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Dentin dysplasia: Single-tooth involvement? Veena V. Naik, MDS1/Alka D. Kale, MDS2 Dentin dysplasia is a genetic defect of dentin formation inherited as an autosomal dominant trait. It is characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology. Once thought to be a single entity, dentin dysplasia has now been divided into type I (radicular) and II (coronal). Type I is by far the more common. Both types include multiple/generalized involvement of primary and permanent dentition. Combinations of both types have also been described in the literature. Four distinct forms of dentin dysplasia type I and 1 form of dentin dysplasia type II are identified. Although there seems to be no need to identify more than 2 distinct types of this relatively rare inherited defect of human dentin, the possible existence of additional forms of the disease cannot be ruled out. Here is a case report of dentin dysplasia in a single tooth, with crown and roots of normal dimensions, associated with severe pain and mobility and histologically involving both coronal and radicular dentin. Focal odontoblastic dysplasia or dentin dysplasia type III could be the new entity. (Quintessence Int 2009;40:183–186)

Key words: dentin dysplasia, odontoblastic dysplasia

Dentin dysplasia is a rare disturbance of dentin formation characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology. The first description of the disease was in 1920 by Ballschmiede who described this phenomenon as rootless teeth.1 Rushton in 1939 first designated it dentin dysplasia.2 Dentin dysplasia, a hereditary disease, is transmitted as an autosomal dominant characteristic. Shields et al described 2 distinct types of dentin dysplasia on the basis of their radiographic and histologic appearances.3 In type I (radicular), both primary and permanent dentitions are affected, although the

1

Reader, Department of Oral Pathology, Karnataka Lingayat Education Society’s Vishwanath Katti (KLE VK) Institute of Dental Sciences, Nehru Nagar, Belgaum, Karnataka, India.

2

Professor and Head, Department of Oral Pathology, VicePrincipal, KLE VK Institute of Dental Sciences, Nehru Nagar, Belgaum, Karnataka, India.

Correspondence: Dr Veena V. Naik, A-14/1, JNMC Staff Quarters, Nehru Nagar, Belgaum–590010, Karnataka, India. Fax: 91831 2470640. Email: veenavnaik@rediffmail.com

teeth appear normal in morphologic appearance and color. Teeth characteristically exhibit extreme mobility as a result of the abnormally short roots. In type II (coronal), both the dentitions are affected; however, the deciduous teeth have a yellow, brown, or bluish-gray, opalescent appearance, while the permanent dentition appears normal. Radiographs of the deciduous dentition in type I show complete pulp obliteration, whereas the permanent dentition may exhibit a crescent-shaped remnant. In addition, there are periapical radiolucencies without an obvious cause. In type II, the pulp chambers and root canals are shaped like a thistle tube with an accumulation of pulp stones. There are no periapical radiolucencies. Histologically, normal dentinal tubule formation appears to be blocked, and new dentin forms around the obstacles, which takes on the characteristics of lava flowing around boulders.4 This article describes a case of dentin dysplasia in a single tooth, with crown and roots of normal dimensions, associated with severe pain and mobility and histologically involving both coronal and radicular dentin.

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Fig 1 Intraoral periapical radiograph of the mandibular right second molar showing obliteration of the pulp cavity and poor density contrast.

Fig 2 (left)

Gross specimen showing normal dimensions.

Fig 3 (right) Bisected specimen revealing obliteration of the pulp chamber and root canals.

CASE REPORT A 45-year-old man reported to our institution complaining of severe pain in the mandibular right second molar that had persisted for the previous 1.5 months. On examination, the tooth appeared clinically normal. It showed grade 2 mobility. Slight probing elicited severe pain. The intraoral periapical radiograph showed obliteration of the pulp cavity and poor density contrast, giving the impression of an enlarged pulp chamber and a thin rim of enamel coronally. The mesial root was curved distally, giving a dancing-tooth appearance with no periapical radiolucency (Fig 1). A panoramic radiograph did not reveal any abnormality in other teeth. A full complement of normal teeth was present. The patient was given antibiotics, and the tooth was extracted after 7 days, as pain did not recede. The extracted tooth was then sent to the Department of Oral Pathology. Clinical examination of the gross specimen revealed an intact, normal tooth with occlusal attrition, some extrinsic orangish-yellow staining, and a few superficial coronal cracks. The tooth was of normal dimensions, the crown measuring about 8 mm and roots about 14 mm (Fig 2). The specimen was sectioned mesiodistally into halves. The entire pulp chamber and root canals appeared obliterated. The pulp chamber appeared to be filled with a whitish, hard material (Fig 3). One half of the cut sec-

tion was decalcified with 10% hydrochloric acid. The specimen was then processed, and sections of 4 µm were stained with hematoxylin-eosin. The other half was prepared for a ground section 7 µm thick and mounted on a glass slide. Histologic examination of the decalcified section revealed total obliteration of the pulp and a layer of mantle dentin (Fig 4). The pulp chamber and root canals were filled with tubular dentin, osteodentin, and irregular, globular trabecular masses of dentin (Fig 5). Collagenous stroma were interspersed among these mineralized areas. Odontoblast cells were present near the dentin masses. Normal cementum was present. The ground section showed normal enamel covering the crown and thin cementum lining the roots. The apical end of the roots consisted of a thick zone of cellular cementum. The entire dentin and pulp space consisted of irregular globular masses of dentin with a crescent-shaped pulpal remnant parallel to the cementoenamel junction. Some areas of normal tubular dentin were adjacent to the enamel. In other areas, a trabecular pattern of osteodentin and dentinlike material was seen in abundance. Pulp space was totally obliterated (Fig 6). Based on gross features and histologic examination of the ground section and decalcified section, diagnosis of dentin dysplasia involving a single tooth was made.

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Fig 4 (left) Hematoxylin-eosin–stained (original magnification  10) decalcified tooth section revealing total obliteration of the pulp (stereophotomicrograph). Fig 5 (center) Pulp chamber and root canals filled with tubular dentin, osteodentin, and irregular, globular trabecular masses of dentin with interspersed collagenous stroma (hematoxylin-eosin, original magnification  40). Fig 6 (right) Ground section (stereophotomicrograph) showing the entire dentin and pulp space consisting of irregular globular masses of dentin.

DISCUSSION The incidence of dentin dysplasia has been estimated at 1:100,000. Though classically described as an isolated defect, dentin dysplasia has been associated with several disorders.5–7 The mechanism of dentin dysplasia is still unclear. Sauk et al, in a scanning electron microscope study, postulated that dentin dysplasia is a defect in the epithelial component of the developing tooth germ in which the invagination of the root sheath occurs too soon and, in a sequence of futile attempts to correct itself, results in a stunted root form with an unusual whorl-like pattern of dentin obliterating the pulp chambers.8 Wesley et al disagreed with this suggestion and offered as an alternative hypothesis an error in the continual induction of odontoblasts after the interaction with the ameloblastic layer.9 Witkop suggested that the dysplasia results from epithelial cells from the sheath of Hertwig breaking off and migrating into dental papilla, where they induce odontoblast differentiation and dentin formation.10 Melnick et al suggested that the abnormal root morphology is caused by abnormal differentiation and/or function of the odontoblasts.11 Clearly, the precise nature of the defect has yet to be determined.

Eastman et al12 in 1977 and Ciola et al13 in 1978 described 2 conditions for which they suggested the name “dentin dysplasia type III.” Ciola et al described 2 or more forms of dentin dysplasia type I in the same patient.13 The case of Eastman et al appears different from type I and type II, being focal rather than generalized.12 They described their case as focal odontoblastic dysplasia. Gertzman et al14 in 1983 reported a condition of interrupted root development that they called a new form of dentin dysplasia. In the present case, although the mandibular right second molar exhibited grade 2 mobility, there was no periapical pathosis. Mobility was due to poor periodontal conditions. The crown and roots were of normal dimensions. The pulp was completely obliterated with few pulpal remnants, and above all, the dysplasia was focal (affecting a single tooth) rather than generalized. Dentin dysplasia, if hereditary, cannot be a localized malformation, but must be observed with all teeth. Therefore, we would like to describe this as focal odontoblastic dysplasia. Reports of other similar cases are probably needed to determine whether another type of designation (dentin dysplasia type III) is warranted.

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REFERENCES 1. Ballschmiede G. Disseration: Berlin, 1920. In: Apffelstaedt M. Malformations of the Jaws and Teeth. New York: Oxford University Press, 1930. 2. Rushton MA. A case of dentin dysplasia. Guys Hosp Rep 1939;89:369–373. 3. Sheilds ED, Bixler D, El-Kafrawy AM. A proposed classification of heritable human dentin defects with a description of a new entity. Arch Oral Biol 1973;18: 543–553. 4. Shafer WG, Hine MK, Levy BM. A Textbook of Oral Pathology, ed 4. Philadelphia: Saunders, 1983:61–63. 5. Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North Am 1975;19:25–45.

9. Wesley RK, Wysocki GP, Mintz SM, Jackson J. Dentin dysplasia type I: Clinical, morphologic and genetic studies of a case. Oral Surg Oral Med Oral Pathol 1976;41:516–524. 10. Witkop CJ Jr. Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: Problems in classification. J Oral Pathol 1976;17: 547–553. 11. Melnick M, Levin LS, Brady J. Dentin dysplasia type I, a scanning electron microscopic analysis of the primary dentition. Oral Surg Oral Med Oral Pathol 1980;50: 335–339. 12. Eastman JR, Melnick M, Goldblatt LI. Focal odontoblastic dysplasia: Dentin dysplasia type III? Oral Surg Oral Med Oral Pathol 1977;44:909–914. 13. Ciola B, Bahn SL, Goviea GL. Radiographic manifes-

6. Morris ME, Augsburger RH. Dentin dysplasia with

tation of an unusual combination of type I and type

sclerotic bone and skeletal anomalies inherited as

II dentin dysplasia. Oral Surg Oral Med Oral Pathol

an autosomal dominant trait: A new syndrome. Oral Surg Oral Med Oral Pathol 1977;43:267–283. 7. Witkop CJ. Clinical aspects of dental anomalies. Int Dent J 1976;26:378–390.

1978;45:317–322. 14. Gertzman GB, Hoffman J, McColgan P. Interrupted root development—A new form of dentin dysplasia. Clin Prev Dent 1983;5:21–24.

8. Sauk JJ Jr, Lyon HW, Trowbridge HO, Witkop CJ Jr. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Oral Surg Oral Med Oral Pathol 1972;33:763–771.

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displasia dentinaria discusion de caso  

Reporte de caso de displasia dentinaria