Dentinogénesis Imperfecta y Displasia Dentinaria. A propósito de un caso / Dentinogenesis Imperfecta and Dentin Dysplasia. Case Report

La flexibilidad de la dentina permite resistir las fuerzas masticatorias. Esta propiedad hace que el esmalte subyacenteno se fracture. En los casos de dentinogénesis imperfecta y displasia dentinaria los pacientes tienen mas riesgo de fracturas y atriciones dentarias en edades muy tempranas. Se presenta un caso clínico de un paciente varón de 3,7 años que acude por primera vez a la consulta por dolor. A la exploración presenta múltiples caries, absceso de origen dentario, atriciones, dimensión vertical disminuida y dentinogénesis imperfecta. En los casos leves de osteogénesis imperfecta es posible que la dentinogénesis imperfecta sea la primera manifestaciónen ser detectada, por lo tanto, el odontólogo debe estar capacitado para saber diagnosticar y derivar al paciente al especialista para permitir un diagnóstico precoz de la enfermedad. La primera visita odontológica del niño/aal cumplir el año de vida es necesaria para detectar cualquier anomalía en el crecimiento de los dientes, huesos y establecer una relación entre el niño/a, el especialista y su entorno. (AU)

The flexibility of dentin allows it to resist masticatory forces. This property prevents the underlying enamel from fracturing. In cases of dentinogenesis imperfecta and dentin dysplasia, patients have a higher risk of fractures and dental attrition at a very early age. A clinical case of a 3.7-year-old male patient who comes to the clinic for the first time due to pain is presented. On examination he presented multiple caries, abscess of dental origin, attrition, decreased vertical dimension and dentinogenesis imperfecta. In mild cases of osteogenesis imperfecta, it is possible that dentinogenesis imperfecta is the first manifestation to be detected, therefore, the dentist must be trained to know how to diagnose and refer the patient to the specialist to allow an early diagnosis of the disease.The first dental visit of the child at the ageof one year is necessary to detect any anomaly in the growth of the teeth, bones and establish a relationship between the child, the specialist and their environment (AU)

Endodontic treatment of dentin dysplasia type I D.

Dentin dysplasia (DD) Type I is a developmental condition affecting dentin, inherited in an autosomal-dominant pattern or occurring due to a new mutation. Whilst the crowns of DD Type I affected teeth appear clinically normal, the roots are blunt and shortened. Pulp necrosis and periapical pathoses may be seen in the absence of obvious causes. Pulp stones and calcifications are frequently encountered. Endodontic management of DD may be challenging. A case of DD Type I, sub-classification d, in which spontaneous irreversible pulpitis developed on three mandibular incisors is documented. The case was managed by conventional endodontic treatment. Knowledge of this uncommon dental condition may assist dentists to adequately diagnose and manage these cases. Extraction should not be considered the first-line treatment option when sufficient root length is available to attempt endodontic treatment. Referral for medical evaluation is recommended to rule out systemic diseases which may mimic this condition.

An unusual variation of radicular dentin dysplasia: A rare case report with review of literature.

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.

Dentin dysplasia type I.

This paper describes a rare case of genetically determined dentin dysplasia type Iin 26-year-old male patient. The paper highlights anatomical and radiologicalaspects of dental abnormalities and emphasizes the significance of the educationof both general practitioners and paediatricians as regards referring patients withdiagnosed dentin dysplasia for a multi-specialty therapy.

Dentin dysplasia type 1 - clinical management dilemmas: A case report of first-generation sufferers.

Dentine Dysplasia is a rare genetic condition. The treatment options and dilemmas associated with the condition remain undiscovered so far. This article highlights the variations in traits and challenges faced in the treatment of the cases.

Symmetric multiquadrant isolated dentin dysplasia (SMIDD), a unique presentation mimicking dentin dysplasia type 1b.

Dentin dysplasia (DD) is a rare developmental dentin disorder that causes root malformation. It is divided into radicular DD type 1 (DD-1) and coronal DD type 2 (DD-2). Recently, a new entity causing localized root malformation of permanent first molars that resembles DD-1b has been described as molar-incisor malformation (MIM). We report and compare 4 new cases that exhibit similar clinical, histologic, and radiographic features to the new entity, MIM. We believe MIM and our 4 cases to be the same entity, which is nonhereditary and, because of the isolated but bilaterally symmetric pattern of involvement, may be caused by a short-duration environmental insult that disrupts normal development/function of Hertwig's epithelial root sheath. We propose the name symmetrical multiquadrant isolated dentin dysplasia as the most appropriate descriptive designation for this unusual but highly distinctive anomaly.

[Difficulties in differential diagnosis of dentin dysplasia. Case report]. / A dentin dysplasia differenciáldiagnosztikai nehézségei. Esetismertetés.

In cases of periapical lesions of unknown origin we have to keep in mind the possibility of dentin dysplasia. From the differential diagnostic point of view of differential diagnosis it is important to recognize this disease as it significantly influences the treatment modality. It is an anomaly of unknown etiology that could affect both deciduous and permanent dentition. Dentin dysplasia presentation varies clinically and radiologically. A rare manifestation which affects 1: 100000 patients is spontaneously occurring either as periapical abscess or odontogenic cyst. The affected teeth could become mobile and eventually lost. Dentin dysplasia is a genetic disease which shows autosomal dominant inheritance and characterized by abnormal formation of dentin structure which occurs during tooth development. For this article we have reviewed available literature and PubMed database. Dentin dysplasia increases the risk of early tooth loss and associated with it esthetic and functional disturbances. As a result it can influence the psychological and social status of the patients and affect their quality of life.

Frameshift mutations in dentin phosphoprotein and dependence of dentin disease phenotype on mutation location.

We describe results from a mutational analysis of the region of the dentin sialophosphoprotein (DSPP) gene encoding dentin phosphoprotein (DPP) in 12 families with dominantly inherited dentin diseases. In eight families (five mutations in the N-terminal third of DPP), the clinical and radiologic features were uniform and compatible with dentin dysplasia type II (DD-II) with major clinical signs in the deciduous dentition. In the other families (four mutations in the more C-terminal part), the permanent teeth also were affected, and the diseases could be classified as variants of dentinogenesis imperfecta. Attrition was not prominent, but periapical infections were common. Discoloring with varying intensity was evident, and pulps and root canals were obliterated in the permanent dentition. All mutations caused a frameshift that replaced the Ser-Ser-Asx repeat by a code for a hydrophobic downstream sequence of approximately original length. We conclude that frameshift mutations in DSPP explain a significant part of dentin diseases. Furthermore, we propose that the location of the mutation is reflected in the phenotypic features as a gradient from DD-II to more severe disease that does not conform to the classic definitions of DI-II.

Multifocal odontoblastic dysplasia in a dog.

A two-year-old Boerboel dog presented for a discolored left maxillary canine tooth. Dental radiographs revealed abnormally mineralized pulp within the discolored tooth. Similar radiographic findings were also seen in both maxillary third incisor teeth and in the remaining canine teeth to varying degrees. The discolored tooth was treated by surgical extraction and histopathology revealed abnormal dentin deposition within the canal indicative of dentin dysplasia. Although not previously documented in the dog, the pulpal changes in multiple teeth of the dog reported here were similar to those described for odontoblastic dysplasia in humans. This case report includes a review of developmental abnormalities of dentin in humans and pulpal response to inflammation and injury.

Florid cemento-osseous dysplasia mimicking multiple periapical pathology--an endodontic dilemma.

A case of florid cemento-osseous dysplasia (COD) mimicking periapical pathology is presented. The fact that the patient's lesion failed to resolve three years after root canal therapy, in addition to the presence of a mixed radiolucency with discreet radiopacities, mandated a biopsy which (along with radiographic co-relation) confirmed the diagnosis of cemento-osseous dysplasia. This case report illustrates the point that periapical radiolucencies may represent benign fibro-osseous lesions that may be overlooked or result in unnecessary endodontic treatment.

Dentin dysplasia type I treated with onlay bone grafting, sinus augmentation, and osseointegrated implants.

Dentin dysplasia is a defect of dentin development that was classified by Shields as type I and type II. The teeth in dentin dysplasia type I are characterized by normal enamel and abnormal dentin in the crown and root. For that reason, this disorder is characterized by dental caries, early exfoliation of the teeth and, consequently, maxillomandibular bony atrophy. In this report, we present a case of a young girl affected by dentin dysplasia type I, treated with a combination of onlay bone grafting and a sinus lift technique to accomplish implant placement. This case showed that onlay autogenous grafting and a sinus lift technique are well-tested methods of bony augmentation and can be useful in patients with congenital dentin defects, such as dentin dysplasia.

Dentin dysplasia type I: report of atypical cases in the permanent and mixed dentitions.

Dentin dysplasia type I is a rare hereditary disturbance of dentin formation characterized clinically by nearly normal appearing crowns and severe hypermobility of teeth. Radiographic analysis shows obliteration of all pulp chambers, short, blunted, and malformed roots, and periapical radiolucencies of noncarious teeth. This paper presents 2 cases demonstrating both classic and atypical features of type I dentin dysplasia in the mixed and permanent dentitions. The clinical, radiographic, and histopathologic\findings of this condition and treatment are described.

Assessment of dysplastic dentin in osteogenesis imperfecta and dentinogenesis imperfecta.

Two semiquantitative scoring systems, Clinical Radiographic Score (CRS) and Dysplastic Dentin Score (DDS), were introduced for analyzing degree of dysplastic manifestations in dentin. The utility of both systems was demonstrated in a large material of teeth from patients with dentinogenesis imperfecta (DI) and osteogenesis imperfecta (OI). Twenty teeth from healthy controls, 81 teeth from 40 patients with OI, and 18 teeth with DI without OI (DI type II) were examined. The degree of dysplasia was correlated with type and form of OI and type of DI. The median DDS did not differ between DI associated with OI (DI type I) and DI type II. DDS in OI patients without clinical signs of DI was above that of control teeth. Both circumpulpal and mantle dentin showed increased DDS, although circumpulpal dentin was more severely affected. The median DDS was highest for the most severe type of non-lethal OI (type III). DDS increased significantly with form (severity) of OI. A significant association between DDS and CRS was found, although diagnosis of DI in less severe cases was not possible based on radiographic or clinical signs alone. Thus, the DDS system proved valuable when the CRS system based on radiographic/clinical manifestations failed, the most significant finding being subclinical histological manifestations of DI in patients with OI but without clinical or radiographic signs of DI. These subtle dysplastic changes are most likely an expression of genetic disturbances associated with OI and should not be diagnosed as DI, but rather be termed histologic manifestations of dysplastic dentin associated with OI.

Hereditary pattern for dentinal dysplasia type Id: a case report.

Generalized pulpal calcifications arouse suspicion of diseases or conditions of systemic or hereditary origin. This case report describes a 45-year-old patient with generalized pulpal calcifications and bulging of the roots in areas corresponding to the pulp chambers in otherwise normal teeth. Similar findings were present in the patient's daughters and brother. This pattern of pulpal calcifications is consistent with the hereditary condition of dentinal dysplasia type Id.

Dentin dysplasia type I--a case report.

A case report on dentin dysplasia type I, a congenital disease (autosomal dominant gene defect) affecting deciduous and permanent teeth, is depicted including representations of clinical and histological features, X-ray and CT-findings. Therapy includes extraction of all teeth, ectomization of cystic alteration, revision of paranasal sinus. Aesthetic and functional rehabilitation by means of insertion of a complete denture was achieved.

Dentin dysplasia type I. Report of case and ultrastructural study.

Radicular dentin dysplasia (DD-I) is a rare hereditary dental alteration. It is characterized clinically by almost normal looking crowns and severe hypermobility of the teeth. The radiographic analysis, on the other hand, discloses the obliteration of all pulp chambers, the short, malformed roots and plenty of periapical bone radiolucencies on noncarious teeth. A case of radicular dentin dysplasia is presented. In this 43-year-old woman the diagnosis was supported, besides the clinical and radiographic analysis, by the pedigree of the proband, which showed the autosomal dominant pattern of feature transmission. Further-more, the electron microscopic analysis of one extracted molar revealed the atubular structure of the secondary dentin, and its globular organization.

Dentin dysplasia type I: five cases within one family.

Five cases of dentin dysplasia type I within one family are described. Clinically and radiologically, such patients are characterized by a delayed eruption pattern, opacity of the incisional margins, hypermobility of the teeth, short and defective roots, and obliterated pulp chambers. A conservative attitude toward the treatment of common conditions in dentin dysplasia type I favors the preservation of a vulnerable dentition.

Dentin dysplasia type I. Radiologic and genetic perspectives in a six-generation family.

This is a report of a kindred of at least 181 members, of whom 35 exhibit or are reported to have dentin dysplasia type I. Six others are suspected of having the condition. Radiographic evidence that included obliterated or semilunar pulp chambers and short or undeveloped roots confirmed the diagnosis in 18 persons. The autosomal dominant mode of inheritance has been confirmed. One hundred percent penetrance has been demonstrated. There were insufficient data to determine the degree of expressivity.