Archives of Medicine and Health Sciences

: 2022  |  Volume : 10  |  Issue : 1  |  Page : 84--86

A case of isolated wide pulp chambers in second premolars along with enamel hypoplasia - Dentinogenesis imperfecta - A diagnostic dilemma

Vela D Desai 
 Department of Oral Medicine and Radiology, Jaipur Dental College, MVGU, Jaipur, Rajasthan, India

Correspondence Address:
Dr. Vela D Desai
Department of Oral Medicine and Radiology, Jaipur Dental College, Maharaj Vinayak Global University, Jaipur 302038, Rajasthan


Dentinogenesis Imperfecta is a rare hereditary dentin developmental disorder that affects both primary and permanent dentition. It is characterized by discolored and translucent teeth ranging from gray to brownish-blue/amber. The enamel may split readily from the dentin when subjected to occlusal stress. Radiographically, there is evidence of cervical construction, short root, and pulp chambers, whereas the root canals are smaller than normal or completely obliterated. Here, the author presents the case of a 28-year-old male with generalized enamel hypoplasia and isolated distinctly translucent second premolars in three quadrants along with root changes. Unlike the classical representation of generalized involvement of the teeth, this case differs and needs to be documented. A thorough history, careful clinical, and radiographic observation are the key to early diagnosis and management of such a rare entity.

How to cite this article:
Desai VD. A case of isolated wide pulp chambers in second premolars along with enamel hypoplasia - Dentinogenesis imperfecta - A diagnostic dilemma.Arch Med Health Sci 2022;10:84-86

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Desai VD. A case of isolated wide pulp chambers in second premolars along with enamel hypoplasia - Dentinogenesis imperfecta - A diagnostic dilemma. Arch Med Health Sci [serial online] 2022 [cited 2022 Aug 19 ];10:84-86
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Full Text


Dentinogenesis imperfecta (DI) is a developmental disorder involving dentin.[1] It was probably first recognized by Barret in 1882. The first published report describing the disorder as an enamel defect was by Talbot as quoted by Witkop.[2] The term “hereditary opalescent dentin” was first used by Skillen,[3] Finn,[4] and Hodges and Finn[5] to describe the brown translucent teeth that have an opalescent sheen and are lacking in pulp chambers. Some authors suggest that the term “hereditary opalescent dentin” should be preferred over “DI” as it describes the general appearance better.

Since 1882, many case reports of DI,[6],[7] numerous researches related to genetic studies,[8],[9],[10] and revision of the classification are available in the literature.[11],[12],[13],[14] In this case report, the authors are highlighting a varied presentation of DI with a brief discussion. Every unique case needs to be recorded and reported which would enhance our knowledge for the better management of cases.

 Case Report

A 28-year-old male patient reported to the Department of Oral Medicine and Radiology with a complaint of spacing in the upper and lower anterior teeth for many years. There was no relevant medical history, family history, drug history (in the present or past), or any adverse oral habits. He did not report consanguineous marriage of his parents. The general physical examination was unremarkable. It was his first dental visit. Intraoral examination revealed relatively good oral hygiene with normally appearing oral mucosa with 28 teeth present. All the third molars were clinically missing, and spacing was present in the upper and lower anterior teeth. On a close observation, the second premolar had a translucent hue. [Figure 1], [Figure 2], [Figure 3] and tooth numbers 35 and 45 were slightly lingually placed. Morphologically, the crowns appeared normal. There was generalized mild enamel hypoplasia evident as chalky white opaque to yellowish presentation [Figure 2] and [Figure 3]. On enquiring, he did not reveal any positive history of trauma or decay either. According to the patient's knowledge, his deciduous dentition was normal with no discoloration or decay, which was confirmed by his parents. Other members of the family were also enquired and examined and found to be normal except for enamel hypoplasia in his mother's dentition [Figure 4]a and [Figure 4]b. The family reported a negative history of residing in a fluoride-affected belt.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

Angles Class I molar occlusion with spacing and a questionable diagnosis of DI was considered. To confirm the same, the patient was subjected to radiographic investigations after written consent. Orthopantomogram (OPG) revealed spacing between the entire upper and lower anterior teeth and missing third molars [Figure 5]. Interestingly, teeth 15, 35, and 45 showed normal enamel, thin dentin, and wide pulp chambers. There was well-demarcated radiolucency at the periapex, approximately measuring 0.8 cm x 0.8 cm without any carious involvement in 15. A final diagnosis of DI was confirmed after radiographic findings. Although the patient was educated and convinced for the treatment, he got one of his premolars extracted. Along with orthodontic correction, intentional root canal treatment was done using plasma-rich protein in 45 and mineral trioxide aggregate in 35 as obturating materials [Figure 6]. The patient is regularly being evaluated and followed for the maintenance of oral hygiene, replacement of the missing teeth, and crowns.{Figure 5}{Figure 6}

The patient was educated and counseled for this rare disorder, so as to observe any similar presentation in his future generations. This genetic disorder may present with variable degrees of penetrance and expressivity.


DI, also known as hereditary opalescent dentin that corresponds to a localized form of mesodermal dysplasia, is observed in histodifferentiation.[6]

The diagnosis is based on family history, pedigree construction, detailed clinical and radiographic examination. There is lot of disagreement on the classification of DI in the past. The Shield system does not account for the molecular etiologies of hereditary dentin defects. Therefore, DI was reclassified as Type I and II,[1],[14] where neither of them is associated with osteogenesis imperfecta, and DI Type I corresponds to Shields Type II, and DI Type II corresponds to Shields Type III, respectively. Thus, there is no substitute for DI Type I in this revised classification.

Classically, “shell teeth” demonstrates normal thickness of enamel in association with extremely thin dentin and dramatically enlarged pulps. The thin dentin may involve the entire tooth or be isolated to the roots as in this case also. This rare abnormality has been seen most frequently in deciduous teeth. The alteration may be unassociated with DI as an isolated finding in both dentitions and demonstrates normal tooth shape and color, a negative family history and diffuse involvement. In the isolated variant, slow but progressive root resorption occurs. Similar features are evident here, i.e., translucent teeth, wide radicular pulp, and positive family history of enamel hypoplasia (mother).[14]

Expressivity is variable even within an individual, with some teeth showing total pulpal obliteration, whereas in others, the dentin appears normal. The trait exhibits close to 100% penetrance. Significantly, enamel hypoplasia is noted in some patients. The enamel abnormality is thought to be a secondary defect and not a direct expression of the dentinogenesis gene.[15] Although the pulps are usually obliterated by excess dentin production, some teeth may show normal-sized pulps or pulpal enlargement (shell teeth).[16] The oral health professional of today is expected to clearly understand the physiology and biology of the oral cavity, to study the impact of diseases on its biophysiology, and to be aware of advances in materials, techniques, and medications available to treat diseases affecting it. Further research and clinical trials should clarify the effect of dentin hypomineralization (developmental conditions such as DI and acquired conditions such as fluorosis) on dentin bond strengths of different types of adhesive systems.

The present case differs from the earlier reports as only a single tooth in three different quadrants was involved. However, enamel hypoplasia was evident that is being mentioned in only a few cases in the literature.[14]

We could ascertain the periapical lesion of the tooth by radiographic (OPG) finding, and the patient lost his tooth due to refusal or delay in the treatment. Hence, every such varied case requires reporting as it adds to the literature, thereby enhancing our knowledge so as to diagnose and plan a better management.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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