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Case reports Annals and Essences of Dentistry Vol. IX Issue 3 Jul– Sep 2017 12 b 10.5368/aedj.2017.9.3.2.3 MULTIPLE DENS EVAGINATUS- A RARE CASE REPORT WITH A REVIEW OF LITERATURE 1 Cecile Priyanthi A 1 Post graduate Student 2 Vathsala Naik 2 Professor and Head 3 Amandeep Sodhi 3 Reader 4 Shriyanka R 4 Senior Lecturer 1-4 Department of Oral Medicine and Radiology, Bangalore Institute of Dental Science and Research Center Hosur Road, Bangalore-560029. ABSTRACT: Densevaginatus (DE) is a developmental disturbance affecting the shape of the teeth which is characterized by the presence of accessory enamel projections along with dentin and pulpal core on the occlusal or lingual surface of the teeth. It affects both the primary and permanent dentition. The clinical intendment is focused on early pulpal exposure due to occlusal trauma, fracture or attrition leading to infection. Treatment may be conservative or interventional. The occurrence of DE either isolated or conjoined with other dental anomalies has been reported. This article presents with a case report of conjoined occurrence of multiple DE (Schulge type 5) along with partial anodontia of maxillary premolars and left molars and also reviews the etiology, classification, diagnosis and management strategies of the teeth with DE. KEYWORDS: Dens evaginatus, DE, hamartomatous, evaginated odontome INTRODUCTION The disturbances in the early stage of teeth development will lead to alteration in the shape of the teeth resulting in anomalies. The dens evaginatus is one such developmental anomaly of teeth that presentson the occlusal surface or cingulum area of teeth as accessory cusps or enamel globules 1 and is composed of enamel, dentin core usually enclosing the pulpal tissue. 2 It was first described by Mitchell in 1892 but the terminology was recommended by Oehler.It may affect both the primary and permanent dentitions either unilaterally or bilaterally 3 and may be localized or generalized.According to literature review, the prevalence of these cusp like projections are more in molars as cusp of Carabelli (68%) followed by Leong’s premolars (8%) and the Talon’s cusp of incisors (1-7.7%). 4 The occurrence may be isolated or concomitant with other dental anomalies such as supernumerary teeth, anodontia, Dens invaginatus, etc 3 Partial anodontia or agenesis of teeth is the congenital absence of one or more teeth. According to literature reviews, most commonly missing teeth are the third molars followed by maxillary lateral incisors and the second premolars, often bilaterally. 1 Both agenesis of teeth and Dens evaginatus can be diagnosed by clinical and radiographic appearances. Very few cases of multiple dens evaginatus have been reported in the literature so far. This case report of multiple dens evaginatus involving maxillary and mandibular premolars, molars and canine along with partial anodontia of maxillary premolars therefore comes up as a unique and rare addition to the literature archive. CASE REPORT: A 23-year-old male patient reported to the outpatient department of Oral medicine and Radiology with the chief complaint of missing teeth in the left upper back teeth region. Patient presented with the history of missing left upper back teeth since the exfoliation of his primary teeth. The medical and the family history of the patient were found to be noncontributory. General physical and extra oral examinations did not show any abnormality except for the presence of mild facial asymmetry in the form of a depression on the left upper third of his face near the malar region. Intraoral examination revealed mild gingivitis with the other soft tissues appearing normal in color, contour and consistency. An intraoral hard tissue examination revealedabsence of maxillary left first premolar, second molar and maxillary second premolars bilaterally. There was a tapered maxillary arch with reduced over jet, incisors showing edge-to-edge biteand posterior crossbite ((Fig.1, Fig.2 and Fig.3) present. There was a physiological drifting of maxillary left canine distally and molar mesially. The molars presented with multiple accessory cusps on their occlusal surface, showing attrition and the premolars presented with Leong’s tubercle (Fig.1 and Fig.2), which resulted in interference in occlusion leading to a premature contact with their antagonists. The maxillary and mandibular incisors were shovel-shaped with prominent lateral margins which encloses the lingual/palatal surface. However, none of the teeth were symptomatic in terms of sensitivity.

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Page 1: Case reports Annals and Essences of Dentistry...Case reports Annals and Essences of Dentistry Vol. IX Issue 3 Jul– Sep 2017 15 b 1 4, FGF-9), transforming growth factor β, and bone

Case reports Annals and Essences of Dentistry

Vol. IX Issue 3 Jul– Sep 2017 12 b

10.5368/aedj.2017.9.3.2.3

MULTIPLE DENS EVAGINATUS- A RARE CASE REPORT WITH A REVIEW OF

LITERATURE

1 Cecile Priyanthi A

1 Post graduate Student

2 Vathsala Naik 2 Professor and Head

3 Amandeep Sodhi 3 Reader

4 Shriyanka R 4Senior Lecturer

1-4 Department of Oral Medicine and Radiology, Bangalore Institute of Dental Science and Research Center Hosur Road,

Bangalore-560029.

ABSTRACT: Densevaginatus (DE) is a developmental disturbance affecting the shape of the teeth which is characterized by the presence of accessory enamel projections along with dentin and pulpal core on the occlusal or lingual surface of the teeth. It affects both the primary and permanent dentition. The clinical intendment is focused on early pulpal exposure due to occlusal trauma, fracture or attrition leading to infection. Treatment may be conservative or interventional. The occurrence of DE either isolated or conjoined with other dental anomalies has been reported. This article presents with a case report of conjoined occurrence of multiple DE (Schulge type 5) along with partial anodontia of maxillary premolars and left molars and also reviews the etiology, classification, diagnosis and management strategies of the teeth with DE. KEYWORDS:Dens evaginatus, DE, hamartomatous, evaginated odontome

INTRODUCTION

The disturbances in the early stage of teeth

development will lead to alteration in the shape of the

teeth resulting in anomalies. The dens evaginatus is one

such developmental anomaly of teeth that presentson the

occlusal surface or cingulum area of teeth as accessory

cusps or enamel globules1and is composed of enamel,

dentin core usually enclosing the pulpal tissue.2

It was first

described by Mitchell in 1892 but the terminology was

recommended by Oehler.It may affect both the primary

and permanent dentitions either unilaterally or bilaterally3

and may be localized or generalized.According to

literature review, the prevalence of these cusp like

projections are more in molars as cusp of Carabelli (68%)

followed by Leong’s premolars (8%) and the Talon’s cusp

of incisors (1-7.7%).4 The occurrence may be isolated or

concomitant with other dental anomalies such as

supernumerary teeth, anodontia, Dens invaginatus, etc3

Partial anodontia or agenesis of teeth is the congenital

absence of one or more teeth. According to literature

reviews, most commonly missing teeth are the third

molars followed by maxillary lateral incisors and the

second premolars, often bilaterally.1

Both agenesis of teeth and Dens evaginatus can be

diagnosed by clinical and radiographic appearances. Very

few cases of multiple dens evaginatus have been reported

in the literature so far. This case report of multiple dens

evaginatus involving maxillary and mandibular premolars,

molars and canine along with partial anodontia of maxillary

premolars therefore comes up as a unique and rare

addition to the literature archive.

CASE REPORT:

A 23-year-old male patient reported to the outpatient

department of Oral medicine and Radiology with the chief

complaint of missing teeth in the left upper back teeth

region. Patient presented with the history of missing left

upper back teeth since the exfoliation of his primary teeth.

The medical and the family history of the patient were

found to be noncontributory. General physical and extra

oral examinations did not show any abnormality except for

the presence of mild facial asymmetry in the form of a

depression on the left upper third of his face near the

malar region.

Intraoral examination revealed mild gingivitis with the

other soft tissues appearing normal in color, contour and

consistency. An intraoral hard tissue examination

revealedabsence of maxillary left first premolar, second

molar and maxillary second premolars bilaterally. There

was a tapered maxillary arch with reduced over jet,

incisors showing edge-to-edge biteand posterior crossbite

((Fig.1, Fig.2 and Fig.3) present. There was a

physiological drifting of maxillary left canine distally and

molar mesially. The molars presented with multiple

accessory cusps on their occlusal surface, showing

attrition and the premolars presented with Leong’s

tubercle (Fig.1 and Fig.2), which resulted in interference in

occlusion leading to a premature contact with their

antagonists. The maxillary and mandibular incisors were

shovel-shaped with prominent lateral margins which

encloses the lingual/palatal surface. However, none of the

teeth were symptomatic in terms of sensitivity.

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Case reports Annals and Essences of Dentistry

Vol. IX Issue 3 Jul– Sep 2017 13 b

Fig.1: Maxillary arch exhibiting multiple Dens Evaginatus

Fig.2: Mandibular arch exhibiting multiple Dens Evaginatus

Fig.3. Occlusal status of the patient - Right and Left occlusal views

Fig.4: Intraoral Periapical radiograph showing multiple Dens evaginatus

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Case reports Annals and Essences of Dentistry

Vol. IX Issue 3 Jul– Sep 2017 14 b

1

Fig.5: Panoramic radiograph showing multiple Dens evaginatus and partial anodontia

The patient was advised for radiological investigations 2

which included a set of full mouth Intra Oral Periapical 3

Radiographs (Fig.4.) followed by extraoral radiographs 4

like Panoramic (OPG) and Postero-Anterior skull (PA) 5

view. The OPG (Fig.5) revealed absence of maxillary 6

second premolar, left first premolar, second and third 7

molars and mandibular third molars. In addition to this, 8

there was presence of tuberculate premolars and multiple 9

extra cuspal projections on the molars. 10

11

Correlating the clinical and radio graphical features, the 12

condition was diagnosed as Densevaginatus of 13

posterior teeth- Schulge type 5with shovel-shaped 14

incisors and partial anodontia involving the maxillary arch. 15

16

The patient was advised for prosthetic rehabilitation of 17

his missing teeth andfor interventional treatments such as 18

occlusal correction and preventive restorations. Since the 19

patient was not concerned about esthetics and occlusal 20

discomfort he was not willing for any interventional 21

treatment however, he has been kept on regular follow 22

ups. 23

24

DISCUSSION: 25

26

Dens evaginatus is the cuspal projection of enamel in 27

the central groove or lingual ridge of the buccal cusp of 28

permanentpremolar or molar teeth. It is also called as 29

evaginated odontome,1,5

occlusal enamel pearl1, the 30

central tubercle or supernumerary occlusal cusp. It is most 31

common in mandibular premolars called as Leong’s 32

premolar, occurring bilaterally although seen as accessory 33

cusps on the occlusal surface molars.It is also seen in 34

anterior teeth on the lingual aspect extending from the 35

cementoenamel junction up to the incisal edge and called 36

as Talon’s cusp.6

Currently Dens evaginatus is used as a 37

generalized terminology to describe the talon’s cusp, 38

Leong’s premolar or occlusal enamel pearl and was first 39

recommended by Oehlers7 in 1967.It is considered as a 40

hamartomatous growth of odontogenic apparatus8 41

comprising of enamel with a dentin core and mostly with 42

pulpal extension. Dens evaginatus varies widely in shape 43

(horn-like, conical or pyramidal), size, structure, location, 44

and site of origin.It can occur bilaterally and affects both 45

primary and permanent dentition.3. It is more common 46

among mongoloid race, about 15% Asians and rare 47

among whites.8

48

49

DE occurs in the early stages (bell stage) of tooth 50

development before the mineralization of hard tissues. It 51

results from the outward folding of the inner enamel 52

epithelial layer into the stellate reticulum of the enamel 53

organ and transient focal hyperplasia of the primitive 54

pulpal mesenchyme during the morphodifferentiation 55

stage of the tooth development.1The etiology of dens 56

evaginatus is still unknown, even though various 57

hypotheses have been put forward.3 Both environmental 58

and genetic factors are said to be involved in the 59

etiological process. The genetic factors may be due to 60

mutations in various genes like human EDA1 61

(Ectodysplasin A), EDAR (Ectodysplasin A receptor), and 62

EDARADD (Ectodysplasin A receptor associated death 63

domain) genes that leads to altered tooth 64

morphology.9The enamel knots which are the central 65

regulators of teeth development, due to accumulation of 66

certain molecules such as fibroblast growth factors (FGF-67

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Case reports Annals and Essences of Dentistry

Vol. IX Issue 3 Jul– Sep 2017 15 b

4, FGF-9), transforming growth factor β, and bone 1

morphogenic proteins (BMP-2, 4,7) may also lead to 2

formation of accessory cusps.10

3

4

According to literature reviews, shovel-shaped 5

incisors,5,11

supernumerary teeth,11

agenesis of teeth11

, 6

impacted teeth and odontomas3are the other common 7

anomalies of teeth seen most frequently in association 8

with dens evaginatus.Among these, the features which 9

were present in our case as highlighted above includes 10

shovel-shaped incisors and agenesis of teeth. The shovel-11

shaped incisorspresent with a thickened marginal ridge 12

enclosing the central groove in the lingual surface 13

resembling the scoop of shovel.11

Partial anodontia or 14

agenesis of teeth is the congenital absence of one or more 15

teeth. Most common missing teeth are the third molars 16

followed by maxillary lateral incisors and the second 17

premolars, often bilaterally.12

In this case maxillary second 18

premolar, left first premolar, second and third molars and 19

mandibular third molars were missing thus correlating with 20

the literature. 21

22

The bulk of the dens evaginatus can cause occlusal 23

interference causing traumatic occlusion and may cause 24

pain in the periodontal ligament secondary to the occlusal 25

trauma and also temporomandibular joint pain. They have 26

deep developmental grooves sometimes extending into 27

the pulp thereby leading to accumulation of plaque and 28

early progression to dental caries.13

In the present case 29

however, none of the teeth showed dental caries. 30

31

Even though DE is not essential component of any 32

specific syndrome, it is seen in association with 33

Rubinstein-Taybi Syndrome,14

Mohr Syndrome (oral-34

facial-digital II Syndrome),15

Sturge-Weber Syndrome 35

(encephalo-trigeminal angiomatosis),16

and Ellis-van-36

Creveld Syndrome.17

37

38

Based on the location of the accessory cuspal tubercles 39

on the posterior teeth, Schulge in 1987 classified DE into 5 40

types: 41

42

1. A cone-like enlargement of the lingual cusp 43

2. A tubercle on the inclined plane of the lingual 44

cusp 45

3. A cone-like enlargement of the buccal cusp 46

4. A tubercle on the inclined plane of the buccal 47

cusp 48

5. A tubercle arising from the occlusal surface 49

obliterating the central groove. 50

51

Based on the anatomical shape of the tubercle Lau 52

classified as smooth, grooved, terraced and 53

ridge.However, Oehler classified DE based on the 54

histological appearance of the pulp content within the 55

tubercle as wide pulp horns (34%), narrow pulp horns 56

(22%), constricted pulp horns (14%), isolated pulp horn 57

remnants (20%), no pulp horn (10%).3 Schulge type 5 was 58

observed in the above discussed case. 59

60

Radiographically, DE will be visualized as radiopaque 61

projections on occlusal surface with differing radio dense 62

structures of enamel, dentin along thin slender radiolucent 63

pulpal component. The IOPARs and OPG aided in the 64

visualization of the above features for the present case. 65

66

Clinical management of these dental anomalies varies 67

from patient to patient presentation. Interventional 68

treatment modalities such coronoplasty, occlusal 69

corrections, application of desensitizing fluorides18

and 70

prosthetic rehabilitation can be done. When the affected 71

teeth are fractured or attrited causing pulpal exposure, 72

endodontic treatment methods should be followed. 73

74

CONCLUSION: 75

76

Early diagnoses and management of dental 77

anomalies are very important for the functional and 78

esthetic considerations of the patients specially in rare 79

conditions as highlighted in this case report. Systemic 80

involvement should be ruled out. Multidisciplinary 81

treatment protocol along with regular follows ups should 82

be done. 83

84

REFERENCES: 85

86

1. Rajendran R. Shafer's textbook of oral pathology. 87

Elsevier India; 2009. 88

2. Sharma G, Mutneja AR, Nagpal A, Mutneja P. Dens 89

evaginatus and dens invaginatus in a double tooth: A 90

rare case report. Indian Journal of Dental Research. 91

2015 Sep 1;26(5):545. 92 3. Al-Ajlouni OM, Sharanesha RB. DENS 93

EVAGINATUS: etiology, classification, prevalence, 94

diagnosis, associations and treatment considerations. 95 A review. 96

4. Cruz CV, Soares AL, Braga DN, Costa MC. Diagnosis 97

and Surgical Management of Nonsyndromic Nine 98

Supernumerary Teeth and Leong’s Tubercle. Case 99

reports in dentistry. 2016 Mar 15;2016. 100 5. Ayer A, Vikram M, Suwal P. Dens Evaginatus: A 101

Problem-Based Approach. Case reports in dentistry. 102

2015 Dec 8;2015. 103

6. Neville BW, Damm DD, Chi AC, Allen CM. Oral and 104

maxillofacial pathology. Elsevier Health Sciences; 105 2015. 106

7. Oehlers FA, Lee KW, Lee EC. Dens evaginatus 107 (evaginated odontome). Its structure and responses 108

to external stimuli. The Dental practitioner and dental 109 record. 1967 Mar;17(7):239-44. 110

8. Levitan ME, Himel VT. Dens evaginatus: literature 111

review, pathophysiology, and comprehensive 112 treatment regimen. Journal of endodontics. 2006 Jan 113

31;32(1):1-9. 114

I. Thesleff and P. Sharpe, “Signalling networks 115 regulating dental development,” Mechanisms of 116 Development, vol. 67, no.2, pp. 111–123, 1997. 117

9. Hegde S, Jain M, Shubha AB. A Rare Bilateral 118

Presentation of Multiple Dens Invaginatus, Shovel-119 Shaped Incisor and Talon Cusp With Mesiodens. 120

Kathmandu University Medical Journal. 2015 Oct 121

19;12(4):292-5. 122

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Case reports Annals and Essences of Dentistry

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10. Mellor JK, Ripa LW: Talon cusp: a clinically significant 1

anomaly. Oral Surg Oral Pathol Oral Med, 29: 225-2

228, 1970. 3

11. Hattab FN, Yassin OM, Al-Nimri KS. Talon cusp in 4

permanent dentition associated with other dental 5

anomalies: review of literature and reports of seven 6

cases. ASDC journal of dentistry for children. 7 1996;63(5):368. 8

12. Viswanathan S, Nagaraj V, Adimoulame S, Kumar S, 9

Khemaria G. Dens evaginatus in proximal surface of 10

mandibular premolar: A rare presentation. Case 11

reports in dentistry. 2012 Sep 13;2012. 12

13. Gardner DG, Girgis SS: Talon cusp: a dental anomaly 13

in the Rubinstein-Taybi syndrome. Oral Surg Oral 14

Med Oral Pathol, 47: 519-521, 1979. 15

14. Goldstein E, Medina JL: Mohr syndrome or oral-facial-16

digital II: report of two cases. J Am Dent Assoc, 89: 17

377-382, 1974. 18

15. Chen RJ, Chen HS: Talon cusp in primary in primary 19

dentition. Oral Med Oral Surg Oral Pathol, 62: 67-72, 20

1986 21

16. Hattab FN, Yassin OM, Sassa IS: Oral manifestations 22

of Ellis-van Creveld syndrome (Chondroectodermal 23

Dysplasia): Report of two siblings with unusual dental 24

anomalies. J Clin Pediatr Dent, Winter, 22(2): 159-25

163, 1998. 26

17. Shekhar MG, Vijaykumar S, Tenny J, Ravi GR. 27 Conservative Management of Dens Evaginatus: 28

Report of Two Unusual Cases. International Journal 29

of Clinical Pediatric Dentistry. 2010 May;3(2):121. 30

31

Corresponding Author 32

33

Dr.Cecile Priyanthi A Post graduate student,

Department of Oral Medicine & Radiology, Bangalore Institute of Dental Sciences,

Bangalore E-mail : [email protected]

Phone No: +91 9986393123

34

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