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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 5  |  Issue : 2  |  Page : 86-90

Multidisciplinary management of a case of dentinogenesis imperfecta


1 Department of Dentistry, Pondicherry Institute of Medical Sciences, Puducherry, Tamil Nadu, India
2 Department of Dental Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

Date of Web Publication28-Jan-2016

Correspondence Address:
Sajani Ramachandran
Pondicherry Institute of Medical Sciences, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-6360.175030

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  Abstract 


Preventive and restorative care are of utmost importance in the management of cases of dentinogenesis imperfecta in order to restore function, esthetics and also prevent further damage of the remaining dental tissues. The multidisciplinary approach would help to provide the optimal treatment outcome. This case report is about management of a case of dentinogenesis imperfecta with endodontic, periodontic, and prosthodontic intervention for a full mouth rehabilitation so as to improve the psychologic well-being of the patient in addition to all the other requirements.

Keywords: Dentinogenesis imperfecta; full mouth rehabilitation; reduced vertical dimension


How to cite this article:
Ramachandran S, Simon SS. Multidisciplinary management of a case of dentinogenesis imperfecta. Indian J Multidiscip Dent 2015;5:86-90

How to cite this URL:
Ramachandran S, Simon SS. Multidisciplinary management of a case of dentinogenesis imperfecta. Indian J Multidiscip Dent [serial online] 2015 [cited 2020 Oct 31];5:86-90. Available from: https://www.ijmdent.com/text.asp?2015/5/2/86/175030




  Introduction Top


Dentinogenesis imperfecta is a rare autosomal dominant disorder originating in the histodifferentiation stage of tooth development in which the dentin is abnormal in structure and is poorly attached to the enamel. This condition is also called as hereditary opalescent dentin due to clinical discoloration of the teeth.[1] Such dental abnormalities may occur as an isolated condition or in association with systemic conditions like osteogenesis imperfecta. The primary teeth are often found to be more severely affected than the permanent teeth, and the permanent teeth that develop early are found to be more affected than those that develop later. The crowns are usually bulbous with pronounced cervical constriction, and the roots may be short. There might be the progressive obliteration of pulp chambers and root canals due to secondary dentin deposition. In this condition periapical radiolucencies are also sometimes seen.[2]

Treatment of dentinogenesis imperfecta requires a team approach that is directed toward protecting the dental tissues from wear and improving the function and esthetic appearance of the teeth. The treatment protocol will depend on the clinical findings in each case.[3] The various factors that need to be considered while planning the treatment includes patients age, socioeconomic status, type of disorder, the quality of existing dental tissues, periodontal condition, and root pulp anomalies. The early diagnosis and appropriate treatment is of paramount significance to prevent psychological and functional morbidity to the patient. The hereditary dental anomalies can have profound negative consequences for the affected individual as well as the family. The problems range from esthetic concern that affects self-esteem to masticatory difficulties and tooth sensitivity. The present case report is about full mouth rehabilitation of a patient with dentinogenesis imperfecta with maxillary and mandibular fixed partial dentures, with utmost importance given to esthetics by obtaining an appropriate vertical dimension, so that the facial profile could be brought to a normal appearance.


  Case Report Top


A male patient aged 22 years reported to the dental outpatient department with the complaints of discolored and disfigured teeth. He complained of the teeth being chipped off frequently and was concerned about his unesthetic, disfigured teeth, and felt very embarrassed to smile. He had abnormal dentition since childhood and had no history of any major illness. He did not report any history of such a dentition in the family, and his parents did not have a consanguineous marriage and there was no history of any bone abnormalities.

On clinical examination, the patient had generalized yellowish brown discoloration and attrition of teeth in the lower arch [Figure 1] as well as the upper arch [Figure 2]. Root stumps of 16, 26, 31, 32, 41, and 42 were present. Some of the teeth were worn down to the gingival margin causing decreased vertical dimension [Figure 3]. An orthopantomogram [Figure 4] and full mouth intraoral periapical radiographs of the upper and lower posterior teeth [Figure 5]a upper and lower anterior teeth [Figure 5]b, and upper right and left posteriors [Figure 5]c were made. Many roots were found to be short and blunted. The periodontal condition was found to be sound with satisfactory oral hygiene. Based on the clinical examination and radiographic findings a diagnosis of dentinogenesis imperfecta was made.
Figure 1: Preoperative intraoral view of the lower arch with attrited anteriors

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Figure 2: Preoperative intraoral view of the upper arch

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Figure 3: Preoperative anterior intraoral view with reduced vertical dimension

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Figure 4: Orthopantomogram

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Figure 5: (a) Intraoral periapical radiograph of upper and lower posteriors. (b) Intraoral periapical radiograph of upper and lower anteriors. (c) Intraoral periapical radiograph of right and left upper posteriors

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Dentinogenesis imperfecta affects the structural integrity of the dentition that can result in some wear and fracture of posterior teeth which leads to loss of occlusal vertical dimension. The patient was explained about the cause of his present oral condition and the treatment plan to correct the same.

The aim of the treatment was to remove the source of infection, restore function, esthetics and protection of posterior teeth from wear, and maintenance of vertical dimension. The sources of infection were removed by endodontic therapy, and this was followed by full coverage crown for all the teeth to prevent further wearing of teeth and to replace the missing teeth in a reorganized occlusal scheme. After oral prophylaxis, endodontic treatment was done on 11, 12, 13, 14, 15, 17, 21, 22, 23, 24, 25, 27, 44, 45, 46, 33, 34, 35. Canals could not be negotiated in 13, 23, 14, 24 and hence was closed with calcium hydroxide. Prefabricated stainless steel post were used and core build up was done in 43 using composite resin and crown lengthening by gingivectomy was done in all the four quadrants so as to have sufficient crown length for restoration. Root stumps of 16, 26, 31, 32, 41, and 42 were extracted. Maxillary and mandibular impressions were made with irreversible hydrocolloid impression material alginate and study casts were made using Type 1 dental stone. The casts were mounted on a semi-adjustable articulator with the use of a face bow and centric record.

After a study of occlusion, it was planned to raise the vertical dimension by 2 mm to provide the space for the definitive restoration. An occlusal splint was provided for a period of 6 weeks to evaluate the adjustment of the patient to the altered vertical dimension of occlusion. Tooth preparation was done for porcelain fused metal fixed partial denture. The preparation was done quadrant wise from premolar to the molars posteriorly. The impression was made with polyvinyl siloxane impression material. The occlusal splint was modified to make space for new provisional crowns. The period was uneventful, and the patient was comfortable with the new centric relation and centric occlusion. The fabricated fixed partial dentures were cemented in the posteriors with glass ionomer luting cement. The anterior teeth preparation was done from canine to canine in the upper and lower arch. Impressions were made with polyvinyl siloxane material and porcelain fused to metal fixed partial denture were fabricated and cemented [Figure 6]. The prosthesis after cementation restored the lost vertical dimension [Figure 7] and the final esthetics and functional treatment outcome satisfied the patient and improved the psychological confidence of the patient [Figure 8].
Figure 6: Postoperative intra oral view

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Figure 7: Postoperative extraoral view with vertical dimension restored

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Figure 8: Postoperative extra oral anterior view

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  Discussion Top


Dentinogenesis imperfecta is an autosomal dominant disorder, and it occurs in 1:80000 whites in the United States. The dentin is often defective and gets easily separated from the enamel as a result of which the exposed dentin gets easily attrited. There is the absence of microscopic scalloping that is normally seen between dentin and enamel that is believed to help to mechanically lock the two hard tissues together. Radiographically often the teeth is found to have a bulbous crown, cervical constriction, thin roots and early obliteration of root canals and pulp chamber though in some cases teeth may show normal pulps or pulpal enlargement. Histologically the dentin appears as short misshapen tubules which coarse through an atypical granular dentin material which demonstrate interglobular calcification. Scanty atypical odontoblasts line the pulp surface, and cells may be seen entrapped within the defective dentin. The root canal becomes thread like and may develop micro exposure resulting in periapical inflammatory lesion.[4]

There are three types of dentinogenesis imperfecta. Type 1 is associated with osteogenesis imperfecta, mostly seen in deciduous dentition. Bone fracture, blue sclera, and progressive deafness are seen in this condition. Type II is hereditary opalescent dentin. The defect is equal in both dentitions. Type III Brandywine type associated with occasional shell teeth and multiple pulp exposures.[5] The severity of discoloration and fracturing of enamel in all types of dentinogenesis imperfecta is variable. Teeth in dentinogenesis imperfecta do not exhibit any greater susceptibility to caries and they may in fact show some resistance because of rapid wear and absence of interdental contacts. Opacification of dental pulps occurs because of continued deposition of abnormal dentin. The dentin is often soft and has abnormally high water content.[6] If left untreated it is not uncommon to see the entire dentition worn out to the gingival margin.

Multidisciplinary management of occlusion helps to provide optimal oral health and the management should be directed toward preventing severe attrition associated with loss of enamel and poorly mineralized dentin. In cases with severe enamel fracture and rapid dental wear the treatment of choice is full coverage restoration. Some of the patients have multiple periapical abscess due to pulpal strangulation secondary to pulpal obliteration, or from pulp exposure. Prophylactic endodontic treatment if done can ensure the long-term prognosis.[7] Attempting to negotiate obliterated canals in dentinogenesis imperfecta can result in lateral perforation due to poorly mineralized dentin.[8]

The objective of full mouth rehabilitation should be restoration and maintenance of health of entire oral cavity. It demands rehabilitation with the psychological and functional harmony of the stomatognathic system. Though many philosophies have been documented for rehabilitation of such cases the choice of treatment plan depends on the skill and experience of the clinician.


  Conclusion Top


Planning and executing the rehabilitation of an occlusion is probably one of the most technically demanding tasks for a restorative dentist. Occlusal rehabilitation in a dentate or partially dentate mouth is with the aim to provide an orderly path of occlusal contact and articulation that will optimize oral function, occlusal stability, and esthetics. Full mouth rehabilitation involves therapy that will by improving the relationship of the teeth will improve the condition and health of the supporting structures as well. This clinical report demonstrates a successful multidisciplinary approach to a full mouth rehabilitation of a patient were dentition has been esthetically and functionally compromised.

Declaration of patient consent

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

Acknowledgment

Professor Usha Carounanidhi, MDS, Principal, IGIDS, Puducherry.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Regezi JA, Sciubba JJ, Jordan RC. Oral Pathology: Clinical Pathologic Correlation. 6th ed. USA: Elsevier Publishing; 2011. p. 378-9.  Back to cited text no. 1
    
2.
Scully C. Oral and Maxillofacial Medicine: The Basics of Diagnosis and Treatment. 2nd ed. UK: Churchill Livingstone; 2008. p. 421-2.  Back to cited text no. 2
    
3.
Goud A, Deshpande S. Prosthodontic rehabilitation of dentinogenesis imperfecta. Contemp Clin Dent 2011;2:138-41.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.
Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology. 3rd ed. USA: Elsevier Publishing; 2009. p. 98-102.  Back to cited text no. 4
    
5.
Cawson RA, Odell EW. Essentials of Oral Pathology and Oral Medicine. 6th ed. UK: Churchill Livingstone Publisher; 2000. p. 20-1.  Back to cited text no. 5
    
6.
Garg SK, Bansal S, Mittal S. Dentinogenesis imperfecta – Aetiology and prosthodontic management. IJDS 2012:1;75-8.  Back to cited text no. 6
    
7.
Kar AK, Parkash H, Jain V. Full-mouth rehabilitation of a case of generalized enamel hypoplasia using a twin-stage procedure. Contemp Clin Dent 2010;1:98-102.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.
Shetty N, Joseph M, Basnet P, Dixit S. An integrated treatment approach: A case report for dentinogenesis imperfecta type II. Kathmandu Univ Med J (KUMJ) 2007;5:230-3.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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Introduction
Case Report
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