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i. Type of manuscript : Case Report

ii. Title : Interdisciplinary treatment approach to the functional and aesthetic

rehabilitation of a young patient with Amelogenesis Imperfecta

iii. Name of the author and degree : Dr.Indrani Das, MDS (Prosthodontics)

iv. Professional affiliation: GNRC

v. Running Head : Functional and Aesthetic rehabilitation of an Amelogenesis

Imperfecta case

vi. Correspondence address

House no 24, Jyoti Path, Janakpur, Kahilipara, Guwahati, PIN-781019

Mobile No. : 8011674356

Mail id: inardni@gmail.com

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Abstract :

Amelogenesis Imperfecta (AI) encompasses a complicated group of hereditary

conditions that cause a developmental alterations in the quality and/or quantity of dental

enamel in the absence of systemic disorder. The treatment of such patients would not only

upgrade their quality of life, but also improves self- esteem. This article describes the

sequenced interdisciplinary treatment for a young female patient mutilated permanent

dentition caused by hypoplastic type using Hobo’s twin stage technique .

Key Words:

Amelogenesis imperfecta, full mouth rehabilitation, hypoplastic, decreased vertical

dimension, hobo twin stage

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Introduction :

Amelogenesis Imperfecta (AI) is a hereditary dysplasia affecting the structure of

dental enamel. The anomaly affect both primary and permanent teeth and causes them to be

unusually small, discoloured, pitted, grooved and prone to rapid wear and breakage (1). The

abnormality can be related to autosomal or x-linked, dominant or recessive modes. The most

common is autosomal dominant form. It is known that the gene responsible to codify the

most abundant protein of enamel, Amelogenin is related to occurrence of hypomineralised

enamel(2)(3). AI has an estimated prevalence of approximately 1:8000 and 1:700(4).

Investigations have demonstrated that it is possible to delineate 14 different hereditary sub-

types of AI that exist with numerous pattern of inheritance and wide variety of clinical

manifestation.

On the clinical and radiographic basis alone 3 broad groups can be distinguished

a) Hypoplastic which has reduced in quantity but well mineralised enamel

b) Hypocalcified which has poorly mineralised normal quantity enamel

c) Hypomaturation in which final stage of enamel maturation are abnormal(5-10).

According to literature, AI, regardless of the sub-types has similar oral complications.

Apart from enamel defects AI has been associated with inclusions and abnormalities in tooth

eruption, tooth sensitivity, poor aesthetics and decreased vertical dimension of occlusion (11).

Although the subtype and severity of AI may limit treatment potential, a recently published

survey reported the importance of treating AI patient not only from functional standpoint but

also from psycho-social healthpoint (12)(13). Treatment planning of AI patient is dependent

on age, socio-economic status of patient, the type and severity of the disorder and the intra-

oral situation at the time of treatment planning. This article describes the interdisciplinary

sequenced treatment approach for a young female patient with mutilated and discoloured

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permanent dentition caused by AI of hypoplastic type. A comprehensive management of this

condition with full mouth metal-ceramic restorations with a corrective vertical dimension of

occlusion was done using Hobo & Takayama approach of full mouth rehabilitation.

Clinical Report :

A 22 year old female patient reported with complaint of stained teeth, generalised

sensitivity and chipping of teeth(fig1). Her family history revealed similar condition of her

maternal cousin.On intraoral examination permanent teeth present were 11, 12, 43, 14,

15,16,17, 21,22, 53, 24, 25, 26, 27,31, 32, 63, 34, 35, 36,37, 41,42, 43, 44, 45, 46,47.

Gingival health of the patient was unremarkable and had a normal palatal arch. Past medical

history of the patient was nonsignificant and the patient appeared to be well nourished with

moderate height and built. Patient was advised for OPG, skull and chest X-Ray and full

mouth IOPA radiographs. Skull and Chest radiographs did not reveal any significant findings.

OPG revealed impacted 13, 23, 33, 18,28, 38,48(fig 2). No evidence of cysts, odontoma or

other abnormalities in the radiographs. To rule out any other associated syndrome complex or

any other metabolic or hormonal disorder, patient was referred to a physician under whose

supervision multiple tests were carried out but all of them were within normal limits.

A initial treatment plan after inter department disciplinary discussion were

i) Oral surgical : Extraction of impacted teeth and deciduous teeth under GA(fig.3)

ii) Periodontics : Crown lengthening of 11, 12 for aesthetic reason(fig.4)

iii) Endodontics : management of 11,12(fig.5)

iv) Prosthodontic rehabilitation.

After completion of pre-prosthetic procedures prosthodontic treatment protocol was

formulated according to Hobo & Takayama philosophy to restore the mutilated and attrided

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dentition in functional harmony with the stomatognathic system. An increase in 3 mm

vertical dimension was also planned. The amount of bite rise to be achieved was evaluated

using closest-S speaking space.

Procedure :

Phase I

Impressions of both arches were made using hydrocolloids and diagnostic casts were

obtained.Facebow transfer of maxillary cast was done on semi adjustable articulator

(Hanau) and mandibular cast was mounted with a Lucia jig in the anterior regions and

inter occlusal records in the posterior region.

Phase II

An occlusal splint was provided to the patient as part of reversible interventional

modalities to evaluate adaptation of the patient to alter VDO. Patient was kept in

diagnostic and observational period of 6 weeks before the definitive restorative phase

of rehabilitation started.A diagnostic wax up of full mouth restoration was carried out

at increased vertical dimension for posterior teeth without the anterior segment of

maxillary cast in place(fig.6). To produce standard effective cusp angles, the condylar

and incisal guidance were set to condition 1. At this position, the diagnostic wax up

was balanced in protrusive and lateral excursion. The anterior segments of cast was

reassembled and condylar and incisal guidance were set again (condition-2) and wax

up completed so as to generate posterior disoclusion.

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Phase III

Mouth preparation was done and stage-II temporaries were fabricated chair side

quadrant by quadrant(fig.7). During several appointments, patients VDO was

maintained by using unprepared second molar as occlusal vertical stops which are to

be prepared later.The second molars were prepared and stage-II temporaries were

fabricated using the index of the diagnostic wax-up and cemented with ZnO non

eugenol cement and left for 3 weeks.

Once the patient was adapted to this position, final full arch impressions were made

using polyvinyl siloxane impression material and casts were poured in die stone. This

casts are mounted on articulator using facebow transfer.Now to transfer the vertical

dimension and centric relation temporaries of left posterior regions were removed

while temporaries of the right side and the anterior regions acted as stop. Inter

occlusal recording material was injected between left maxillary and mandibular

prepared tooth. Similarly interocclusal record of right side was taken by keeping left

posterior temporaries as stop. Anterior inter-occlusal record was obtained by keeping

right and left posterior temporaries as stop(fig.8)(fig.9).Thus 3 segmental intra

occlusal records obtained were used to mount the mandibular cast.Wax pattern was

fabricated with anterior mandibular segment following conditions 1 & 2.All the wax

patterns were cast(fig.10) and bisque trial was done in the patient’s mouth. After

correcting the interferences the restorations were glazed and luted.PFM definite

restorations were cemented using GIC type-1 luting cement(fig.11). A group

functional type of occlusal scheme was provided.

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Phase IV

Oral hygiene instructions were given and follow up was done at regular intervals.

Anterior metal ceramic crowns were satisfactory both aesthetically and functionally at

the end of one year of clinical service and the patients oral hygiene was satisfactory.

Psychology of the patient was found to have greatly improved due to aesthetic

outcome. Restorations were intact without discoloration or carious lesions.

Discussion:

Treatment plan for AI cases is dependent on upon certain factors such as age, socio-

economic status of patients, type and severity of AI and intraoral condition at the time of

treatment planning. In the past such cases were radically treated with multiple extraction and

construction of complete denture. Such an approach has deleterious influence on

psychological health of patients. With advances in fixed prosthodontics for management of

mutilated dentition, such cases can be conservatively treated. In this case, the patient

presented with a decreased vertical dimension and increased freeway space so it was decided

to increase VD by 3 mm. The severely attrided anteriors resulted in loss of anterior guidance

so as to protect the posterior teeth from excursive movements. The posterior teeth attrition

resulted in the loss of occlusal plane and decreased vertical dimension. So in order to restore

function and health of the wornout dentition, the Hobo’s Twin stage procedure was planned.

There is some difference of opinion on whether to work simultaneously on both arches or

individually on different segments of the arch. Those who go for the segmental approach

state that it takes less time and is comfortable to patients.

Earlier gnathologists laid utmost importance to condylar guidance, and anterior

guidance determination was done by dentist. Thus, anterior guidance and condylar path were

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considered independent factors. However, recent concepts reveal that anterior guidance

influence the working condylar path and even changes when lateral component of incisal path

deviates from optimal orbit, thus supporting the hypothesis that anterior guidance and

condylar path are dependent factors.

Normally, anterior guidance is 50 steeper than condylar path in sagittal plane. Hence

when mandible is protruded, the anterior teeth guide it downward, creating posterior

disclusion. Similar disclusion is seen laterally because of steeper lingual inclination maxillary

canine. The angular difference between anterior guidance and condylar path assists posterior

disclusion, but is not solely accountable. The residual amount can be attributed to cusp shape

factor. However since CG, AG and cusp shape factor remain integral to Hobo’s approach so

this procedure cant be performed in cases of patient with abnormal curve of Spee , abnormal

curve of Wilson or having rotated or inclined tooth.

Conclusion :

This clinical report describes the interdisciplinary cooperation between Periodontist,

Oral Surgeon, Endodontist and Prosthodontist for the oral rehabilitation of a young female

patient affected by hypoplastic type of AI. Restoration of aesthetics and function with

meticulously done metal ceramic restoration based on concept of hobo’s twin stage technique

resulted in improved oral health impact profile

Fig 1: Pre-Operative Photos

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 Fig 2: Preoperative OPG Fig 3: Postoperative OPG

Fig 4: Crown Lengthening Was Done

Fig 5: RCT done in 11 & 2 Fig 6: Diagnostic wax up

Fig 7: Mouth Preparation Fig 8: Right and left Fig 9: Temporary restorations
Temporaries as occlusal stop

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 Fig 10: metal copings

Fig 11: Final prosthesis

References :

1. 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(3):161-170.

2. Lau EC, Mohandas TK, Shapiro LJ, Slavkin HC, Snead ML, Human and mouse

amelogenin gene loci are on the sex chromosomes. Genomics. 1989 Feb; 4(2):162-8.

3. Takagi Y, Fujita H, Katano H, Shimokawa H, Kuroda T. Immunochemical and

biochemical characteristics of enamel proteins in hypocalcified amelogenesis

imperfecta.. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998 Apr; 85(4):

424-30.

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4. Crawford PJ, Aldred MJ. X-linked amelogenesis imperfecta. Presentation of two

kindreds and a review of the literature. Oral Surg Oral Med Oral Pathol. 1992 Apr;

73(4): 449-55.

5. Witcop CJ, Sauk JJ. Heritable Defects of Enamel. In: Stewart R, Prscott G. Oro

Facial genetics. St. Louis: C. V. Mosby Company, 1996; 151-226

6. Sari T, Usumez A. Restoring function and esthetics in a patient with amelogenesis

imperfecta: a clinical report. J Prosthet Dent. 2003 Dec; 90(6): 522-5.

7. Winter GB, Lee KW, Johnson NW. Hereditary amelogenesis imperfecta. A rare

autosomal dominant type. Br Dent J. 1969 Aug 19; 127(4): 157-64.

8. Witkop CJ Jr. Partial expression of sex-linked recessive amelogenesis imperfecta in

females compatible with the Lyon hypothesis. Oral Surg Oral Med Oral Pathol. 1967

Feb; 23(2): 174-82.

9. Witkop C J, Kuhlmann W, Sauk J. Autosomal Recessive Pigmented Hypomaturation

Amelogenesis Imperfecta. Report of a Kindred. Oral Surg Oral Med Oral Pathol 1973;

36 (3), 367-382. 

10. Haug RH, Ferguson FS. X-linked recessive hypomaturation amelogenesis imperfecta:

report of case. J Am Dent Assoc. 1981 Jun; 102(6): 865-7.

11. Seow WK. Clinical diagnosis and management strategies of amelogenesis imperfecta

variants. Pediatr Dent. 1993 Nov-Dec; 15(6): 384-93.

12. Seow WK. Clinical diagnosis and management strategies of amelogenesis imperfecta

variants. Pediatr Dent. 1993 Nov-Dec; 15(6): 384-93.

13. Coffield KD, Phillips C, Brady M, Roberts MW, Strauss RP, Wright JT. The

psychosocial impact of developmental dental defects in people with hereditary

amelogenesis imperfect. J Am Dent Assoc. 2005 May; 136(5): 620-30.

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 Cover Letter For Submitting Manuscript For Publication

To,

The Editor in Chief ,

Journal of Applied Dental and Medical Sciences

I wish to submit a case report entitled “Interdisciplinary Treatment Approach to the

Functional and Aesthetic Rehabilitation of a young patient with Amelogenesis Imperfecta”. I

confirm that this work is original, and has not been published elsewhere, nor is it currently

under consideration for publication elsewhere.

I further like to state that I have no conflict of interest with anyone regarding this case

or publication.

Thank you for your kind consideration of this manuscript

Dr Indrani Das

MDS (Prosthodontics)

Consultant, GNRC Medical, North Guwahati

Author and person responsible for correspondence

Dr. Indrani Das,

House no 24, Jyoti Path,

Janakpur, Kahilipara,

Guwahati, PIN-781019

Mob No: 8011674356

Mail ID: inardni@gmail.com

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