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CASE REPORTCASE REPORTCASE REPORTCASE REPORT

42 ARCHIVES OF DENTAL AND MEDICAL RESEARCH

Dentinogenesis Imperfecta: A Case Report Versha Rani Giroh1, Manjula Hebbale1Post Graduate Student, Department of Oral Medicine and Radiology, Bharati Dental College and Hospital, Pune, IndiaBharati Vidyapeeth Deemed University Dental College and Hospital, Pune, IndiaDepartment, Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune, India Address for Correspondence: Dr. Versha Rani Giroh, Post Graduate Student, Department of Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune, India

ABSTRACT: Dentinogenesis imperfecta (DGI) is an autosomal dominant disorder which is characterized by defective dentine formation. The defect arises in the histodifferentiation stage during the tooth development. The teeth appeardentitions or both. The condition may or may not be found in association with osteogenesis imperfecta. This condition has a reported incidence of 1/6000 to 1/8000. namely type I (DGI with osteogenesis imperfecta), type II (DGI without osteogenesis imperfecta) and type III (DGI associated with Brandywine triracial isolates). Treatment involves multidisciplinary approach to provide optimal oral health, restoration of function and aesthetics. Keywords: Endodontic failure, Retreatment, V INTRODUCTION Dentinogenesis imperfecta is a hereditary disorder of dentin formation resulting inabnormal dentin formation.1 reported by Barret in 1882.2 It is in accordance to autosomal dominant Mandelian trait.Synonyms are Hereditary opalescent dentin due to clinical discoloration of teeth or capdepont dysplasia.4 Molecular etiology is found to be related with dentine sialo phosphoprotein geneDSPP is located within the chromosome 4q22.1 in human. DSPP expression is hundred times higher in dentin, it is expressed in bone, kidney, salivary glands as well.1 Absence of microscopic scalloping which is normally seen between dentin and enamel is reported. Due to this, the dentin gets easily separated from the enamel resulting attrition.5 DGI may affect both the dentitions but has preponderance towards the primary dentition. The permanent teeth which erupt early are found to be more affected.Clinically, the color of the teeth varies from brown to blue, amber or grey, with an

ARCHIVES OF DENTAL AND MEDICAL RESEARCH Vol 2 Issue 5

Dentinogenesis Imperfecta: A Case Report

Manjula Hebbale2, Amit Mhapuskar3, Shweta ThakarePost Graduate Student, Department of Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University

Dental College and Hospital, Pune, India; 2Associate Professor, Department of Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune, India; 3 Professor and Head of the

ral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and

Post Graduate Student, Department of Oral Medicine and Radiology, Bharati sity Dental College and Hospital, Pune, India.

(DGI) is an autosomal dominant disorder which is characterized by

defective dentine formation. The defect arises in the histodifferentiation stage during the tooth development. The teeth appear opalescent. The condition may affect the primary or permanent dentitions or both. The condition may or may not be found in association with osteogenesis

This condition has a reported incidence of 1/6000 to 1/8000. There are three subnamely type I (DGI with osteogenesis imperfecta), type II (DGI without osteogenesis imperfecta) and type III (DGI associated with Brandywine triracial isolates). Treatment involves multidisciplinary

l health, restoration of function and aesthetics.

dodontic failure, Retreatment, Variations in dental anatomy.

Dentinogenesis imperfecta is a hereditary disorder of dentin formation resulting in

It was first It is in accordance

al dominant Mandelian trait.3 Synonyms are Hereditary opalescent dentin due to clinical discoloration of teeth or

is found to be related with dentine sialo phosphoprotein gene (DSPP). DSPP is located within the chromosome

DSPP expression is hundred times higher in dentin, it is expressed in bone,

c scalloping which is

normally seen between dentin and enamel is reported. Due to this, the dentin gets easily separated from the enamel resulting in

DGI may affect both the dentitions but has preponderance towards the primary

rmanent teeth which erupt affected.8

Clinically, the color of the teeth varies from brown to blue, amber or grey, with an

opalescent shine.9 The crowns are bulbous with cervical constriction. Radiographically, Obliteration of pulp chambers and root canals due to secondary dentin deposition are seen. Normal roots or short roots are seen. Sometimes, root may be absent. CASE REPORT A 32-year-old female patient reported to the department of oral medicine and radiology with a chief complaint of sensitivity in all her teeth since 2 months. She complains of sensitivity on eating cold, sweet and sour food items. Sensitivity lingers for around 2minutes after removal of the stimulus.History revealed that her primary dentition had a similar yellowish discoloration. Her mandibular right and left back teeth were extracted as the teeth were decayed. Her family and social history was noncontributory. No history of consanguineous parents reported. There was no history of any unusual bone brittleness or any other systemic illness. Extra-oral examination was noncontributory.

AODMR

Shweta Thakare1

Vidyapeeth Deemed University Associate Professor, Department of Oral Medicine and Radiology,

Professor and Head of the ral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and

Post Graduate Student, Department of Oral Medicine and Radiology, Bharati

(DGI) is an autosomal dominant disorder which is characterized by defective dentine formation. The defect arises in the histodifferentiation stage during the tooth

opalescent. The condition may affect the primary or permanent dentitions or both. The condition may or may not be found in association with osteogenesis

There are three sub-types namely type I (DGI with osteogenesis imperfecta), type II (DGI without osteogenesis imperfecta) and type III (DGI associated with Brandywine triracial isolates). Treatment involves multidisciplinary

The crowns are bulbous with cervical constriction. Radiographically,

p chambers and root canals due to secondary dentin deposition are seen. Normal roots or short roots are seen.

times, root may be absent.1,8

old female patient reported to the department of oral medicine and radiology

complaint of sensitivity in all her teeth since 2 months. She complains of sensitivity on eating cold, sweet and sour food items. Sensitivity lingers for around 2-3 minutes after removal of the stimulus. History revealed that her primary dentition had

imilar yellowish discoloration. Her mandibular right and left back teeth were extracted as the teeth were decayed. Her family and social history was non-contributory. No history of consanguineous parents reported. There was no history of any

rittleness or any other systemic oral examination was non-

Giroh et al


On intra-oral examination, all maxillary teeth were present whereas mandibular right and left posterior teeth were missing. Root stump of maxillary left canine was seen.yellowish discoloration of teeth was seen(Figure 1). Generalized attrition was present. The maxillary left first premolar and second

A)

E) F) G)

Giroh et al: Dentinogenesis Imperfecta


oral examination, all maxillary teeth were present whereas mandibular right and left posterior teeth were missing. Root stump of maxillary left canine was seen. Generalized yellowish discoloration of teeth was seen

1). Generalized attrition was present. The maxillary left first premolar and second

molar were carious. Also, premolars and right third molar were be decayed. Investigations advised were intraoral periapical radiographs (Figure Orthopantomogram (Figure computed tomography scanprosthetic rehabilitation.

Figure 1: Intra oral images (A,B,C,D,E)

B) C)

E) F) G)

Figure 2: Intraoral Radiograph (A,B,C,D,E,F,G)

Also, mandibular left and right third molar were found to

tions advised were al periapical radiographs (Figure 2),

(Figure 3) and cone beam computed tomography scan (Figure 4) for

B) C)

Giroh et al


On the basis of clinical examination, a provisional diagnosis of dentinogenesis imperfecta was made. The Intraoral periapical radiographs revealed complete loss of enamel and obliteration of pulp space, bulbous crowns with cervical constriction. Orthopantomogram revealed generalized loss of enamel. Cone beam computed tomography scan was noncontributory in the diagnosis but helped in the evaluation for the implant placement.

deposition, leading to defective dentinoenamel junction and hence the easy chipping of the enamel. The transparent appearance of the dentin is due to reduced number of dentinal tubules or complete absence of dentinal tubules. There is less of calcium (Ca),phosphorus (P), magnesium, a higher Ca:P ratio, and higher water content within the affected dentin. The main cause for premature fracture in DGI is the lower mineral concentration as compared to normal



Figure 3: Orthopantomogram

On the basis of clinical examination, a provisional diagnosis of dentinogenesis

The Intraoral periapical radiographs revealed complete loss of enamel and obliteration of

ns with cervical constriction. Orthopantomogram revealed generalized loss of enamel. Cone beam computed tomography scan was non-contributory in the diagnosis but helped in the evaluation for the implant placement.

The treatment plan advised included prosthrehabilitation and restoration of the decayed teeth. Also, extraction of the maxillary left canine (root stump) was advised. DISCUSSION Kerbel et al.,10 and Wright et abnormal dentinal tubules with defect in the dentinal calcification. Though the dentin structure found to be defective, the enamel and other structures such as cementum, periodontal ligament are normal. Due to defective dentin

Figure 4: Reconstructed CBCT Panoramic Image.

deposition, leading to defective dentinoenamel junction and hence the easy chipping of the enamel. The transparent appearance of the dentin is due to reduced number of dentinal tubules or complete absence of dentinal tubules. There is less of calcium (Ca), phosphorus (P), magnesium, a higher Ca:P ratio, and higher water content within the affected dentin. The main cause for premature fracture in DGI is the lower mineral concentration as compared to normal dentin.15

Shields and co-workers classified DGI intothree types:13 Type I - DGI in association with osteogenesis imperfecta Type II - DGI without osteogenesis imperfectaType III – Brandywine type. It is associated with Brandywine triracial isolates and characterized by shell teeth, with very less dentin and multiple pulp exprimary teeth.14

The treatment plan advised included prosthetic rehabilitation and restoration of the decayed teeth. Also, extraction of the maxillary left canine (root stump) was advised.

and Wright et al11 showed abnormal dentinal tubules with defect in the dentinal calcification. Though the dentin structure found to be defective, the enamel and

ementum, periodontal re normal. Due to defective dentin

workers classified DGI into

DGI in association with osteogenesis

DGI without osteogenesis imperfecta Brandywine type. It is associated

with Brandywine triracial isolates and characterized by shell teeth, with very less

nd multiple pulp exposures in the


45 ARCHIVES OF DENTAL AND MEDICAL RESEARCH Vol 2 Issue 5

A revised classification was given on the basis of the extensive research that has shown DGI and osteogenesis imperfecta are two different unrelated entities. The classification is as follows:

• DGI I corresponds to DI type II • DGI II corresponds to the DI type III

of Shields classification, respectively.4 In the present case, the absence of osteogenesis imperfecta and any other systemic disease,makes the diagnosis of DG I II favorable. According to revised classification, present case falls under the category DGI I. Also, generalized yellowish discoloration, distributed in all the four quadrants was seen. It was observed that generalized attrition of the crowns caused a decrease in vertical dimension. Treatment includes protection of the teeth from attrition, functional rehabilitation and aesthetic improvement. The treatment varies according to the patient’s age and presenting complaint.1 If the primary teeth are involved, stainless steel crowns may be used and to improve the aesthetics, composite facing crowns plays a crucial role.12,13 Overdentures may be given in conditions when severe attrition is present.1 If the permanent teeth are involved, preservation or restoration of the vertical dimension is mandatory. Importance should be given to minimal tooth preparation until child reaches adulthood. Full mouth rehabilitation is generally the choice of treatment in case of severely affected teeth.14 Endodontic treatment may be difficult to perform as obliteration of pulp chamber are seen.1 Prognosis is questionable even after endodontic treatment due to the increased probability of fracture. CONCLUSION Due to the excessive wear and premature micro injuries, the treatment planning is the most important factor. Treatment is multidisciplinary which includes pedodontics, prosthodontics, orthodontics, endodontics. Full mouth rehabilitation is required to improve the

condition and health of the supporting structures. Early diagnosis and long term follow up plays an important role to prevent the early teeth wear and to intercept at the earliest. REFERENCES 1.Barron MJ, McDonnell ST, Mackie I. Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. Orphanet Journal of rare Diseases 2008(3);31:1-10. 2.Subramaniam P, Mathew S, Sugnani SN. Dentinogenesis imperfecta: A case report. J Indian Soc Pedod Prev Dent 2008;26:85-7. 3.Bhandari S, Pannu K. Dentinogenesis imperfecta: A review and case report of a family over four generations. Indian J Dent Res 2008;19:357-61. 4.Rajendran R. Developmental disturbances of oral and paraoral structures. In: Rajendran R, Sivapathasundram B, editors. Shafers textbook of oral pathology. Elsevier; 2006. p. 75-7 5.Ramachandran S, Simon SS. Multidisciplinary management of a case of dentinogenesis imperfecta. Indian J Multidiscip Dent 2015;5:86-90. 6.Scully C. Oral and Maxillofacial Medicine: The Basics of Diagnosis and Treatment. 2nd ed. UK: Churchill Livingstone; 2008. p. 421-2. 7.Kumar V, Mitra R. A case report on dentinogenesis imperfecta. Int J Health Sci Res 2015;5(7):436-40. 8.Sapir S, Shapira J. Dentinogenesis imperfecta:an early treatment strategy. Paediatr Dent 2001;23:232-7. 9.Tahmassebi JF, Day PF, Toumba KJ, Andreadis GA. Paediatric dentistry in the new millennium :6. Dental anamolies in children. Dent Update 2003,30:534-40. 10.Kerebell B, Daculsi G, Menanteau J, Kerebel LM. Inorganic phase in dentinogenesis imperfecta. J Dent Res 1981;60:1655-60. 11.Wright JT, Gantt DG. Ultrastructure of dental tissues in dentinogenesis imperfecta in man. Arch Oral Biol 1985;30:201-6. 12.Kilpatrick NM, Welbury RR. Advanced Restorative Dentistry. In Paediatric Dentistry


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Edited by: Welbury RR, Duggal MS, Hosey MT. Oxford University Press; 2005:226-268. 13.Shields ED, Bixler D, El-Kafrawy AM. A proposed classification for heritable human dentine defect with a description of a new entity. Arch Oral Biol 1973;18:543-53. 14.Levin LS, Leaf SH, Jelmini RJ, Rose JJ, Rosenbaum KN. Dentinogenesis imperfecta in the Brandywine isolate: Clinical radiologic and scanning electron microscopic studies of the dentition. Oral Surg 1983;56:267-74. 15.Kinney H, Pople JA, Driessen CH, Breunig M, Marshall GW, Marshall SJ. Intrafibrillar mineral may be absent in dentinogenesis imperfecta type II. J Dent Res 2001;80:1555-9. How to cite this article: Giroh VR, Hebbale

M, Mhapuskar A, Thakare S. Dentinogenesis Imperfecta: A Case Report. Arch of Dent and Med Res 2016;2(5):42-46.

case report aodmr and paraoral structures. in: ... mitra r. a case report on dentinogenesis...

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