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Year : 2016  |  Volume : 5  |  Issue : 4  |  Page : 299-302

Dentin dysplasia: A rare case report

1 Department of Oral and Maxillofacial Surgery, Tamil Nadu Government Dental College and Hospital, Tamil Nadu, India
2 Department of Oral Pathology and Microbiology, Priyadharshini Dental College and Hospital, Chennai, Tamil Nadu, India
3 Department of Oral Pathology and Microbiology, Tamil Nadu Government Dental College and Hospital, Tamil Nadu, India

Date of Web Publication23-Dec-2016

Correspondence Address:
Arun Kumar Kamalakaran
Department of Oral and Maxillofacial Surgery, Tamil Nadu Government Dental College and Hospital, Chennai - 600 003, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2277-8632.196594

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Dentin dysplasia (DD) is an autosomal dominant disorder affecting both the deciduous and permanent dentitions. The affected individual has normal size, shape, and color of crown, and pattern of eruption but abnormally short and stunted roots leading to premature exfoliation. DD is classified into two types: DD type I - radicular DD and DD type 2 - coronal DD. We present a case of DD type Ia in a 12-year-old boy, highlighting the clinical, radiographic, and histopathologic features with a brief note on treatment and preventive care.

Keywords: Dentin dysplasia (DD), developmental, tooth

How to cite this article:
Kamalakaran AK, Rajeswari MR, Bharathi R, Parthiban N. Dentin dysplasia: A rare case report. J NTR Univ Health Sci 2016;5:299-302

How to cite this URL:
Kamalakaran AK, Rajeswari MR, Bharathi R, Parthiban N. Dentin dysplasia: A rare case report. J NTR Univ Health Sci [serial online] 2016 [cited 2022 Jan 17];5:299-302. Available from: https://www.jdrntruhs.org/text.asp?2016/5/4/299/196594

  Introduction Top

Dentin is a specialized mineralized connective tissue that makes the bulk of the teeth. It serves as the protective covering for the pulp and as a support for the overlying enamel and cementum. One of the rare genetic developmental defects affecting the dentin is dentin dysplasia (DD). It is an autosomal dominant disorder affecting 1 in 100,000. [1] The etiopathogenesis is unknown. The primary and the permanent dentitions are affected.

This condition was first described by Ballschmiede in 1920. [2] Rhuston in 1939 [3] named this condition as DD. Witkop [4] classified DD in 1972 as DD type I - radicular DD and DD type 2 - coronal DD. In type I, both the dentitions are affected in a similar fashion. The affected individual has normal size, shape, and color of crown, and pattern of eruption but has progressive mobility and premature exfoliation because of abnormally short and stunted roots. Also, this may be presenting with multiple pulp and periapical abscesses or cysts.

This article presents a case of DD type Ia in 12-year-old boy. It highlights the clinical, radiographic, and histopathologic variations, along with early intervention and preventive care of this developmental anomaly.


A 12-year-old boy visited a private dental clinic with complaints of pain and swelling of the upper front tooth region with mobility of the upper front tooth for the past 2 days. Past dental history revealed extraction of a deciduous molar for mobility 2 years back. Past medical history was irrelevant. Oral examination showed the presence of all permanent teeth with 22 missing.

The morphology and color of the crowns of all teeth were normal [Figure 1]. All teeth appeared to be a little tilted or rotated with grade I mobility. The maxillary central incisors were extremely mobile with pain and tenderness; two small pointing abscesses of the palatal gingiva were also noted. There was no discoloration or caries. Past dental history revealed spontaneous exfoliation of all the primary teeth expect one which was extracted.
Figure 1: Maxillary and mandibular teeth with normal crown morphology, with missing 22 and tilting of teeth

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Orthopantomogram (OPG) revealed all the teeth were without pulp chamber and root [Figure 2]. The crown appeared tilted with spacing and widening of the periodontal ligament space in 11 and 21. Small periapical radiolucencies in relation to all mandibular anteriors were noted. Complete clinical and radiographic examinations of the patients' parents and sisters were normal.
Figure 2: OPG showing teeth without pulp chamber and root

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The case was provisionally diagnosed as DD type Ia and the patient was treated with drainage of pus, short-term antibiotic therapy, dietary supplements, vitamins, calcium, and follow-up. The central incisors were exfoliated during follow-up. The central incisor was subjected to histopathological examinations.

The ground sections showed the enamel with normal thickness and enamel rods. The superficial dentin showed normal dentinal tubules. The pulp chamber was completely obliterated by some atubular dentin-like material, which was seen in the form of fused globules. The formation of atubular dentin around the obstacles gave the characteristic lava flowing around boulders appearance [Figure 3] and [Figure 4].
Figure 3: Ground section 100X showing lava flowing over boulder appearance under transmitted light

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Figure 4: Ground section 100X showing lava flowing over boulder appearance under polarized light. The case was diagnosed as dentin dysplasia type Ia. The case is under follow-up

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The case was diagnosed as DD type Ia. The case is under follow-up.

  Discussion Top

DD is a rare genetic, structural developmental disorder of the dentin with unknown etiopathogenesis.

Logan et al. proposed that the dental papilla was responsible for the defective root development and pulp obliteration. They suggested that multiple degenerative foci within the papilla become calcified, leading to reduced growth and obliteration of pulp space. [5]

Witkop suggested that breakage of Hertwig's epithelial root sheath and migration of these cells into the dental papilla will induce undifferentiated mesenchymal cells of the dental papilla to differentiate into odontoblasts and result in dentin formation. [6]

Sarik et al. suggested that DD I occurs because of the defect in the epithelial component of developing tooth germ. The authors postulated that the invagination of root sheath occurs too soon, followed by a sequence of unsuccessful attempts to correct itself, resulting in a stunted root form and unusual whorl-like pattern of the dentin obliterating the pulp chamber. [7]

Wesley et al. disagreed and said that DD I results from inaccurate interaction of the odontoblast with ameloblast layer, resulting in the abnormal differentiation and/or function of these odontoblasts. [8]

Melnick et al. also supported this since the coronal dentin was normal, early induction of odontoblasts by the ameloblasts was undisturbed and abnormal induction happened when development proceeded in the cervical region. [9]

Carroll et al. classified DD I into four distinct forms according to the severity measured by the shape of the pulp chambers, and size of roots on radiographs. [10]

DD Ia, which is the most severe form, shows total pulp obliteration with little or no root formation. It has the greatest prevalence of periapical lesion, pulpal pathosis, and early tooth exfoliation.

DD Ib is a thin, horizontal, crescent-shaped pulpal remnant at the level of the cementoenamel junction (CEJ) with very short, conical roots in the permanent teeth and multiple periapical radioluncies in the radiographs.

DD Ic shows two horizontal crescent-shaped radiolucent lines being concave toward each other at the level of CEJ with significant but incomplete root formation. Usually, there is no evidence of the pulp canal in the roots. Periapical radioluncencies may or may not be seen on radiographs.

DD Id is the least severe form and is characterized by a radiographically visible pulp chambers, oval pulp stones in the coronal one third of the root canal. The root is of normal length but is usually bulbous around the stone with few, if any, periapical radiolucencies.

The authors also highlighted that the four subgroups might overlap and that DD I occured at different stages of tooth development; the earlier it occurs, the more severe is the pulpal obliteration and stunting of roots.

DD I has to be differentiated from DD II, dentiogenesis imperfecta and regional odontodysplasia. Our case clearly belongs to the category of DD Ia because there were clinically normal crowns, abnormally stunted roots, and a completely obliterated pulp chamber in the radiographs. Also periapical radiolucency was seen in all the mandibular anterior teeth.

Though malocclusion has not been suggested in DD I, our case showed severe malocclusion with rotated, tilted, and spaced crowns. Few cases of malocclusion have been reported. DD is an autosomal dominant condition but in this case, there is no family history of such a disease and so the patient may be a first-generation sufferer.

The periapical radiolucencies in this patient could be periapical granuloma rather than a cyst. Failure of eruption of 22 could be because of abnormal tilting of the crown.

The management of patients of DD presents a challenge to dentists. Extraction is the suggested treatment for extremely mobile teeth and teeth with periapical radiolucencies, as endodontic treatments are contraindicated in teeth with pulpal obliteration. Orthodontic treatments are suggested, which may lead to further root resorption, mobility, and premature exfoliation. However, regular follow-up and routine conservative treatment are the choice of treatment.


DD Ia is a rare defect of the dentin, which leads to premature exfoliation of primary and permanent teeth. The suggested etiopathogenesis was reviewed none remains conclusive. Treatment in young patients is symptomatic and preventive. Preventive treatments are aimed at retaining the teeth for a longer period in the mouth with maintenance of proper oral care. Early diagnosis of this condition by the dentist helps in taking measures for prolonged retention of teeth and avoiding development of cyst.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Kim JW, Simmer JP. Hereditary dentin defects. J Dent Res 2007;86:392-9.  Back to cited text no. 1
Ballschmiede G. Dissertation. In: Herbst E, Apffelstaedt M, editors. Malformation of Jaws and Teeth. Berlin, New York: Oxford University Press; 1920. p. 286, 930.  Back to cited text no. 2
Rhuston MA. A case of dentin dysplasia. Guys Hospital Report 1919;89:369-73.  Back to cited text no. 3
Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North Am 1975;19:24-45.  Back to cited text no. 4
Logan J, Becks H, Silverman S Jr, Pindborg JJ. Dentinal dysplasia. Oral Surg Oral Med Oral Pathol 1962;15:317-33.  Back to cited text no. 5
Wiktop CJ Jr. Amelogenesis imperfecta, Dentiogenesis imperfecta and dentin dysplasia revisited: Problems in classification. J Oral Pathol 1988;17:547-53.  Back to cited text no. 6
Sauk JJ Jr, Lyons HW, Trowbridge HO, Wiktop CJ Jr. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Oral Surg Oral Med Oral Pathol 1972;33:763-71.  Back to cited text no. 7
Wesley RK, Wyosocki GP, Minty SM, Jackson J. Dentin dysplasia type I: Clinical, morphologic and genetic studies of a case. Oral Surg Oral Med Oral Pathol 1976;41:516-24.  Back to cited text no. 8
Melnick M, Levin LS, Brady J. Dentin Dysplasia type I: A scanning electron microscopic analysis of primary dentition. Oral Surg Oral Med Oral Pathol 1980;50:335-40.  Back to cited text no. 9
O'Carroll MK, Duncan WK. Dentin dysplasia type I. Radiologic and genetic perspective in a six-generation family. Oral Surg Oral Med Oral Pathol 1994;78:375-81.  Back to cited text no. 10


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


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