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Case Report
Interdisciplinary management for
restoration of function and esthetics in
a patient with hereditary amelogenesis
imperfecta
Sushma Dhiman, Saba Khan, Sandhya Maheshwari, Jay S. Upadhyay

ABSTRACT
Amelogenesis imperfecta (AI) is a type of the hereditary disorder which is expressed as a group of conditions
Department of causing developmental alterations in the structure of enamel. It is associated with a reduction of oral
Orthodontics and
health‑related quality‑of‑life, has an impact on psychological well‑being, and leads to various physiological
Dentofacial Orthopaedics,
A.M.U., Aligarh, Uttar
problems. Children or adults with AI express varying degree of malocclusions either in the form of crowding,
Pradesh, India 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
Address for correspondence: dental anomalies.
Dr. Saba Khan,
E‑mail: sabakhan.ortho@
gmail.com KEY WORDS: Amelogenesis imperfecta, occlusal rehabilitation, porcelain crown

Introduction restore these enamel defects as early for esthetic and functionally
stable outcome, and also for psychological well‑being of the

A melogenesis imperfecta (AI) is a group of inherited

abnormalities of dental enamel.[1‑3] 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
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DOI:
10.4103/2348-2915.154646
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
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

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.

 30 Journal of Dental Research and Review ● Jan-Mar 2015 ● Vol. 2 ● Issue 1

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Dhiman, et al.: Interdisciplinary management of amelogenesis imperfecta

Figure 1: Intra-oral pretreatment photographs

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.

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
Figure 2: Pretreatment orthopantomogram
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 treatment duration unnecessarily. Hence, it was planned to go
respect to maxillary left central incisor. for the extraction of impacted 13, 23.

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.
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.

As impacted canines were not in favorable for orthodontic In phase II, extraction of impacted 13 and 23 was done.
eruption and surgical extrusion would have increased the Treatment was then began with preadjusted edgewise

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Dhiman, et al.: Interdisciplinary management of amelogenesis imperfecta
Figure 3: Intra-oral posttreatment photographs
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
Fitting of all restoration was good as shown in Figure 3. Altered
occlusal table was restored, and her masticatory efficiency was
increased.
Discussion
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
 32 Journal of Dental Research and Review ● Jan-Mar 2015 ● Vol. 2 ● Issue 1
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.[7‑10] 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
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
1. Rao S, Witkop CJ Jr. Inherited defects in tooth structure. Birth Defects
Orig Artic Ser 1971;7:153‑84.
2. Weinmann J, Svoboda J, Woods R. Hereditary disturbances of enamel
formation and calcification. J Am Dent Assoc 1945;32:397‑418.
3. Sundell S, Koch G. Hereditary amelogenesis imperfecta. I.
Epidemiology and clinical classification in a Swedish child population.
Swed Dent J 1985;9:157‑69.
4. Sari T, Usumez A. Restoring function and esthetics in a patient
with amelogenesis imperfecta: A clinical report. J Prosthet Dent
2003;90:522‑5.
5. 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.
6. Seow WK. Dental development in amelogenesis imperfecta: A
controlled study. Pediatr Dent 1995;17:26‑30.
7. 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.
8. Siadat H, Alikhasi M, Mirfazaelian A. Rehabilitation of a patient with
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J Prosthet Dent 2007;98:85‑8.
9. 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.
10. 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.
Source of Support: Nil, Conflict of Interest: None declared.

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Journal of Dental Research and Review ● Jan-Mar 2015 ● Vol. 2 ● Issue 1 33 