Table of Contents    
CASE REPORT
Year : 2015  |  Volume : 6  |  Issue : 2  |  Page : 468-471  

Karyotype, Pedigree and cone-beam computerized tomography analysis of a case of nonsyndromic pandental anomalies


1 Department of Conservative Dentistry and Endodontics, Dental College, RIMS, Imphal, Manipur, India
2 Department of Conservative Dentistry and Endodontics, Sinhgad Dental College and Hospital, Pune, Maharashtra, India
3 Department of Pedodontics and Preventive Dentistry, Dental College, RIMS, Imphal, Manipur, India
4 Department of Conservative Dentistry and Endodontics, Maulana Azad Institute of Dental Sciences, New Delhi, India
5 Department of Conservative Dentistry and Endodontics, SMBT Dental College, Sangamner, Maharashtra, India
6 Department of Orthodontics, Oral Health Science Centre, Postgraduate Institute of Education and Research, Chandigarh, India

Date of Web Publication6-Jul-2015

Correspondence Address:
Dr. Ganesh Ranganath Jadhav
Department of Conservative Dentistry and Endodontics, Sinhgad Dental College and Hospital, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0976-9668.160044

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   Abstract 

This case report presented a karyotype and pedigree analysis of a case with unusual combination of dental anomalies: Generalized short roots, talon cusps, dens invagination, low alveolar bone heights, very prominent cusp of carabelli and protostylid on first permanent molars, taurodontism of second permanent molars, rotated, missing and impacted teeth. None of the anomalies alone are rare. However, until date, nonsyndromic pandental anomalies that are affecting entire dentition with detailed karyotype, pedigree and cone-beam computerized tomography analysis have not been reported. The occurrence of these anomalies is probably incidental as the conditions are etiologically unrelated.

Keywords: Cone-beam computerized tomography, conventional karyotyping, dense invaginatus, short root anomalies, talon cusp, taurodontism


How to cite this article:
Dharmani U, Jadhav GR, Kaur Dharmani CK, Rajput A, Mittal P, Abraham S, Soni V. Karyotype, Pedigree and cone-beam computerized tomography analysis of a case of nonsyndromic pandental anomalies. J Nat Sc Biol Med 2015;6:468-71

How to cite this URL:
Dharmani U, Jadhav GR, Kaur Dharmani CK, Rajput A, Mittal P, Abraham S, Soni V. Karyotype, Pedigree and cone-beam computerized tomography analysis of a case of nonsyndromic pandental anomalies. J Nat Sc Biol Med [serial online] 2015 [cited 2021 Mar 8];6:468-71. Available from: http://www.jnsbm.org/text.asp?2015/6/2/468/160044


   Introduction Top


During tooth morphogenesis, hereditary disturbances or environmental factors are responsible for various dental anomalies. [1],[2] Usually, the occurrence of multiple dental anomalies in individuals or families is associated with other systemic manifestations or syndromes. [3] The commonly occurring dental anomalies are include talon cusp, tooth agenesis, transmigration, idiopathic generalized short root, microdontia, macrodontia, taurodontism, obliterated pulp chambers, dens invaginatus (DI), generalized enamel hypoplasia, root resorption, etc. To the authors best of knowledge, this was the first reported case that highlighted a karyotype, pedigree and cone-beam computerized tomography (CBCT) analysis of a case of nonsyndromic pandental anomalies that is affecting entire permanent dentition.


   Case Report Top


A 22-year-old boy reported to the department with the chief complaint of irregularly placed maxillary anterior teeth for which he wanted aesthetic correction. The medical history was noncontributory. Intraoral examination revealed irregular dentition, with generalized spacing, rotated and missing teeth in the upper arch. The permanent anterior teeth are larger in size with different crown morphology of posterior teeth, including prominent cusp of Carrabelle on maxillary first molars and protostylid on mandibular first molars [Figure 1]. The maxillary central incisors and canines (i.e., 11, 21.13, 23) showed the presence of talon cusp [Figure 1]. There was the presence of over-retained left lateral primary incisor while the right lateral incisor was missing [Figure 2]. Among the posteriors, the mesio-palatal cusps of maxillary molars and mesio-lingual cusps of mandibular molars were prominent. The patient exhibited angle's Class I molar relationship. Teeth present in the arch were as follows:
Figure 1: Intraoral photograph (a and b) and models of maxillary and mandibular teeth (c) multiple developmental dental anomalies like talons cusps involving 11, 13, 21, 22, 23 missing 12; over retained 62, prominent cusp of carabelli on maxillary fi rst molar and protostylid (P) on the mandibular first molars

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Figure 2: Intraoral photograph of maxillary and mandibular teeth (a) macrodontia (M) of all teeth. Panoramic radiograph (b) generalized short root anatomy, dense in dente with respect to 22, 24, 25, 34, 45 and congenital missing 12, 28, 38 with impacted immature 18, 48

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17, 16, 15, 14, 13, 11 … 21, 22, 52, 23, 24, 25, 26, 27.

47, 46, 45, 44, 43, 42, 41 … 31, 32, 33, 34, 35, 36, 37.

The preliminary radiographic examination of the patient (intraoral periapical radiographs and orthopantomogram) revealed tooth anomalies involving majority of teeth. Hence, a CBCT scan of mandibular and maxillary arches for proper diagnosis was advised. The detailed radiographic examination of all the results showed the following observations:

Congenitally missing teeth buds of 12, 28, 38.

Dens invaginatus seen in relation to 14, 22, 24, 34, 45.

Taurodontism seen in relation to 17, 27, 37, 47 [Figure 3].
Figure 3: Cone-beam computerized tomography parasagittal section shows the dense invaginatus involving 34, 45 and taurodontism in relation with 47 (a), 37 (b), 17(c), 27 (d)

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All teeth exhibited idiopathic short root anatomy [Figure 2].

External root resorption in relation to 11, 21, 22.

The appearance of developmental anomalies in a large number of teeth of the same patient made it mandatory to rule out any underlying systemic disorder. A detailed family history of the patient revealed that none of his other family members suffered from any similar dental complaints as shown in the pedigree chart [Figure 4]. The patient also reported that he did not have such complaint in the primary set of dentition. The patient was referred to the physician for a thorough medical history, physical examination and growth assessment. The physical examination gave no symptoms of any underlying systemic/genetic disorder. Further blood examination revealed normal blood profile. Endocrinological evaluation showed hormonal levels to be within normal limits. The patient was further underwent cephalometric analysis and conventional karyotyping to definitely rule out any underlying genetic disorder [Figure 5]. The results of these diagnostic aids were within normal limits and hence, final a diagnosis of nonsyndromic case of multiple developmental dental anomalies affecting entire dentition was made. As the patient was mainly concerned with upper anterior teeth, fixed prosthesis fabrication was done to restore aesthetics.
Figure 4: Pedigree of the family of the patient with Black Square indicating proband. Squares – males; circles – females

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Figure 5: Karyogram of the male chromosomal complement with no numerical or structural chromosomal anomalies detected (i.e., 46, XY)

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   Discussion Top


This article reported karyotype, pedigree and CBCT analysis of a case of nonsyndromic pandental anomalies that is affecting the entire dentition. The simultaneous occurrence of multiple abnormalities involving groups of teeth or the entire dentition is usually genetically determined and is associated with specific syndromes. Occurrence of pandental anomalies in a single patient without any other systemic abnormalities or familial tendency confirmed by karyotyping and pedigree analysis is certainly rare and possibly unique.

A talon cusp is an accessory crown structure composed of enamel, dentine and varying degree of pulp. More than 90% of such cases occur in the maxilla - both for the primary and permanent dentitions. This anomaly is usually unilateral, with only 20% cases having a bilateral occurrence. [4],[5],[6] Talon cusp can be accompanied by syndromes such as Berardinelli-Seip, Mohr, Rubinstein and Taybi, Ellisvan Creveld, Sturge-Weber and incontinentia pigmenti achromians. The co-occurrence of talon cusp and other dental anomalies such as dens in dente and tooth gemination has also been reported. [7] DI is resulting from invagination of the enamel organ into dental papilla. This anomaly was first reported by Ploquet in 1794. Socrates in 1856, first described dense in dente in human teeth. Tomes first described a case of coronal DI as early as 1859 and the prevalence rate varies from 0.04% to 10% in a permanent dentition. [8] Short root anomaly (SRA), described first by Lind in 1972, is defined as developmentally very short, blunt dental roots. Root that is equal to or shorter than the crown size is considered as SRA. This condition most commonly affects maxillary central incisors and premolars, and canines are rarely involved. [9]

Taurodontism is a developmental disturbance of a tooth that lacks constriction at the level of cementoenamel junction and is characterized by vertically elongated pulp chambers with apically displaced pulpal floor and bifurcated or trifurcated the roots. The prevalence of taurodontism is reported to range from 2.5% to 11.3% of the human population. [10] This condition has been reported in both primary and permanent teeth, with a prevalence of approximately <1% and 3-35%, respectively. The second and third molars show a high prevalence in a permanent dentition. It can be seen in a single or multiple teeth in the same patient, with unilateral or bilateral occurrence. Taurodontism has been reported either as an isolated anomaly or as a feature of multiple-system malformation syndromes such as Klinefelter syndrome, ectodermal dysplasia, tricho-dento-osseous syndrome, Down syndrome, and X-linked hypophosphatemic rickets. [11] Taurodontism has also been found in association with various dental anomalies, including amelogenesis imperfecta and hypodontia. Taurodontism, short roots, and external resorption have been described in patients with small head and short stature. However, it occurs in 2.5-3.5% of chromosomally normal Caucasians.

Patients suffering from such combination of multiple anomalies need to be differentially diagnosed against hypopararthyrodisim, pseudohypoparathyrodism, Vitamin D resistant ricket, hypophosphatasia, oculodentoosseous dysplasia, dystrophic epidermolysis bullosa, tricho-dento-osseous syndrome, dwarfism, russel silver syndrome, seckel syndrome, Williams syndrome. It can be ruled out by patient history, physical examination, blood examination, conventional and advanced radiographs, cephalometric analysis, pedigree analysis and conventional karyotyping. Conventional cytogenetics with karyotyping is useful in the diagnosis of chromosomal abnormalities related to developmental or mental retardation and dysmorphic features. In the presented case, no systemic abnormalities with any familial interrelation were seen. Hence, it was a diagnosed as a case of nonsyndromic occurrence of multiple dental anomalies and the wide variations in clinical manifestation of such nonsyndromic cases remain intriguing and are area of further research.


   Summary Top


This case report presented a karyotypic and pedigree analysis of a case with unusual combination of dental anomalies: Generalized short roots, talon cusps, DI, low alveolar bone heights, very prominent cusp of carabelli and protostylid on first permanent molars, taurodontism of second permanent molars. None of the anomalies alone are rare but they have not previously been reported together affecting entire dentition with detailed karyotypic, pedigrial and CBCT analysis. The occurrence of these anomalies is probably incidental as the conditions are etiologically unrelated.

 
   References Top

1.
Winter GB, Brook AH. Tooth abnormalities. In: A Companion to Dental Studies. Clinical Dentistry. Vol. 3. Oxford: Blackwell Scientific Publications; 1989. p. 55-45.  Back to cited text no. 1
    
2.
Nguyen AM, Tiffee JC, Arnold RM. Pyramidal molar roots and canine-like dental morphologic features in multiple family members: A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;82:411-6.  Back to cited text no. 2
    
3.
Mader CL. Talon cusp. J Am Dent Assoc 1981;103:244-6.  Back to cited text no. 3
    
4.
Lomçali G, Hazar S, Altinbulak H. Talon cusp: Report of five cases. Quintessence Int 1994;25:431-3.  Back to cited text no. 4
    
5.
Rayen R, Muthu MS, Sivakumar N. Aberrant talon cusps: Report of two cases. J Indian Soc Pedod Prev Dent 2006;24 Suppl 1:S7-10.  Back to cited text no. 5
    
6.
Solanki M, Patil SS, Baweja DK, Noorani H, Pk S. Talon cusps, macrodontia, and aberrant tooth morphology in Berardinelli-Seip syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;105:e41-7.  Back to cited text no. 6
    
7.
Segura JJ, Jiménez-Rubio A. Talon cusp affecting permanent maxillary lateral incisors in 2 family members. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:90-2.  Back to cited text no. 7
    
8.
Tavano SM, de Sousa SM, Bramante CM. Dens invaginatus in first mandibular premolar. Endod Dent Traumatol 1994;10:27-9.  Back to cited text no. 8
    
9.
Jafarzadeh H, Azarpazhooh A, Mayhall JT. Taurodontism: A review of the condition and endodontic treatment challenges. Int Endod J 2008;41:375-88.  Back to cited text no. 9
    
10.
Blumberg JE, Hylander WL, Goepp RA. Taurodontism: A biometric study. Am J Phys Anthropol 1971;34:243-55.  Back to cited text no. 10
    
11.
Hattab FN, Yassin OM, Sasa IS. Oral manifestations of Ellis-van Creveld syndrome: Report of two siblings with unusual dental anomalies. J Clin Pediatr Dent 1998;22:159-65.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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