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Table of Contents
Year : 2022  |  Volume : 12  |  Issue : 2  |  Page : 62-64

Full-mouth rehabilitation of amelogenesis imperfecta

Department of Pediatric Dentistry, Institute of Dental Studies and Technologies, Modinagar, Ghaziabad, Uttar Pradesh, India

Date of Submission30-Jun-2021
Date of Decision05-Dec-2021
Date of Acceptance18-Jun-2022
Date of Web Publication23-Aug-2022

Correspondence Address:
Dr. Nandita Waikhom
Department of Pediatric Dentistry, Institute of Dental Studies and Technologies, Modinagar, Ghaziabad, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jid.jid_24_21

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Amelogenesis imperfecta (AI) is a hereditary disorder expressed by a group of conditions which cause developmental alterations in the structure of enamel. Management of AI is very important because of esthetic and functional concerns as they create a positive psychological impact on the patient. A 12-year-old female patient reported with a complaint of yellowish discoloration of teeth with unesthetic appearance and painful sensitivity to mastication. The intraoral examination observed brownish discoloration of permanent anterior as well as posterior teeth of all segments. The height of the crowns of the upper and lower posterior teeth was also reduced. A hypoplastic type of AI was diagnosed in permanent dentition. A multidisciplinary planning was performed mainly to relieve dental sensitivity and improve masticatory function and esthetics of the patient and was carried out in multiple visits that ranged from preventive phase and restorative phase. Posttreatment improvements in esthetic as well as successful reduction in hypersensitivity were observed.

Keywords: Amelogenesis imperfecta, enamel hypoplasia, hypoplastic teeth

How to cite this article:
Jabin Z, Waikhom N, Agarwal N, Anand A. Full-mouth rehabilitation of amelogenesis imperfecta. J Interdiscip Dentistry 2022;12:62-4

How to cite this URL:
Jabin Z, Waikhom N, Agarwal N, Anand A. Full-mouth rehabilitation of amelogenesis imperfecta. J Interdiscip Dentistry [serial online] 2022 [cited 2023 Mar 22];12:62-4. Available from: https://www.jidonline.com/text.asp?2022/12/2/62/354449

   Clinical Relevance to Interdisciplinary Dentistry Top

This case report describes a multidisciplinary method that facilitates the functional, esthetic, and psychological management of a patient with amelogenesis imperfect.

  • Composite resin restorations were done in the anterior teeth to restore the esthetic of the patient.
  • Full-coverage stainless-steel crowns for the posterior teeth were indicated to fulfill the functional demands of the patient.

   Introduction Top

Amelogenesis imperfecta (AI) is a developmental disorder of the ameloblasts during the formation of enamel. During organogenesis, the enamel changes from soft tissue to its final form. The final form of enamel is a reflection of the molecular and cellular activities during its genesis. Any changes in this pattern may lead to AI.[1],[2] Causes of AI may be due to local infection or trauma, genetic, febrile illness, vitamin deficiency, fluoride ingestion, congenital syphilis, birth defects, or idiopathic factors.[3]

Studies have found that mutation or alteration in any of the genes such as enamelin gene (ENAM), amelogenin gene (AMELX), kallikrein 4 gene, matrix metalloproteinase 20 gene, and distal-less homeobox 3 genes can lead to AI.[4] The trait can be transmitted by either autosomal dominant, autosomal recessive, or X-linked modes of inheritance. It is seen affecting both primary and permanent dentition.[3],[5] Furthermore, only the enamel is affected, whereas dentin and root form are normal.[6] Besides the malformation of enamel, AI may be associated with other abnormalities such as delayed dental eruption, congenitally missing teeth, anterior open bite, pulpal calcifications, root and crown resorption, hypercementosis, root malformations, and taurodontism.[7]

The most commonly used classification was proposed by Witkop,[3] which was based on enamel appearance and hypothesized developmental defects. AI was classified into four main groups: Type I that involves disturbances related to enamel secretion (hypoplastic), Type II related to enamel maturation (hypomaturation), Type III that affects the mineralization process (hypocalcified), and Type IV, which is marked by the involvement of hypoplastic and hypomature enamel defects associated with taurodontism.

The primary clinical problems of AI are tooth sensitivity, breakdown of hard tissues resulting in loss of occlusal vertical dimension, dysfunction, and esthetics.[7],[8] Many factors are associated when managing patients with AI, i.e., the age and socioeconomic status of the patient, the type and severity of the disorder, and the intraoral situation.[9] The treatment should aim to reduce pain and sensitivity, prevent further tooth loss, and maintain mastication. Furthermore, care must be taken to improve the esthetics because this has a great psychological impact on the patient's confidence.

This case report describes the management of a young patient with a hypoplastic form of AI.

   Case Report Top

A 12-year-old female patient reported to the department of pedodontics and preventive dentistry with a chief complaint of yellowish discoloration of teeth for 5–6 years. Her dental history was suggestive of discolored deciduous teeth. When the parents were further questioned about the presence of similar abnormalities in the family, prenatal history, medication history during pregnancy, birth injury, and trauma, no relevant history was found.

Extraoral examination did not reveal any relevant findings. On intraoral examination, permanent anterior as well as posterior teeth of all segments showed brownish discoloration. A thin enamel layer covering all teeth was observed, which resulted in painful sensitivity to mastication and decrease of esthetics of anterior region. The height of the crowns of the upper and lower posterior teeth was reduced. Consistency of enamel and dentin was hard. Chipping of enamel was not present in any tooth [Figure 1].
Figure 1: (a) Rough pitted surface on labial surface of all the teeth. (b) Maxillary occlusal view. (c) Mandibular occlusal view

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Treatment was planned to relieve dental sensitivity and improve masticatory function and esthetics of the patient. The treatment was done in multiple visits that ranged from preventive phase and restorative phase, which included composite resin restorations for the permanent anterior teeth and placement of stainless-steel crowns for permanent molars [Figure 2].
Figure 2: (a) Composite resin restorations done for the front teeth. (b) Maxillary occlusal view. (c) Mandibular occlusal view

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Preventive phase

Application of 22,600 ppm of sodium fluoride varnish was done.

Restorative phase

Stainless-steel crowns were placed in all the four permanent first molars to improve the masticatory function. Anterior teeth were restored with composite resin restorations to improve the esthetics of the patient.

After the completion of the treatment, oral hygiene instructions were given to the patient. Regular brushing of the teeth using the modified bass technique was taught to the patient to be practiced twice a day (morning and at night before going to bed) using a soft toothbrush and a fluoridated toothpaste (containing 1000 ppm of available fluoride) followed by rinsing with 2% w/v of chlorhexidine gluconate for 15 days was also advised.

   Discussion Top

The finding in the present report showed discolored permanent anterior and posterior teeth with increased sensitivity to physical and chemical stimuli. Provisional diagnosis of hypoplastic AI was proposed along with differential diagnosis as fluorosis, molar-incisor hypomineralization. However, since there was no chipping of enamel and all teeth were involved, the case was identified as hypoplastic type of AI. The findings in the present report also correspond to the main complaints reported from patients affected by AI, which are unesthetic appearance, extensive loss of tooth structure, dental sensitivity, and loss of vertical dimension.[1],[6],[9]

In the present case report, the patient gives a history of similar clinical presentation in her deciduous dentition. Similar history was also observed in the case presentation by Mehta et al.[10]

The treatment planning of AI varies according to the patient's age, symptoms, type, the severity of the defect, and the intraoral situation at the time of the treatment.[9] Rehabilitation treatment is indicated in pediatric patients with AI with its main goal to improve the quality of life, which includes improving the function, protecting the enamel structure, and reducing the sensitivity and esthetics.[11]

Since the patient presented with generalized AL, comprehensive treatment was planned, which was undertaken in multiple appointments. AI patients usually present a thinner enamel, dental sensitivity, and more fragile structure that may result in enamel breakdown and fractures during the masticatory function. Hence, the first step for this case report was the preventive approach with 22,600 ppm of sodium fluoride. According to Petersson, it was observed that fluoride preparation in combination with dentin fluid obstruction agents is beneficial in reducing dental sensitivity. Fluoride's chemical ability reduces and blocks the fluid movements in the dentin tubules through the formation of calcium-phosphorous precipitates as well as calcium fluoride and fluorapatite.[12]

Many case reports have shown the predictability and high esthetics achieved with complete crowns. However, this approach requires the removal of a substantial amount of dental structure.[11] In this case report, the treatment performed was based on a conservative approach, as the child was young and in her teeth was in developing stage. Direct composite resin restorations were chosen for incisors as it requires minimal preparation of the enamel structure.

Attrition of teeth is a major problem in cases of AI. Therefore, the use of stainless-steel crowns as a preventive measure was advocated for posterior teeth. The crowns placement imparted better relationship between the upper and lower arches, protected the enamel structure, and promoted an adequate vertical dimension for the patient, improving the masticatory function. Since AI also results in pitting and roughened enamel which attracts plaque and increases caries susceptibility, the molars needed to be protected with SS crowns.

   Conclusion Top

AI is a serious dental problem that can result in reduced oral health-related quality of life and cause psychological problems. The performed conservative treatment resulted in decreasing the mentioned discomfort and contributed to protecting the pulp without further loss of dental hard tissues. Thus, it is important to diagnose the condition as early as possible to offer early intervention and improvement of the quality of life.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Chaudhary M, Dixit S, Singh A, Kunte S. Amelogenesis imperfecta: Report of a case and review of literature. J Oral Maxillofac Pathol 2009;13:70-7.  Back to cited text no. 1
[PUBMED]  [Full text]  
Paine ML, White SN, Luo W, Fong H, Sarikaya M, Snead ML. Regulated gene expression dictates enamel structure and tooth function. Matrix Biol 2001;20:273-92.  Back to cited text no. 2
Witkop CJ Jr. Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: Problems in classification. J Oral Pathol 1988;17:547-53.  Back to cited text no. 3
Sockalingam S. Dental rehabilitation of amelogenesis imperfecta using thermoformed templates. J Indian Soc Pedod Prev Dent 2011;29:53-6.  Back to cited text no. 4
[PUBMED]  [Full text]  
Crawford PJ, Aldred M, Bloch-Zupan A. Amelogenesis imperfecta. Orphanet J Rare Dis 2007;2:17.  Back to cited text no. 5
Bhateja S, Sahni P, Arora G, Solanki J. Amelogenesis imperfecta – A case report and literature review. Dent Impact 2014;6:15-9.  Back to cited text no. 6
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. 7
Parekh S, Almehateb M, Cunningham SJ. How do children with amelogenesis imperfecta feel about their teeth? Int J Paediatr Dent 2014;24:326-35.  Back to cited text no. 8
Bharath Shetty Y, Shetty A. Oral rehabilitation of a young adult with amelogenesis imperfecta: A clinical report. J Indian Prosthodont Soc 2010;10:240-5.  Back to cited text no. 9
Mehta DN, Shah J, Thakkar B. Amelogenesis imperfecta: Four case reports. J Nat Sci Biol Med 2013;4:462-5.  Back to cited text no. 10
Sabatini C, Guzmán-Armstrong S. A conservative treatment for amelogenesis imperfecta with direct resin composite restorations: A case report. J Esthet Restor Dent 2009;21:161-9.  Back to cited text no. 11
Petersson LG. The role of fluoride in the preventive management of dentin hypersensitivity and root caries. Clin Oral Investig 2013;17 Suppl 1:S63-71.  Back to cited text no. 12


  [Figure 1], [Figure 2]


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