition of calculus and unsatisfactory oral hygiene [Figure 1].
Figure 1: Periodontium revealed the presence of chronic, generalized, marginal, and papillary gingivitis with calculus deposits and yellowish brown discoloration of teeth with diffuse pitting present on tooth surfaces

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A provisional diagnosis of hypoplastic type ofAIwas made on the basis of typical, clinical, radiographic, and histological features, along with a differential diagnosis from environmental enamel hypoplasia, dentinogenesis imperfecta, dentin dysplasia, regional odontodysplasia, and the tricho-dento-osseous syndrome.

Radiographic investigations included an orthopantomogram (OPG) and full-mouth intraoral periapical (IOPA) radiographs. The OPG showed the presence of all unerupted permanent canines, premolars, and second molars [Figure 2].
Figure 2: An orthopantomogram (OPG) showing the presence of all unerupted canines, premolars, and second molars

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  Discussion Top

AI is a rare inherited disorder, ranging from a defect in the formation of the enamel to the coexistence of other medical disorders. [12],[13],[14] Both the primary and permanent dentitions are affected. In this case, the patient presented with severe tooth wear due to inherited abnormal development of the enamel. The poor appearance was due not only to the innate color of the teeth, but also to increased staining, chipping, and spacing between the teeth. Treatment of such patients with AI requires special care. They are sometimes psychologically affected due to the poor appearance of their teeth, and esthetic improvement can affect their self-confidence and esteem. In general, little is known about both the etiology of AI and the appropriate dental materials used to restore tooth defects. An appropriate selection of restorative materials is important to preserve function and occlusal harmony and to restore the natural dentition morphology. Resin composites, glass ionomers, modified glass ionomer cements, or a combination of these materials are recommended for restoring developmental dental enamel defects. Besides the esthetic properties of these materials, their adhesion to the structure of the tooth is an advantage that may avoid the unnecessary removal of sound teeth. In this case, it was clearly observed after esthetic restorations with the help of polycarboxylate crowns. Owing to the highly discolored dentine, a more opaque ceramic material had to be used. Although the crowns could have been improved further, as he was contended and were acceptable to him [Figure 3]; thus, no further alteration was done. The treatment required the commitment of a very long period, which was not possible as it would mean visits to the hospital for the patient from a far-flung area of the hilly state.
Figure 3: Postoperative photograph, showing markedly improvement in esthetic appearance of the patient

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During the treatment period, oral hygiene and dietary advice were reinforced so that future risk to the periodontium and development of dentalcaries could be prevented to achieve long-term success goals. In AI with the esthetic improvement, psychological health improves to restore quality of life. [15]

  Conclusion Top

Unfortunately, some patients seek clinical advice only when they present with problems like: poor esthetics, sensitivity of teeth, and gingivitis. These are the most common problems related to AI patients. Rather I would reiterate that, more complex problems like loss of occlusal vertical dimension and skeletal open bite should also be taken into consideration. In nutshell this case report demonstrates a minimally invasive, relatively simple, and cost-effective option for the correction of hypoplastic AI with composite restorations.

  References Top

1.Crawford PJ, Aldred MJ. Amelogenesis imperfect - A case report. Hong Kong Dent J 2006;3:123-7.   Back to cited text no. 1
2.Sundell S, Koch G. Hereditary amelogenesis imperfecta. Epidemiology and clinical classification in a Swedish child population. Swed Dent J 1985;9:157-69.  Back to cited text no. 2
3.Wright JT, Robinson C, Shore R. Characterization of the enamel ultra structure and mineral content in hypoplastic amelogenesis imperfecta. Oral Surg Oral Med Oral Pathol 1991;72:594-601.  Back to cited text no. 3
4.Robinson C, Briggs HD, Atkinson PJ, Weatherell JA. Matrix and mineral changes in developing enamel. J Dent Res 1979;58:871-82.   Back to cited text no. 4
5.Simmer JP, Fincham AG. Molecular mechanisms of dental enamel formation. Crit Rev Oral Biol Med 1995;6:84-108.   Back to cited text no. 5
6.Mahoney EK, Rohanizadeh R, Smail FS, Kilpatrick NM, Swain MV. Mechanical properties and microstructure of hypo mineralized enamel of permanent teeth. Biomaterials 2003;25:5091-100.   Back to cited text no. 6
7.Aldred MJ, Savarirayan R, Crawford PJ. Amelogenesis imperfecta: A classification and catalogue for the 21st century. Oral Dis 2003;9:19-23.  Back to cited text no. 7
8.Weinmann JP, Svoboda JF, Woods RW. Hereditary disturbances of enamel formation and calcification. J Am Dent Assoc 1945;32:397-418.  Back to cited text no. 8
9.Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. 2 nd ed. Philadelphia: Elsevier; 2002. p. 89-94.  Back to cited text no. 9
10.Rao S, Witkop CJ Jr. Inherited defects in tooth structure. Birth Defects Orig Artic Ser 1971;7:153-84.  Back to cited text no. 10
11.Coffield KD, Phillips C, Brady M, Roberts MW, Strauss RP, Wright JT. The psychological impact of developmental dental defects in people with hereditary amelogenesis imperfecta. J Am Dent Assoc 2005;136:620-30.  Back to cited text no. 11
12.Pindborg JJ. Aetiology of developmental enamel defects notrelated to fluorosis. Int Dent J 1982;32:123-34.  Back to cited text no. 12
13.Schulze C. Developmental abnormalities of the teeth and jaws. In: Gorlin RJ, Goldman HM, editors. Thomas' Oral Pathology. 6 th ed. St Louis: Mosby; 1970. p. 96-178.  Back to cited text no. 13
14.Michaelides M, Bloch-Zupan A, Holder GE, Hunt DM, Moore AT. An autosomal recessive cone-rod dystrophy associated with amelogenesis imperfecta. J Med Genet 2004;41:468-73.  Back to cited text no. 14
15.Croll TP. Restorative options for malformed permanent molars in children. Compend Contin Educ Dent 2000;21:676-8, 680, 682.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3]


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