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Developmental dental hard tissues disorders are rarely diagnosed both in children and adults, and their treatment is a problem for dentist. Despite the fact that molecular genetics studies allow to discriminate some dentine disorders, pathogenesis of radicular dentin dysplasia is still obscure. Type I dentin dysplasia is characterized by normal or slightly coloured crowns with no roots or only rudimentary roots, reduced pulp space in permanent teeth and incomplete or total obliteration of the pulp chambers, and periapical radiolucent areas or cysts which might result in premature loss of tooth. Fortunately, teeth demonstrate higher resistance to caries than normal teeth do. Dentin dysplasia type II is characterized by yellow, brown, grey, translucent primary teeth with complete pulpal obliteration. Permanent teeth are normal or might be slightly coloured. Roots are normal in size, but pulp chamber has pulp stones. A third type of dentine dysplasia or focal odontoblastic dysplasia has radiographic aspects of the other two types of disease. Also dentine dysplasia type I has subtypes Ia, Ib, Ic and Id. Dentine dysplasia Ia is characterized by complete obliteration of the pulp, disorder of root development, and many periapical radiolucent areas. A single small horizontally oriented and half-moon shaped pulp is presented in case of dentine dysplasia Ib, roots have a few millimetres length and frequent periapical radiolucencies. Dentine dysplasia Ic is characterized by the presence of two horizontal or vertical semicircle-shaped pulpal remnants which surround dentine in pulp chamber. Also teeth have shortened roots and variable periapical radiolucencies. Dentine dysplasia Id is characterized by the distinct pulp chambers with pulp stones in the coronal third of the root canal; periapical radiolucencies are possible as well. Aim of this study was to analyse clinical case of dentine dysplasia in 8,5-year-old boy. A 8,5-year old boy patient with parents came to a dental office due to caries cavity in tooth 62. Shape of tooth crowns was not changed and teeth with no signs of mobility; white lines and spots on teeth are were detected. Based on age, the decision of orthopantomogram was made to evaluate status of primary and permanent teeth. The image showed almost obliterated pulp chamber in both erupted and developing teeth and considerably shortened, blunted and malformed roots without a visible lumen of root canals in primary teeth. Periapical lesions were found about roots of primary molars and follicles of teeth 12 and 14 without caries lesions. Patient's medical history revealed no disturbance in general health. Clinical examination of the patient's mother did not show dental hard tissues disorders, but radiograph examination revealed such disorder in the orthopantomogram. Thus, history, clinical and radiographic findings revealed this case as dentine dysplasia type I subtype 1c inherited from mother. Oral rehabilitation of patients with dentine dysplasia type I require effective prevention and a complex approach. Thus, meticulous oral hygiene measures and dietary instructions, regular check-ups twice a year were established for this patient; orthodontic consultation was recommended. Varnish «Ftoplen-LC» was applied on the permanent teeth 3 times. To prevent early exfoliation, plan of preventive measures was developed included application of fluoride varnish twice a year and fissure sealing in the premolars in 9-10 years and in the second premolars in 12-13 years. Treatment of dentine dysplasia I type that depends on patient age and severity of disease may include also filling of carious teeth, orthodontic, surgical and orthodontic treatment. In this regard, dentist has to know developmental dental hard tissues disorders and select measures to prolong the retention of affected teeth maintaining them as long as possible.

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.

Dentin dysplasia is an autosomal-dominant genetic abnormality that occurs in humans and results in diffuse radiographic dental abnormalities and variable tooth discoloration due to an underlying defect in secondary dentinogenesis. This case report presents distinctive radiographic and histopathologic dental abnormalities in a dog that are consistent with generalized dentin dysplasia. These findings are similar to but not completely analogous to any specific clinical type of dentin dysplasia in humans. Grossly, the majority of the teeth in this case were discolored and most were determined to be vital. Dentin dysplasia should be included in the list of differential diagnoses of discolored teeth and notably this form of discoloration does not necessarily indicate loss of vitality.

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.

Background: Dentin dysplasia (DD) is a rare disturbance of dentin formation, characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology. In DD type I, the teeth appear clinically normal in morphologic appearance and color. Radiographic analysis shows obliteration of all pulp chambers as well as short, blunted, and malformed or absent roots with multiple periapical radiolucencies involving apparently intact teeth. Case Presentation: We present a case of a 15-year-old girl with a developmental disturbance of dentin similar to DD type 1. Radiographic examination revealed multiple affected teeth with unique findings in accordance with DD 1d subtype. This article highlights the clinical and radiographic findings of this condition along with the differential diagnosis and management. Conclusion: Management of patients with DD is difficult and requires a multidisciplinary approach. Early diagnosis of the condition is important for the initiation of effective preventive treatment.

Dentinogenesis Imperfecta and dentin dysplasia are genetic oral diseases inherited in a simple autosomal dominant mode, with high penetrance and a low mutation rate. Both of them are present with bulbous crowns, marked cervical constrictions, severe attritions, few periapical radiolucencies, and premature tooth loss. The diagnosis is based on family history, and detailed clinical examination, while genetic diagnosis may become useful in the future once sufficient disease-causing mutations have been discovered. Here, we present a case with overlapping features of both dentinogenesis imperfecta and dentin dysplasia asserting both the anomalies to be part of the same continuum of the genetic event.

AIM: The research has been aimed at optimization of approaches to diagnosis, prevention, and treatment strategies to the hereditary dental diseases based on the analysis of modern relevant information. METHODS: Using the methods of content analysis and cluster analysis, the information included in periodicals, as well as educational and non-regulatory publications for the period of 2011–2019, was systematized and structured; the analysis of sources devoted to modern approaches to the diagnosis, prevention, and treatment of hereditary dental diseases, their genetic etiology, with the description of clinical cases and illustrations for each example, was conducted; hereditary dental diseases were classified; based on a comparative analysis, approaches to the diagnosis, treatment, and prevention of these diseases were optimized. RESULTS: The most significant hereditary dental diseases have been identified, the classification of approaches to their diagnosis and treatment has been presented, the scheme of drug therapy has been optimized depending on the pathology. CONCLUSION: The systematic analysis of information on hereditary dental diseases with the description of clinical cases for each nosology has been carried out for the 1st time, and the approaches to their diagnosis and treatment have been optimized with due consideration of domestic and foreign experience.

Introduction: “Molar-incisor malformation” (MIM) or “Molar root-incisor malformation” is a recently reported dental anomaly of unknown etiology, possibly associated with systemic complications, which affects the development of first permanent molar roots and dental enamel of central incisors. Objective: To conduct a literature review on “Molarincisor malformation”, also known as “Molar root-incisor malformation”, discussing its clinical, radiographic/tomographic and microscopic aspects; differential diagnosis and treatment possibilities. Sources of data: Electronic search was performed on the MEDLINE database in March 2021, without limit regarding the year of publication. The terms used were “molar-incisor malformation”, “molar-root incisor malformation”, “root malformation”, “root development”, “tooth roots”, “abnormalities”. Synthesis of data: Fifteen articles, most of them case series, were included. In general, medical historyrevealed clinical complications during pregnancy and / or the first years of life. Clinical features included tooth enamel defects in the cervical region of incisors and marked mobility of permanent molars and incisors. Radiographically, partially obliterated pulp chambers, short, thin and incomplete roots of first permanent molars and incisors, were observed. Microscopically, the occurrence of a hypercalcified dentin layer, in the form of a lens, inside the pulp chamber, at the level of the cementum-enamel junction, called“mineralized cervical diaphragm”, was reported. Conclusion: “Molar-incisor malformation” is an anomaly characterized by changes in root development, pulp chamber and enamel in permanent molars and incisors. The differential diagnosis includes Dentin Dysplasia type I and Regional Odontodysplasia. Medical and familyhistories are essential for the final diagnosis, and treatment, which despite not having an established protocol, requires a multidisciplinary approach and conventional treatments such as tooth extraction, endodontics, orthodontics, and dental implants. Introdução: “Malformação molar-incisivo” (MIM) ou “Malformação radicular molarincisivo” é uma anomalia dental recém-reportada de etiologia desconhecida, possivelmente associada a complicações sistêmicas, que afeta o desenvolvimento de raízes dos primeiros molares permanentes e esmalte dentário de incisivos centrais. Objetivo: Realizar uma revisão da literatura sobre “Malformação molar incisivo”, também conhecida como “Malformação raiz-molar incisivo”, discutindo seus aspectos clínicos, radiográficos/tomográficos, diagnóstico diferencial e possibilidades de tratamento. Fonte dos dados: Busca eletrônica foi realizada na base MEDLINE, em março de 2021, sem limite quanto ao ano de publicação. Os termos pesquisados foram “molarincisor malformation”, “molar-root incisor malformation”, “root malformation”, “root development”, “tooth roots”, “abnormalities”. Síntese dos dados: Quinze artigos, na maioria série de casos, foram incluídos. Em geral, a história médica revelou complicações clínicas durante a gestação e/ou primeiros anos de vida. As características clínicas incluíram defeitos de esmalte dentário na região cervical de incisivos e mobilidadeacentuada de molares e incisivos permanentes. Radiograficamente, observou-se a presença de câmaras pulpares parcialmente obliteradas, raízes de molares e incisivos permanentes curtas, finas e incompletas. Microscopicamente, reportou-se a ocorrência de camada de dentina hipercalcificada, em forma de lente, no interior da câmara pulpar, ao nível da junção cemento-esmalte, denominada de “diafragma cervical mineralizado”. Conclusão: A “Malformação molar-incisivo” é uma anomalia caracterizada por alterações do desenvolvimento radicular, da câmara pulpar e do esmalte em molares e incisivos permanentes. O diagnóstico diferencial inclui Displasia dentinária tipo I e Odontodisplasia regional. Históricos médico e familiar são essenciais para o diagnóstico final, e o tratamento, o qual apesar de não ter protocolo estabelecido requer abordagem multidisciplinar e tratamentos convencionais como exodontia, endodontia, ortodontia e implantes dentários.

This microsurgical clinical study evaluated if teeth that have undergone endodontic retreatment are associated with more dentinal defects than primary root canal-treated teeth.One hundred fifty-five patients who underwent periapical microsurgery treatment in a private practice setting were evaluated. The root ends were resected, and the roots were inspected for the presence of dentinal defects through the surgical operating microscope with the help of a 0.8-mm-diameter light-emitting diode probe light and methylene blue dye. The root canal treatment history (primary vs retreatment) of the teeth was documented and related to the presence or absence of dentinal defects. Bivariate analysis was performed using the chi-square test, and a multivariate analysis was performed using logistic regression to evaluate possible confounding effects of patient age, sex, and tooth location on the association between treatment and the presence of dentinal defects.Of the 155 treated teeth, 33 were excluded (3 fractured and 30 missing treatment history). Of the remaining 122 included teeth, 73 (59.8%) had undergone primary root canal treatment and 49 (40.2%) retreatment. Sixteen teeth (22.5%) of the primary root canal group versus 33 (64.7%) of the retreatment group had dentinal defects. The proportion of retreated teeth with dentinal defects compared with primary treatment was statistically significant (P .001) with a higher proportion of retreated teeth having dentinal defects. In the multivariate analysis, only the type of treatment was statistically significant (P .001).This clinical study showed that root canal-retreated teeth are associated with more dentinal defects than primary root canal-treated teeth.

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.

Type-1 dentine dysplasia is a rare hereditary condition, associated with an abnormality in dentine formation. Deceptively, teeth have the clinical appearance of normality, however, radiographically, a different picture is seen; with multiple periapical radiolucencies associated with non-carious, unrestored teeth. This article reports the diagnostic and management challenges associated with dentine dysplasia in adults. CPD/Clinical Relevance: Early diagnosis and preventive advice within primary care are imperative in the long-term outcomes.

ObjectivesIn this article, we report two cases of type I dentin dysplasia (DD): the first case is subtype “d” DD and second case is severe form of DD type I, which presents all of four subtypes and describes characteristics and the management of this type of teeth anomalies.Materials and methodsDentin dysplasia is a rare abnormality of dentin that involves both primary and permanent dentition. The normal shape of crown, short roots or its total absence, pulp chamber obliteration, crescent-shaped pulp, pulp stone, and periapical radiolucencies are the characteristics of DD type I.ConclusionThere are many problems in the management of a patient with DD. Depending on root development and pulp canal situation, endodontic treatment and tooth extraction are applied in teeth with pulp necrosis and periapical abscess. The prognosis of DD largely depends upon the age at which the diagnosis is made, and the quality of the treatment provided.How to cite this articleElmi R Z, Azimi S, Jafari S, Farzanegan A. Dentin Dysplasia Type I: Report of Two Cases. Int J Experiment Dent Sci 2017;6(1):44-47.

Background Molar-incisor malformation (MIM) is a recently described dental abnormality. While MIM mimics dentin dysplasia, it presents in a localized pattern. Furthermore, it is speculated that MIM is caused by significant early-life medical history. Aim The purpose of this study is to present a series of MIM cases and compare the findings with the literature. Design An extensive search of all published cases of MIM in the English-language literature was conducted. Additionally, an institutional review board-approved retrospective search was performed within the University of Florida oral pathology biopsy service archives. Radiographic consultation cases were also included. Cases lacking radiographs were excluded. Results Seventy-nine cases were identified in the literature, and eight cases were identified in our retrospective search. All but one case involved the permanent first molars. The average age at diagnosis was 9 years. Many patients reported significant early-life medical histories. Conclusions MIM usually affects the permanent first molars and may be linked to early-life medical conditions or interventions. Oral healthcare providers, especially paediatric dentists, should be aware of MIM to avoid misdiagnosing it as dentin dysplasia. Long-term follow-up studies with thorough medical history documentation are essential to understand the pathogenesis and aetiology and to create treatment guidelines.

Poly(ADP-ribose) polymerase (Parp)-1 catalyzes polyADP-ribosylation using NAD+ and is involved in the DNA damage response, genome stability, and transcription. In this study, we demonstrated that aged Parp-1&minus, /&minus, mouse incisors showed more frequent dental dysplasia in both ICR/129Sv mixed background and C57BL/6 strain compared to aged Parp-1+/+ incisors, suggesting that Parp-1 deficiency could be involved in development of dental dysplasia at an advanced age. Computed tomography images confirmed that dental dysplasia was observed at significantly higher incidences in Parp-1&minus, mice. The relative calcification levels of Parp-1&minus, incisors were higher in both enamel and dentin (p &lt, 0.05). Immunohistochemical analysis revealed (1) Parp-1 positivity in ameloblasts and odontoblasts in Parp-1+/+ incisor, (2) weaker dentin sialoprotein positivity in dentin of Parp-1&minus, incisor, and (3) bone sialoprotein positivity in dentin of Parp-1&minus, incisor, suggesting ectopic osteogenic formation in dentin of Parp-1&minus, incisor. These results indicate that Parp-1 deficiency promotes odontogenic failure in incisors at an advanced age. Parp-1 deficiency did not affect dentinogenesis during the development of mice, suggesting that Parp-1 is not essential in dentinogenesis during development but is possibly involved in the regulation of continuous dentinogenesis in the incisors at an advanced age.

A amelogênese imperfeita compreende um grupo de condições com alterações do desenvolvimento na estrutura do esmalte dentário que afetam a dentição decídua e permanente e as principais implicações clínicas são: estética prejudicada, hipersensibilidade dentinária, dificuldade na higiene bucal, cáries recorrentes, inflamação gengival e perda da dimensão vertical. O tratamento varia de acordo com o tipo e a gravidade do caso, a fase do desenvolvimento dentário, o nível socioeconômico e as expectativas e anseios do paciente, podendo ser levados anos até o estabelecimento do tratamento definitivo. O presente trabalho tem como objetivo relatar e descrever as etapas clínicas de um caso de amelogênese imperfeita com restabelecimento da estética e função através de laminados cerâmicos ultrafinos, confeccionados em IPS e.max (IvoclarVivadent), com acompanhamento de um ano após a cimentação das restaurações. Conclui-se que os laminados cerâmicos podem ser uma alternativa interessante para o tratamento definitivo de casos mais brandos de amelogênese imperfeita, com otimização dos resultados estéticos e recuperação da autoestima do paciente.Descritores: Amelogênese Imperfeita; Facetas Dentárias; Estética Dentária.ReferênciasAldred MJ, Savarirayan R, Crawford PJ. Amelogenesis imperfecta: a classification and catalogue for the 21st century. Oral Dis. 2003;9(1):19-23.Bäckman B, Holm AK. Amelogenesis imperfecta: prevalence and incidence in a northern Swedish county. Community Dent Oral Epidemiol. 1986; 14(1):43-7.Chaudhary M, Dixit S, Singh A, Kunte S. Amelogenesis imperfecta: Report of a case and review of literature. J Oral Maxillofac Pathol. 2009;13(2):70-7.Witkop CJ Jr. Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: problems in classification. J Oral Pathol. 1988;17(9-10):547-53.Ooya K, Nalbandian J, Noikura T. Autosomal recessive rough hypoplastic amelogenesis imperfecta. A case report with clinical, light microscopic, radiographic, and electron microscopic observations. Oral Surg Oral Med Oral Pathol. 1988;65(4):449-58.Witkop CJ Jr, Kuhlmann W, Sauk J. Autosomal recessive pigmented hypomaturation amelogenesis imperfecta. Report of a kindred. Oral Surg Oral Med Oral Pathol. 1973;36(3):367-82.Crawford PJ, Aldred M, Bloch-Zupan A. Amelogenesis imperfecta. Orphanet J Rare Dis. 2007;2:17.Seow WK. Clinical diagnosis and management strategies of amelogenesis imperfectavariants. Pediatr Dent. 1993;15(6):384-93.Yip HK, Smales RJ. Oral rehabilitation of young adults with amelogenesis imperfecta. Int J Prosthodont. 2003 Jul-Aug;16(4):345-9.McDonald S, Arkutu N, Malik K, Gadhia K, McKaig S. Managing the paediatric patient with amelogenesis imperfecta. Br Dent J. 2012;212(9):425-28.Ozturk N, Sari Z, Ozturk B. An interdisciplinary approach for restoring function and esthetics in a patient with amelogenesis imperfecta and malocclusion: a clinical report. J Prosthet Dent. 2004;92(2):112-5.Harley KE, Ibbetson RJ. Dental anomalies--are adhesive castings the solution? Br Dent J. 1993;174(1):15-22.Walter B. Prosthetic rehabilitation of a case of total amelogenesis imperfecta. Actual Odontostomatol. 1991;45(174):213-26. French.Chen C-F, Hu JCC, Estrella MRP, Peters MC, Bresciani E. Assessment of restorative treatment of patients with amelogenesis imperfecta. Pediatr Dent. 2013;35(4):337-42.Ramos AL, Pascotto RC, Iwaki Filho L, Hayacibara RM, Boselli G. Interdisciplinary treatment for a patient with open-bite malocclusion and amelogenesis imperfecta. Am J Orthod Dentofacial Orthop. 2011;139(4 Suppl):S145-53.Pousette Lundgren G, Wickström A, Hasselblad T, Dahllöf G. Amelogenesis imperfecta and early restorative crown therapy: an interview study with adolescents and young adults on their experiences. PLoS One. 2016;11(6):e0156879.Seymen F, Kiziltan B. Amelogenesis imperfecta: a scanning electron microscopic and histopathologic study. J Clin Pediatr Dent. 2002;26(4):327-35.Gilchrist F, Rodd H, Deery C, Marshman Z. Assessment of the quality of measures of child oral health-related quality of life. BMC Oral Health. 2014;14:40.Pousette Lundgren G, Karsten A, Dahllöf G. Oral health-related quality of life before and after crown therapy in young patients with amelogenesis imperfecta. Health Qual Life Outcomes. 2015;13:197.Toksavul S, Ulusoy M, Türkün M, Kümbüloğlu O. Amelogenesis imperfecta: the multidisciplinary approach. A case report. Quintessence Int. 2004;35(1):11-4.Greenfield R, Iacono V, Zove S, Baer P. Periodontal and prosthodontic treatment of amelogenesis imperfecta: a clinical report. J Prosthet Dent. 1992;68(4):572-74.Coffield KD, Phillips C, Brady M, Roberts MW, Strauss RP, Wright JT. The psychosocial impact of developmental dental defects in people with hereditary amelogenesis imperfecta. J Am Dent Assoc. 2005;136(5):620-30.

Dental development is a process by which several genes participate. These genes can be thus characterized as having a multifactorial, or complex, mode of inheritance. The identification of these processes was accelerated by the identification of gene mutations which can explain amelogenesis imperfecta, dentinogenesis imperfecta, and dentin dysplasia. This knowledge has been applied to the study of conditions that are multifactorial in nature such as dental caries (both incipient lesions in enamel and deep lesions extending into dentin), enamel hypoplasia (in particular, molar-incisor hypomineralization), erosive tooth wear, and fluorosis.

Dentin Dysplasia (DD) is a rare autosomal dominant anomaly that disturbs the formation of dentin in primary and/or permanent dentitions. This condition is classified into types I (radicular) and II (coronal) based on radiological findings. A case of DD type I in an 11-year old Iranian boy is presented and the clinical and radiological findings and selected treatment plan are discussed.

Tumoral calcinosis is a rare familial disorder characterized by masses of calcification in periarticular soft tissues. Few reports in the literature have described dental abnormalities in patients with tumoral calcinosis. These dental abnormalities include short bulbous roots, pulp stones, and obliteration of the pulp cavity. In this case report, we describe the dental radiographic findings in a patient with tumoral calcinosis; these findings were similar to dentin dysplasia. Periapical radiolucencies with noncarious teeth were observed in addition to the dental findings previously reported.

Introduction: Turner's hypoplasia is classified as acquired dental dysplasia (dysplasia dentales acquisitae). Turner's hypoplasia is a localized tooth dysplasia. Most often one of the permanent teeth is affected, but a group of adjacent permanent teeth may also be affected. Case Description : The survey was conducted in 2018/2019. It was conducted at the Faculty of Dental Medicine and the clinical offices of Pediatric Dental Medicine, Varna, Bulgaria. In order to conduct the study, we obtained informed consent from the parents of the patient, who is a child at 11 years of age. We have diagnosed Turner's hypoplasia on teeth 11, 12 and 21. The presence of hypoplastic and hypomineralized areas of the enamel of the occlusal 1/3 of the permanent incisor crowns affecting the cutting edges. Tooth 22 is with hypodontia dentis. Discussion : Turner's hypoplasia is an anomaly found in the structure of permanent teeth. Its appearance is variable, although it usually manifests as part of missing or reduced enamel on permanent teeth. Conclusion/clinical significance : A traumatic accident during childhood is the etiological reason for the development of Turner's hypoplasia on permanent teeth 11, 12, 21. Enamel hypoplasia has different variations and studies can be conducted to conclusion of their family habits.

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.

Background Dentinogenesis imperfecta (DGI) is a heritable disorder of dentin. Genetic analyses have found two subgroups in this disorder: DGI type I, a syndromic form associated with osteogenesis imperfecta (OI), and DGI type II, a non-syndromic form. The differential diagnosis between types I and II is often challenging. Thus, the present cross-sectional study had two aims: to (i) investigate the prevalence and incidence of DGI type II among Swedish children and adolescents and (ii) search out undiagnosed cases of DGI type I by documenting the prevalence of clinical symptoms of OI in these individuals. We invited all public and private specialist pediatric dental clinics (n = 47) in 21 counties of Sweden to participate in the study. We then continuously followed up all reported cases during 2014−2017 in order to identify all children and adolescents presenting with DGI type II. Using a structured questionnaire and an examination protocol, pediatric dentists interviewed and examined patients regarding medical aspects such as bruising, prolonged bleeding, spraining, fractures, hearing impairment, and family history of osteoporosis and OI. Joint hypermobility and sclerae were assessed. The clinical oral examination, which included a radiographic examination when indicated, emphasized dental variables associated with OI. Results The prevalence of DGI type II was estimated to be 0.0022% (95% CI, 0.0016–0.0029%) or 1 in 45,455 individuals. Dental agenesis occurred in 9% of our group. Other findings included tooth retention (17%), pulpal obliteration (100%), and generalized joint hypermobility (30%). Clinical and radiographic findings raised a suspicion of undiagnosed OI in one individual, a 2-year-old boy; he was later diagnosed with OI type IV. Conclusions These results show a significantly lower prevalence of DGI type II than previously reported and point to the importance of excluding OI in children with DGI. Electronic supplementary material The online version of this article (10.1186/s13023-018-0887-2) contains supplementary material, which is available to authorized users.

Dentin dysplasia type I (DD-I) is a rare human dentin disorder that may affect both the primary and permanent dentitions. The teeth present crowns with normal morphology but short or absent roots. Pulp chamber obliteration and early exfoliation of primary teeth are also observed. We describe herein the typical and atypical features of DD-I presented by a 6-year-old patient, the diagnostic rationale and assessment emphasizing the histological and scanning electron microscopic analysis and the therapeutic approach. The DD-I diagnosis in patients in the mixed dentition period is challenging, especially when only some teeth are affected.

Hereditary dentin disorders include dentinogenesis imperfecta (DGI) and dentin dysplasia (DD), which are autosomal dominant diseases characterized by altered dentin structure such as abnormality in dentin mineralization and the absence of root dentin. Shields classified DGI into three subgroups and DD into two subtypes. Although they are all hereditary dentin diseases, they do not share the same causative genes. To date, the pathogenic genes of DGI type I, which is considered a clinical manifestation of syndrome osteogenesis imperfecta, include COL1A1 and COL1A2. Mutations of the DSPP gene, which encodes the dentin sialophosphoprotein, a major non-collagenous protein, are responsible for three isolated dentinal diseases: DGI-II, DGI-III, and DD-II. However, DD-I appears to be special in that researchers have found three pathogenicity genes-VPS4B, SSUH2, and SMOC2-in three affected families from different countries. It is believed that DD-I is a genetically heterogeneous disease and is distinguished from other types of dentin disorders. This review summarizes the DD-I literature in the context of clinical appearances, radiographic characteristics, and functions of its pathogenic genes and aims to serve clinicians in further understanding and diagnosing this disease.

BACKGROUND: Dentinogenesis imperfecta (DI) is a rare debilitating hereditary disorder affecting dentin formation and causing loss of the overlying enamel. Clinically, DI sufferers have a discolored and weakened dentition with an increased risk of fracture. The aims of this study were to assess genotype-phenotype findings in three families with DI-II with special reference to mutations in the DSPP gene and clinical, histological, and imaging manifestations.METHODS: Nine patients participated in the study (two from family A, four from family B, and three from family C). Buccal swab samples were collected from all participants and extracted for genomic DNA. Clinical and radiographic examinations had been performed longitudinally, and the dental status was documented using photographic images. Four extracted and decalcified tooth samples were prepared for histological analysis to assess dysplastic manifestations in the dentin. Optical coherence tomography (OCT) was applied to study the health of enamel tissue from in vivo images and the effect of the mutation on the function and structure of the DSPP gene was analyzed using bioinformatics software programs.RESULTS: The direct DNA sequence analysis revealed three distinct mutations, one of which was a novel finding. The mutations caused dominant phenotypes presumably by interference with signal peptide processing and protein secretion. The clinical and radiographic disturbances in the permanent dentition indicated interfamilial variability in DI-II manifestations, however, no significant intrafamilial variability was observed.CONCLUSION: The different mutations in the DSPP gene were accompanied by distinct phenotypes. Enamel defects suggested deficit in preameloblast function during the early stages of amelogenesis.

Dentin dysplasia, a rare hereditary disorder of dentin formation, is characterized by normal enamel but atypical dentin formation along with abnormal pulpal morphology. It is inherited as an autosomal dominant trait. It has been divided into two clinical entities: type I (radicular) and type II (coronal). Early diagnosis and initiation of effective regular dental treatments may help the patients with this condition to delay or prevent the loss of the entire dentition and help them in cope up with edentulous state in early ages. The condition undoubtedly has a negative impact on the physical and psychological well-being of the affected individual. Numerous factors have to be considered during the prosthetic rehabilitation of patients with dentin dysplasia. Treatment protocol varies according to clinical case. Although literature reports suggest general guidelines for treatment planning, the present case report describes a full mouth rehabilitation of an 8-year-old female patient with dentin dysplasia. How to cite this article: Khandelwal S, Gupta D, Likhyani L. A Case of Dentin Dysplasia with Full Mouth Rehabilitation: A 3-year Longitudinal Study. Int J Clin Pediatr Dent 2014;7(2): 119-124.

Dentin dysplasia is a rare hereditary disturbance characterized by a dental anomaly of the dentin layer. The etiology is unclear, and this rare hereditary disturbance affects approximately one person in every 100,000. Dentin dysplasia is classified into two types, radicular dentin dysplasia as type I and coronal dentin dysplasia as type II. The characteristic clinical findings of dentin dysplasia type I are normal appearance of the crown and hypermobility of teeth. The radiographic findings are obliteration of all pulp canals, short, blunted and malformed or absent roots. Dentin dysplasia type II as coronal dentin dysplasia shows similar clinical features with dentinogensis imperfecta. This report shows a case of dentin dysplasia type I affecting one family except the father. The clinical, radiographic and histopathologic findings of this family are presented. Dentin dysplasia type I is difficult to diagnose unless dentist performs radiographic examination. If the affecting patient does not get regular dental care, dental abscesses or cysts may form spontaneously without caries. In this regard, early diagnosis is important to prevent premature loss of dentition.

Dentin dysplasia (DD) is an uncommon developmental disturbance affecting dentin, resulting in enamel with atypical dentin formation and abnormal pulpal morphology. Type I (radicular) and Type II (coronal) are the two types of DD. Type I is more common, and both types include single or multiple teeth in primary and permanent dentition. Combinations of both types have also been described in literature. Four distinct forms of Type I and one form of Type II were identified. This case report documents one such rarity of DD in an 11-year-old female with clinical and radiographical findings and management aspects.

Dentin dysplasia is a rare inherited autosomal dominant disorder characterized by rootless teeth. We hereby report a case of dentin dysplasia type 1b with typical radiographic findings: short and blunt roots, pulpal obliteration, horizontal/crescent shaped radiolucencies in pulp chambers, and multiple periapical radiolucencies. However, the present case did not show the autosomal dominant pattern of inheritance and the patient also exhibited concurrent dental fluorosis, transposition of 13 and 14, and multiple cusps in maxillary first molars. Moreover, on careful review of previously documented cases of radiographs of dentin dysplasia, the horizontal/crescent shaped radiolucencies in pulp chambers are a rare finding, which is characteristically seen in the present case.

Dentin dysplasia (DD) type I is a hereditary disturbance in dentin formation. In this anomaly, the teeth generally appear clinically unremarkable with normal crowns; and radiographically it is characterized by normal appearing crowns, absence or severe restriction of root formation, obliterated pulp chambers, and periapical radiolucencies without an obvious cause. Clinically, the teeth can be poorly aligned, drifting and mobile, and can prematurely exfoliate due to abnormal root formation. This condition is rarely encountered in dental practice. Little is known about the specific treatment of this disorder and management of patients with DD has presented with problems. This report documents a case of DD type I in a 19-year-old male and the clinical, radiographic, and histopathologic findings of this condition and treatment are described.

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. A displasia dentinária é uma alteração do desenvolvimento da dentina rara, de origem autossômica dominante, o qual é geralmente dividida em 2 tipos principais baseados na aparência clínica e radiográfica dos tecidos dentais afetados: tipo I, que afeta a porção radicular e o tipo II, que afeta a porção coronária do dente. O objetivo deste trabalho é relatar o caso de um paciente de 10 anos e 8 meses de idade com ambas alterações clássicas e atípicas da displasia dentinária do tipo I na dentição permanente. Alguns dentes da arcada inferior foram acometidos, apresentando-se com coroas clinicamente normais, mobilidade dental de moderada a severa e raízes curtas ou ausentes, porém sem evidência de obliteração das câmaras pulpares e de lesões periapicais. As características clínicas e radiográficas observadas neste paciente foram diferentes daquelas relatadas na literatura, o que sugere que esta possa ser uma variação da expressão da displasia dentinária do tipo I.

Hypophosphatasia is caused by mutations of the tissue-non-specific alkaline phosphatase ( TNSALP) gene with deficiency of dentin structure. The aim of this study was to examine whether TNSALP mutation in dental pulp cells contributes to dentin dysplasia in hypophosphatasia. Mutation analysis showed that compound heterozygous mutations of TNSALP were identified in three hypophosphatasia patients, including 3 novel mutation sites. Exfoliated teeth from the patients showed abnormal dentin mineralization and loss of cementum, as assessed by ground sections and scanning electron microscope analysis. Dental pulp cells isolated from one of the patients showed a significantly reduced TNSALP activity and mineralization capacity when compared with those in dental pulp cells from the unaffected individuals. Our results suggested that dentin dysplasia in hypophosphatasia may be associated with the decreased mineralization ability of dental pulp cells.

Dentin dysplasia(DD) is a rare autosomal dominant disorder associated with disturbance of the dentin. While the crowns appear clinically normal, on radiography, the pulp spaces appear partially or completely obliterated, with short blunted roots, and multiple periapical radiolucencies affecting the apparently sound teeth. Clinical signs include spontaneous abscess formation or increased tooth mobility which can lead to exfoliation. DD can therefore have a significant impact on the patient’s dentition, and treatment is often challenging. Shields’ classification of dentin disorders has been recently criticised for failing to consider differential variations and expressions of these disorders. This paper describes a case of a 23-year-old woman with previously undiagnosed DD, who presented with clinical and histological features belonging to several of these diseases, thus highlighting the potential diagnostic challenges faced with Shields’ classification.

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.

Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe mobility of teeth and spontaneous dental abscesses or cysts. Radiographic analysis shows obliteration of pulp chambers, short, blunted and malformed or absent roots and periapical radiolucencies of non carious teeth. We present a case of dentin dysplasia type I; the clinical, radiographic and histopathologic findings of this condition & treatment are described.

Dentinogenic ghost cell tumor (DGCT) is a rare neoplasm, representing 1.9% to 2.1% of all odontogenic tumors. Few cases of DGCT have been reported and only 11 show no bone involvement. A rare case of peripheral DGCT is reported, located in the anterior mandible of a 45-year-old man. The patient presented a slow painless growth in the canine region of an edentulous mandible. Radiographically, no bone involvement was registered. The lesion was enucleated and microscopically characterized by islands of epithelial cells showing ameloblastomalike features in fibrous tissue. Dysplasic dentin and ghost cells were frequently observed. Areas showing a connection between tumor cells and the overlying mucosa were also identified. Immunohistochemical analysis demonstrated positivity for pan-cytokeratin, cytokeratin-14, and 2 neural markers. Denditric cells (Langerhans cells and melanocytes) were identified inside tumoral islands. A rare case of peripheral DGCT is reported, with immunohistochemical analysis and a review of the English literature.

Dentin defects represent a cluster of relatively rare heritable disorders affecting the formation and mineralization of dentin. Major clinical features include loss of enamel with extreme wear of exposed dentin, opalescent tooth discoloration, short roots with pulpal calcification and increased tooth mobility, and frequent periapical radiolucencies. Historically, these inherited conditions have been classified by Shields et al. (1) as either dentinogenesis imperfecta (DGI) types I, II and III, or dentin dysplasia (DD) types I and II. DGI type I is now universally designated as osteogenesis imperfecta with dentinogenesis imperfecta (OI/DGI) and is caused by type I collagen mutations. Among the genes expressing the non-collagenous proteins in dentin, only the DSPP gene has been implicated in the etiology of DD type II and all DGI types, suggesting that heritable dentin defects can be viewed as a continuum rather than as distinct disease entities. Dental treatment strategies should aim to preserve the affected dentitions by protecting teeth from wear, restoring them to compliance with the specific structural characteristics of the defective dentin, and anticipating early tooth loss. Endodontic treatment of the affected teeth is very challenging because of the peculiar pulp morphology and most often has poor prognosis. If conventional therapy is not an option, periapical curettage and retrograde filling is a possible alternative, except in the case of teeth with short roots.

This paper presents a case of dentin dysplasia, a rarely reported disorder that is also associated with pyogenic granuloma in a 12-year-old girl. The case presented as excessively mobile teeth that appeared radiographically as rootless teeth and also as a soft tissue lesion in the right maxillary anterior region.

Dentin dysplasia type I (DDI) is a rare hereditary disturbance of dentin formation. It is characterized by clinically normal-appearing crowns; obliteration of pulp chambers; and short, blunted and malformed roots that are commonly associated with periodontal attachment loss (PAL). In this context, we report three cases within a family with similar clinical and radiographic features of DDI but with differing microbiologic and periodontal conditions.A 42-year-old white female and her two daughters (25 and 10 years of age) presented with a diagnosis of DDI. Probing depth (PD), clinical attachment level (CAL), visible plaque, and bleeding on probing (BOP) were recorded. Subgingival biofilm samples were randomly collected and analyzed by checkerboard DNA-DNA hybridization.The mother presented 34.9% of sites with PDor =4 mm, 41.3% of sites with CALor =4 mm, and 57% of sites with BOP; both daughters presented no sites with PD or CAL3 mm and10% of sites with BOP. Microbiologic analysis detected Gemella morbillorum, Neisseria mucosa, and Staphylococcus aureus inor =50% of the mother's samples. The daughters showed high levels (10(4) bacterial cells) of some periodontopathic bacteria, including members of the red (Porphyromonas gingivalis) and orange (Fusobacterium periodonticum and F. nucleatum polymorphum) complexes and beneficial species of the yellow (Streptococcus gordonii) and purple (Veillonella parvula) complexes. The mother presented high mean levels only for four tested species (N. mucosa, Prevotella melaninogenica, Treponema denticola, and V. parvula).A combination of radiographs, microbiologic analysis, and preventive professional monitoring care is important to avoid PAL and to provide oral health in patients with DDI.

Dentin, the most abundant tissue in teeth, is produced by odontoblasts, which differentiate from mesenchymal cells of the dental papilla. Dentinogenesis is a highly controlled process that results in the conversion of unmineralized predentin to mineralized dentin. By weight, 70% of the dentin matrix is mineralized, while the organic phase accounts for 20% and water constitutes the remaining 10%. Type I collagen is the primary component (>85%) of the organic portion of dentin. The non-collagenous part of the organic matrix is composed of various proteins, with dentin phosphoprotein predominating, accounting for about 50% of the non-collagenous part. Dentin defects are broadly classified into two major types: dentinogenesis imperfectas (DIs, types I–III) and dentin dysplasias (DDs, types I and II). To date, mutations in DSPP have been found to underlie the dentin disorders DI types II and III and DD type II. With the elucidation of the underlying genetic mechanisms has come the realization that the clinical characteristics associated with DSPP mutations appear to represent a continuum of phenotypes. Thus, these disorders should likely be called DSPP-associated dentin defects, with DD type II representing the mild end of the phenotypic spectrum and DI type III representing the severe end.

Background Dentin dysplasia type I (DD-I) is a rare autosomal dominant hereditary disorder which seriously affects the root development of teeth, causing spontaneous tooth loss (in teenagers). At present, the study of DD-I focuses on familial and phenotypic analyses and reports regarding the ultrastructural study of DD-I are few. The purpose of this study was to clarify and discuss the clinical, histopathological, and ultrastructural features of the dentin defects in DD-I. In addition, the study further explores the root development and provides clues for uncovering virulent genes associated with the disease. Methods We recruited 31 members of a four-generation Chinese family, including eleven with dentin defects. Four permanent teeth and four deciduous teeth were obtained from individuals affected by DD-I. At the same time, two caries-free like-numbered permanent teeth and deciduous teeth served as controls, respectively. Analyses of these teeth were carried out using stereomicroscopy, light microscopy, and scanning and transmission electron microscopy, respectively. Results Similar to previous reports, extracted teeth showed typical histopathological and ultrastructural features of DD-I and teeth had short roots with obliterated pulp chambers. Furthermore, several novel discoveries were found in teeth affected by DD-I, including; (1) thinner dentin; (2) larger scalloped dentinoenamel junctions; (3) teardrop-shaped lacunae in the enamel; (4) rodless enamel and (5) irregular collagen fibers. Conclusions The results exhibited defined features of DD-I in the family and further confirmed that abnormal dentin structure affected both the deciduous and permanent dentitions. In addition, these findings may contribute to a better understanding of the pathogenesis of DD-I as well as aid in the subclassification of this disease.

An unusual case of generalised pulpal calcifications (pulp stones) with normal clinical crowns is reported in a 13-year-old boy. Radiographic examination revealed pulp stones in the single rooted and premolar teeth, situated at the midroot level, with the roots bulging around them. The apical portion of the roots, periodontal ligament space, and surrounding bone had a normal radiographic appearance, apart from the upper premolars, and no periapical pathology was discernible. The upper premolars exhibited considerably shortened roots. No medical, dental, or family history was found to be contributory. Reviewing the literature revealed similar cases, but with differing diagnoses including dentine dysplasia (DD) or idiopathic cases. This report suggests either a variation of DD or possibly a new nonsyndromic dentine defect, and highlights the difficulties in establishing a definitive diagnosis by traditional methods. The recent discovery that mutation of the bicistronic dentine sialophosphoprotein gene (DSPP) is involved in DD may provide solutions to this problem.