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Variation of dentin dysplasia type I: report of atypical findings in the permanent dentition. Dentin dysplasia is a rare defect of dentin development with an autosomal dominant pattern of inheritance, which is generally divided into 2 main classes based on the clinical and radiographic appearance of the affected dental tissues: type I, which affects the root portion and type II, which affects the coronal portion of the tooth.

This paper reports the case of a child aged 10 years and 8 months with both classic and atypical features of dentin dysplasia type I in the permanent dentition. Only few mandibular teeth were affected and presented clinically normal appearing crowns, moderate to severe mobility, short, blunt or almost absent roots. However, no evidence of pulp chamber obliteration or periapical radiolucencies was found.

The clinical and radiographic characteristics observed in this patient are different from those reported in the literature, which suggests that this may be a variation of dentin dysplasia type I expression. KeyWords: dentin dysplasia, permanent dentition, dental treatment.

Dentin dysplasia is a rare defect of dentin development with an autosomal dominant pattern of inheritance 1,2 that affects one in every , individuals 3,4 and manifests in both primary 2, and permanent 2,5, dentitions.

Shields et al. A third type of dentin dysplasia or focal odontoblastic dysplasia, with radiographic aspects of both types of dysplasia, has also been described DD1 is characterized clinically by nearly normal appearing crowns and hypermobility of the teeth 11, Frequent findings include delayed dental eruption pattern 13,14 , opaque incisal borders 14 , spontaneous premature exfoliation 14 , because of the short, blunt, tapering, or even absent roots 1 , and discomfort caused by the severe tooth mobility, especially after meals 2,8,9.

Radiographic examination is an important diagnostic tool for identifying this dental anomaly 12 , revealing short, blunt and malformed roots, similar to those observed in taurodontic teeth, obliteration of all pulp chambers with crescent-shaped pulp remnants parallel to the cementoenamel junction and several periapical radiolucencies in noncarious teeth 1,2,5,7,8, Microscopic examination of affected teeth reveals a thin enamel layer 7 with atubular secondary dentin, globular organization and, more centrally, spherical structures suggestive of multiple pulp calcifications.

Since the mantle dentin is not affected, the teeth have an apparently normal clinical crown Although numerous theories have been proposed, the etiology of dentin dysplasia remains unknown. Logan et al. Sauk et al. Witkop 3 proposed that internal cells of the developing dental organ would be displaced and proliferate in the dental papilla, producing ectopic dentin formation. On the other hand, according to Wesley et al. Since there is no consensus in the literature regarding the etiology of dentin dysplasia and considering that this is a rare defect of dentin development, case reports can be useful to help elucidating and understanding some aspects related to this condition.

This paper reports the case of a child with dental alterations suggestive of DD1, in whom only few permanent mandibular teeth were affected and different features from those found in the literature were observed. The mother reported that the patient was deaf since birth, and no other medical condition was mentioned. Ethics Committee approval protocol The intraoral clinical examination revealed the presence of the following teeth: 16, 15, 53, 12, 11, 21, 22, 63, 65, 26, 35, 74, 73, 32, 31, 41, 42, 43, 44 and Panoramic radiographic examination confirmed that teeth 36 and 46 were missing and showed that teeth 34, 35 and 37 presented alteration in the pattern of root formation compared to the contralateral teeth Fig.

According to the mother, teeth 36 and 46 had been extracted due extensive carious lesions. Dental care included extraction of tooth 63, endodontic treatment of tooth 16, restoration of teeth 11 and 16 with composite resin, placement of pit-and-fissure sealant in teeth 35, 14, 15 and 25, and placement of a stainless steel crown in tooth A new panoramic radiograph taken 1 year later revealed arrest of root formation in teeth 34, 35 and 37 Fig. Clinically, moderate to severe mobility was observed in teeth 34 and 35, and areas of enamel hypomineralization were found in teeth 31, 32, 33, 42 and All other teeth presented normal clinical and radiographic appearance.

One year later, the panoramic radiographic was repeated and showed no evolution in root formation in teeth 34, 35 and 37 Fig. Neither pulp chamber obliteration nor periapical radiolucencies were observed in any of the panoramic radiographic images, which were confirmed by periapical radiograph Fig.

In the present case, the installation of a functional removable space maintainer was no not necessary because the premolars had already erupted.

The progression of the case is under continuing evaluation. In this case, the patient presented permanent dentition with normal root development on 3 hemi-arches. However, in the left posterior mandibular area, the teeth presented either short, blunt tooth 35 or almost absent teeth 34 and 37 roots and consequent hypermobility. Periodontal ligament thickening was observed in tooth 34 due to the incidence of occlusal forces in this region. The clinical and radiographic findings of this case led us to suspect of a variation of DD1, since some of the typical signs of this dental developmental abnormality, such as pulp chamber obliteration and periapical radiolucencies associated with sound teeth 2,5,14,16 , were not present.

Scola and Watts 13 proposed a subclassification of DD1 as: total DD1, characterized by the presence of teeth with significantly narrowed or obliterated pulp spaces and permanent teeth with short roots, and subtotal DD1, characterized by permanent teeth with roots of intermediate length.

Four subtypes were proposed: in the first, total pulp chamber obliteration, no root development and multiple periapical radiolucencies are observed; in the second and third, the pulp chamber is less obliterated and present crescent-shaped radiolucent areas, minimal root formation and periapical radiolucencies are less frequent; in the fourth subtype, there are radiographically visible and delimited pulp chambers, pulpal nodules are found in the coronal third of the canal, the roots have a significant development and few or no periapical radiolucencies are observed.

However, no radiolucent areas suggestive of periapical lesions were observed. The patient presented clinical and radiographic characteristics of DD1 only in the left mandibular area, which agrees with the outcomes of a previous study In that study, only the mandibular left quadrant exhibited typical findings of DD1, while the maxillary right and left molars presented taurodontism.

These findings suggest that this type of developmental defect of human dentin may occur due to localized abnormalities in cell proliferation and function, in the same way as observed in regional odontodysplasia Recently, a case of dentin dysplasia involving a single tooth was reported. The authors described as focal odontoblastic dysplasia and suggested that reports of other similar cases are probably needed to determine another type of classification dentin dysplasia type III Van Dis and Allen 4 reported four cases of DD1 in which the radiographic features were somewhat different from those of other cases.

Anterior teeth appeared to be less affected than posterior, and tooth loss was less prevalent, suggesting thereby that this type of dentin dysplasia can be divided into 2 subgroups, as proposed by Scola and Watts Variations of DD1 may occur 14 , which leads us to assume that the absence of pulp chamber obliteration and periapical lesions associated with sound teeth, as observed in the present case, represents another possible expression of this disorder.

Most DD1 cases have autosomal dominant inheritance, which means that individuals of the same family may be affected 2, However, reports of cases without signs of a family heritage are found in the literature 6,16 in the same way as described for our patient. There is no specific treatment for this rare genetic disorder affecting the dentin development of the teeth.

Only procedures to avoid the premature loss of hypermobile teeth and to stimulate the normal development of the occlusion can be undertaken. The early diagnosis and continuous follow-up of cases of DD1 by a pediatric dentist is of paramount importance 6 because the affected teeth have a very unfavorable prognosis due to the short roots and presence of associated periapical radiolucencies.

If necessary, space loss due to spontaneous exfoliation of hypermobile teeth can be avoided by the placement of removable space maintainers or orthodontic appliances While numerous cases of DD1 have been described, some clinical and theoretical issues remain inconclusive. More detailed documentation of cases of DD1 is needed in order to establish relevant clinical and radiographic signs that will aid in the diagnosis of this disorder 9. The presentation of case reports in which classic signs of DD1 are found, but other typical features are not present highlights the clinical and radiographic variations of DD1.

In the present case, both classic clinically normal appearing crowns, moderate to severe tooth mobility, short, blunt or almost absent roots and atypical no evidence of pulp chamber obliteration or periapical radiolucencies in noncarious teeth features of DD1 were found in the permanent teeth of boy aged 10 years and 8 months. A proposed classification for heritable human dentine defects with a description of a new entity.

Arch Oral Biol ; Ansari G, Reid JS. Dentinal dysplasia type I: review of the literature and report of a family. Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North Am ; Dentinal dysplasia type I: a report of four cases. Dentomaxillofac Radiol ; Seow WK, Shusterman S. Spectrum of dentin dysplasia in a family: case report and literature review.

Pediatr Dent ; Dentinal dysplasia type I: report of an atypical case in the primary dentition. Dentin dysplasia type I - a case report. Ann Anat ; Dentinal dysplasia type I: report of a case.

Int J Paediatr Dent ; Dentin dysplasia type I: report of atypical cases in the permanent and mixed dentitions. Focal odontoblastic dysplasia: dentin dysplasia type III? Dentinal dysplasia. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Dentinal dysplasia type I. A subclassification. Br J Orthod ; Dentin dysplasia type I: five cases within one family.

Dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case. Dentin dysplasia type I: a case report and review of the literature. J Med Case Reports ; Dentin dysplasia: review of the literature and a proposed subclassification based on radiographic findings. Localized deficient root development associated with taurodontism: case report.

Dentin dysplasia: Single-tooth involvement? Quintessence Int ; Clinical, radiographic, and histological manifestations of dentin dysplasia, type I: Report of case. J Am Dent Assoc ; Correspondence: Profa. All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License.

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