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Table of Contents   
CASE REPORT  
Year : 2020  |  Volume : 31  |  Issue : 5  |  Page : 807-812
An unusual variation of radicular dentin dysplasia: A rare case report with review of literature


Department of Dentistry, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

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Date of Submission27-Apr-2019
Date of Decision27-May-2019
Date of Acceptance30-Jul-2019
Date of Web Publication08-Jan-2021
 

   Abstract 


Dentin dysplasia (DD) is a rare autosomal dominant disorder of dentin development, which is generally divided into two types based on the clinical and radiographic appearance of the affected dentinal tissues: Type-I (Radicular DD) and Type-II (Coronal DD). This paper reports the case of a 17-year-old female patient with both classical and atypical features of radicular DD in the permanent dentition. The present case shows clinically normal appearing crowns, localised mobility in the maxillary teeth, completely obliterated pulp chambers, widened root canals without any obliterations and the presence of multiple periapical radiolucencies. The clinical and radiographic findings observed in this present case report are different from those reported in the past literature, which suggests that the present case could be a variation of radicular DD.

Keywords: Dentin dysplasia, periapical radiolucencies, radicular dentin dysplasia

How to cite this article:
Patnana AK, Chugh VK, Chugh A. An unusual variation of radicular dentin dysplasia: A rare case report with review of literature. Indian J Dent Res 2020;31:807-12

How to cite this URL:
Patnana AK, Chugh VK, Chugh A. An unusual variation of radicular dentin dysplasia: A rare case report with review of literature. Indian J Dent Res [serial online] 2020 [cited 2021 Jan 18];31:807-12. Available from: https://www.ijdr.in/text.asp?2020/31/5/807/306449



   Introduction Top


Dentin dysplasia (DD) is an autosomal dominant hereditary disorder of dentin formation, observed one in every 100,000 individuals affecting both primary and permanent dentitions.[1] Rushton coined this term in 1939 and characterised it as abnormal dentine that contains an enormous number of spherical bodies.[2] Shields ED et al. proposed a classification that divided DD into two main classes based on the clinical and radiographic appearance of the affected dentinal tissues: Type-I 'DD' (DD1) and Type-II 'Anomalous dysplasia of dentin' (DD2). Over time, DD1 replaced by 'Radicular DD' and DD2 by 'Coronal DD', indicating the parts of the teeth that are primarily affected.[3] Radicular DD is clinically characterised by nearly normal appearing crowns with hypermobility of the teeth.[4] Regular findings of radicular DD include, delayed dental eruption pattern, opaque incisal borders and premature exfoliation because of the short, blunt, tapering or even absent roots.[5] The third type of DD or focal odontoblastic dysplasia has also been described, representing the radiographic findings of both the types of DDs.[4]

Radiographic examination is an important diagnostic tool for identifying radicular DD, which typically shows short, blunt and malformed roots. The other typical findings include the obliteration of pulp chambers with crescent-shaped pulpal remnants parallel to the cemento-enamel junction and several periapical radiolucencies involving the non-carious teeth.[5] Microscopic examination of affected teeth reveals a thin enamel layer with tubular secondary dentin, globular organisation and more centrally spherical structures suggesting multiple pulp calcifications. Since the mantle dentin is not affected, the teeth have an apparently normal clinical crown.[6]

Although the radicular DD was fairly divided into four types based on the radiographic findings by Carroll et al., the past literature presents different variations in the clinical and radiographic presentation of the radicular DD.[7] The variations are either regarding the number of teeth affected,[5] or variations in the type of pulp chamber calcifications or variable length of the roots.[6] In such a context of variable presentations of radicular DD, the present case report presents a rare variable of radicular DD, which shows the normal root development, atypically widened root canals and multiple periapical radiolucencies associated with clinically non-carious teeth.


   Case Presentation Top


A 17-year-old female patient came to the Department of Dentistry with the chief complaint of pus discharge in the chin region since 1 month and mobile teeth in the upper front teeth region for 2 weeks. No significant medical history was presented by the patient and she was visiting the dentist for the first time. The patient's mother gives a history of multiple extracted teeth 10 years before and was presently using the removable partial dentures. The extraoral examination showed a draining sinus in the submental region [Figure 1]. The intraoral examination showed decreased vestibular depth in the mandibular anterior teeth associated with clinically normal mandibular anterior teeth. The maxillary right and left second molars were grossly destroyed and presented grade-II mobility, maxillary left central incisor was grade-II mobile, root stumps were seen in relation to maxillary left first molar, maxillary right first premolar was fractured, right mandibular second molar was decayed, and mandibular left second molar was restored [Figure 2].
Figure 1: Extraoral sinus tract in the chin (pointed arrow marks show the sinus tract)

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Figure 2: Intraoral images: (a) Maxilla; (b) Mandible. (c) Teeth in occlusion

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   Investigations Top


The orthopantomogram (OPG) examination revealed multiple diffuse radiolucent areas associated with roots of maxillary and mandibular teeth [Figure 3]. The OPG also revealed the completely obliterated pulp chambers throughout the dentition. The root canals were widened with closed root apices in maxillary and mandibular, anterior and premolar teeth. The maxillary and mandibular molars showed completely fused roots with narrow constrictions at the apical region. The OPG also showed the shortened roots in relation to maxillary right second molar, root fracture in relation to maxillary left central incisor and it also confirms the missing mandibular left and right first molars. The patient's parents were also examined clinically and radiographically to determine the role of hereditary transmission to the child.
Figure 3: Orthopantomographic image of the patient

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The patient was examined further by a cone beam computed tomography (CBCT) scan performed on Carestream 9000 to evaluate the maxilla and mandible. Images were acquired 360° around the patient's head with a slice thickness of 0.2 mm. The acquired data was reconstructed into multiplanar reformation image to depict the maxilla and mandible; cross-sectional images were made starting from right to left. The CBCT interpretation had shown

  • Cervical root fracture in the maxillary left central incisor root with periapical radiolucency and perforation of the palatal cortex is visualised [Figure 4]a
  • Perforation of the inter-dental alveolar bone in the maxillary left lateral incisor and canine was observed [Figure 4]a
  • Periapical radiolucency in the maxillary right second premolar extends inferiorly up to the crest along the labial periradicular space [Figure 4]b
  • Fractured palate-coronal surface of the maxillary left first premolar with perforation of the buccal cortex [Figure 4]c
  • Multiple teeth in the mandibular region (31, 33, 34, 35, 37, 41, 44, 45, 47) reveal the evidence of internal resorption with periapical radiolucencies [Figure 5]a, [Figure 5]b, [Figure 5]c
  • Axial view showing multiple radiolucencies in mandible and maxilla [Figure 6]a and [Figure 6]b
  • Coronal view showing multiple radiolucencies in mandible and maxilla [Figure 7]a and [Figure 7]b.
Figure 4: Three dimensional images of the maxilla. (a) Anterior view. (b) Right-side view. (c) Left side view

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Figure 5: Three dimensional images of the mandible. (a) Anterior view. (b) Right-side view. (c) Left-side view

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Figure 6: Axial sections of patient CBCT: (a) Mandible; (b) Maxilla

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Figure 7: Coronal sections of patient CBCT: (a) Mandible; (b) Maxilla

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   Differential Diagnosis Top


The clinical examination of extraoral sinus discharge in the mandibular anterior teeth has led to the provisional diagnosis of periapical cyst of mandibular anterior teeth. However, no carious teeth were observed in the mandibular anterior region. The patient gives no history of trauma in the mandibular anterior teeth region and, thus, the periapical cysts associated with any non-vital teeth were ruled out.

The intraoral clinical examination showed normal teeth with no enamel deformities, which ruled out the enamel deformities. The presence of normal teeth with no deformities in the crown structure has led to the differential diagnosis of dentinogenesis imperfecta (DI), DD and regional odonto dysplasia (ROD). However, in DI, the teeth will be bluish-grey or yellow-brownish coloured, which was not observed in the present condition and, hence, DI was ruled out.

The radiographic investigations were advised to make the final diagnosis from the provisional diagnosis of DD or ROD. The OPG has shown multiple periapical radiolucencies in the mandibular and maxillary teeth. The multiple periapical radiolucencies will not be observed in the ROD and, hence, it was also ruled out. The type-II DD (Coronal DD) was ruled out based on the radiographic findings of the absence of enlarged pulp chambers and the presence of multiple periapical radiolucencies. The four types of type I DD (Radicular DD) were analysed further to make the final diagnosis. However, with the presence of completely calcified pulp chambers, completely developed root canals with closed apices, enlarged root canal spaces and the presence of calcifications parallel to the long axis of the tooth, a final diagnosis of variation of the radicular type of DD was made [Figure 8].
Figure 8: Flow diagram for arriving final diagnosis

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   Treatment Top


The absence of a specific treatment approach complicates the regular management of radicular DD. The early diagnosis and continuous follow-up of DD cases by the clinicians are of paramount importance.[8] The extraction of the teeth involving the periapical radiolucencies followed by prosthetic rehabilitation is also considered as a conventional treatment approach in completely obliterated canals.[5] However, endodontic procedures to treat the periapical radiolucencies were suggested in teeth with long roots and teeth with good prognosis.[2]

Considering the patient age and the adequate length of the roots, conventional root canal treatment was planned. The conventional root canal opening was done in the teeth associated with periapical radiolucencies (15, 14, 13, 12, 11, 21, 22, 24, 25, 37, 35, 34, 33, 32, 31, 41, 42, 43, 44, 45, 46), biomechanical preparation was done till F1 ProTaper files (Dentsply Tulsa Dental Specialties) and calcium hydroxide (Metapex, MetaBiomed) dressing was given as intracanal medicament. The obturation of the teeth was postponed until complete healing of the periapical lesions were observed. Extraction of maxillary second molars was done considering the gross decay and resorbed roots. The polyethylene fibre reinforced composite splinting was done from canine to canine in the maxillary anterior region to stabilise the coronal fragment of the maxillary left central incisor.


   Outcome and Follow-up Top


The extraoral draining sinus was healed 6 weeks postoperatively [Figure 9]. After the 12-month follow-up, there was a significant reduction in the periapical lesion sizes corresponding to all the teeth [Figure 10]. Although the root fracture in the maxillary left central incisor had not shown any favourable root healing after 6 months, the tooth was not extracted to maintain the normal bone level till the fixed prosthetic rehabilitation was made. The crown fracture was observed in the mandibular left central incisor at 12-month recall period.
Figure 9: Healed extraoral sinus after 6 weeks

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Figure 10: Post-operative OPG after 12-month follow-up

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   Discussion Top


The routine clinical examination in the present case has shown complete normal crowns with no generalised mobility in the maxillary and mandibular teeth. Radiographically, there was evidence of calcified pulp chambers with very minimal or no calcifications in the root canals. Shortened roots were observed only in the maxillary right second molar. Multiple periapical radiolucencies were observed in both maxillary and mandibular regions with no frank carious exposure of the teeth. The correlation of clinical and radiographic findings led the authors to suspect a variation from the regular subgroups of radicular DD, because the typical features like generalised short roots with complete obliteration and generalised mobility of the teeth were absent in the present case.

According to O Carroll et al.,[7] the permanent teeth in radicular DD could have any one of the four distinct radiographic findings: A. Complete obliteration of pulp chambers and no root development with many periapical radiolucent areas (DD1a); B. Horizontal, crescent-shaped, radiolucent pulpal remnants and few millimetres of root development with many periapical radiolucent areas (DD1b); C. Two horizontal, crescent-shaped, radiolucent lines and significant but incomplete root development, with or without periapical radiolucent areas (DD1c); D. Visible pulp chambers and oval pulp stones in the coronal third of the canal, with bulging of the root around the stone, and few, if any, periapical radiolucent areas (DD1d).[7] The present case showed multiple periapical radiolucencies and almost or completely developed roots with no obliteration of root canals, which does not fit into any of the subgroups suggested by O Carroll et al.[7]

The presence of short roots was observed only in maxillary right second molar and maxillary left central incisor; this atypical feature of short roots for only two teeth in the entire dentition was also considered as a variant from the regular types of radicular DD. The presence of localised radicular DD was observed in the case report of Rocha et al.,[5] where they observed that the radicular DD was localised to the left mandibular region affecting only the first and second premolars. Similarly, Kosinski RW et al.,[8] have also observed a case with localised radicular DD affecting only the mandibular right quadrant. However, the present case differs from the above-mentioned case reports, where the teeth were affected in the maxillary region rather than the mandibular region. These findings suggest that the present autosomal disorder of dentin may occur due to localised abnormalities in cell proliferation and function as suggested by Kosinski RW et al.[8]

This case presents a contradictory finding to the case report presented by Van Dis ML et al.,[9] where they observed that posterior teeth are more commonly affected than anterior teeth in radicular DD. In the present case, anterior teeth are equally affected as posterior teeth with the presence of multiple radiolucencies in maxillary and mandibular anterior regions. The presence of multiple periapical radiolucencies was another classical sign to diagnose the present case as a radicular DD.[1],[6],[7] The present case varies with the earlier case reports,[1],[6],[7] where the multiple periapical radiolucencies were associated with long root canals without any obliterations rather than the periapical radiolucencies associated with completely obliterated short root canals. However, the present case presents similar findings to the case reports by Van Dis ML et al.,[9] regarding atypical features of posterior teeth showing the fused roots with the mere absence of furcations in the maxillary and mandibular molar teeth [Figure 3].

The autosomal inheritance of the DD suggests the need of evaluating dental findings in the affected patient's family.[1] Hence, the routine dental check was done for the parents of the present case. The patient's mother gives a history of multiple extracted teeth because of mobile teeth 10 years before. The intraoral examination of the patient's mother had shown multiple missing teeth and grade-III mobile mandibular left lateral incisor and canine. The clinical findings were confirmed with the OPG examination [Figure 11]. The presence of similar history supports the autosomal inheritance of DD in the present case report.
Figure 11: Orthopantomographic image of the patient

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The periapical radiolucencies were significantly reduced after 12-month recall period by using calcium hydroxide in the present case. The healing of the periapical lesions by calcium hydroxide in the present case was in accordance with the literature available.[10] However, the complete resolution of the periapical lesions was not observed and, thus, the obturation of the root canals followed by the prosthetic rehabilitation was postponed.

Although numerous case series regarding radicular DD were presented in the literature, many clinical and theoretical questions were left unanswered in the diagnosis and treatment parts of radicular DD. Well-explained documentation of cases regarding radicular DD is required to establish relevant clinical and radiographic signs that will aid in the accurate diagnosis of radicular DD.


   Conclusion Top


The present rare case report showed classical findings of radicular DD like normal clinical crowns and multiple periapical radiolucencies associated with non-carious teeth accompanied by atypical features like long roots without any canal obliterations, which suggests an unusual variation of radicular DD.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Ansari G, Reid JS. Dentinal dysplasia type I: Review of the literature and report of a family. ASDC J Dent Child 1997;64:429-34.  Back to cited text no. 1
    
2.
Ravanshad S, Khayat A. Endodontic therapy on a dentition exhibiting multiple periapical radiolucencies associated with dentinal dysplasia type 1. Aust Endod J 2006;32:40-2.  Back to cited text no. 2
    
3.
Shields ED, Bixler D, El-Kafrawy AM. A proposed classification for heritable human dentine defects with a description of a new entity. Arch Oral Biol 1973;18:543-53.  Back to cited text no. 3
    
4.
Barron MJ, McDonnell ST, Mackie I, Dixon MJ. Hereditary dentine disorders: Dentinogenesis imperfecta and dentine dysplasia. Orphanet J Rare Dis 2008;3:31.  Back to cited text no. 4
    
5.
Rocha CT, Nelson-Filho P, Silva LA, Assed S, Queiroz AM. Variation of dentin dysplasia type I: Report of atypical findings in the permanent dentition. Braz Dent J 2011;22:74-8.  Back to cited text no. 5
    
6.
Neumann F, Würfel F, Mundt T. Dentin dysplasia type I-A case report. Ann Anat1999;181:138-40.  Back to cited text no. 6
    
7.
O Carroll MK, Duncan WK, Perkins TM. Dentin dysplasia: Review of the literature and a proposed sub classification based on radiographic findings. Oral Surg Oral Med Oral Pathol 1991;72:119-25.  Back to cited text no. 7
    
8.
Kosinski RW, Chaiyawat Y, Rosenberg L. Localized deficient root development associated with taurodontism: Case report. Pediatr Dent 1999;21:213-5.  Back to cited text no. 8
    
9.
Van Dis ML, Allen CM. Dentinal dysplasia type I: A report of four cases. Dentomaxillofac Radiol 1989;18:128-31.  Back to cited text no. 9
    
10.
De Souza-Filho FJ, Soares Ade J, Vianna ME, Zaia AA, Ferraz CC, Gomes BP. Antimicrobial effect and pH ofchlorhexidine gel and calcium hydroxide alone andassociated with other materials. Braz Dental J 2008;19:28-33.  Back to cited text no. 10
    

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Correspondence Address:
Dr. Arun K Patnana
Department of Dentistry, All India Institute of Medical Sciences, Jodhpur - 342005, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdr.IJDR_372_19

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

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    Abstract
   Introduction
   Case Presentation
   Investigations
    Differential Dia...
   Treatment
    Outcome and Foll...
   Discussion
   Conclusion
    References
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