Three-dimensional evaluation of dental characteristics in patients with Cleidocranial dysplasia.

BACKGROUND: Cleidocranial dysplasia (CCD) is an autosomal dominant hereditary disorder. Besides skeletal abnormalities, CCD is often associated with dental complications, such as multiple supernumerary teeth and permanent teeth impaction or delayed eruption. METHODS: Supernumerary teeth of axial, sagittal and coronal CBCT view was characterized in detail and 3D image reconstruction was performed. Number and location of teeth, morphology of supernumerary teeth, positional relationship between supernumerary and adjacent permanent teeth, direction of supernumerary teeth in CCD patients were analyzed. RESULTS: The mean age of the 3 CCD patients in this study was 16.7 years. Among 36 supernumerary teeth, the majority of them were identified as apical side located and lingual side located. Normal orientation was the most common type in this study, followed by sagittal orientation, and horizontal orientation. Horizontal orientation teeth were all distributed in the mandible. Supernumerary teeth exhibited significantly shorter crown and dental-root lengths, as well as smaller crown mesiodistal and buccolingual diameters (P < 0.01). There was no difference in the number of supernumerary teeth between the maxilla and mandible, and the premolars region had the largest number of supernumerary teeth and the incisor region had the smallest number. CONCLUSIONS: This study compares number and location of teeth, morphology of supernumerary teeth, positional relationship between supernumerary and adjacent permanent teeth and direction of supernumerary teeth, this study also provides a reference for the comprehensive evaluation of CCD patients before surgery.

The ectopic mandibular canines can start tooth formation in three different locations: a case series study based on single orthopantomograms from 47 individuals.

INTRODUCTION: A former study on orthopantomograms from young children with abnormal dental development (not canine ectopia) demonstrated that the tooth bud of the mandibular canine, compared to a stable longitudinal canine axis, could be located normally, anteriorly or posteriorly, with close relation to the first premolar. AIM: The aim of the present study is to analyse on orthopantomograms if the canine axis can demonstrate where the ectopic mandibular canine started tooth formation. MATERIALS: The material consists of orthopantomograms with ectopic mandibular canines and presence of primary mandibular canines from 47 cases (29 cases 9-21 years old and 18 cases with unknown ages). The primary canines demonstrated from minor apical resorption to more severe apical resorption. METHODS: Based on canine maturity, location of the canine axes and the interrelationships between the roots of the permanent canine and first premolar, the location from where the canine started tooth formation was determined. Canine maturity. Maturity stage below half root length and maturity stage above half root length revealed that 11 ectopic canines had less than half root length and 36 cases more than half root length. Canine axes. The canine axis, through the length of the primary canines Ax, is inserted on drawings of the orthopantomograms using the tracing programme Inkscape®. Interrelationship between roots. By visual inspection, the distance between the canine and first premolar was designated close distance, normal distance and extended distance. RESULTS: The results are divided into 3 groups. Group 1: The initial site of the permanent ectopic canine is located within the canine axis (6 cases). Group 2: The initial site of the permanent ectopic canine is located posterior to the canine axis (36 cases). Group 3: The initial site of the permanent ectopic canine is located anterior to the canine axis (5 cases). CONCLUSION: The study explained that the canine axis could divide cases of ectopic canines into three groups according to the location from where tooth formation starts. For getting closer to the pattern of the ectopic canine eruption, it is necessary to analyse series of orthopantomograms taken from the same individual over several years.

Molar-root incisor malformation - a systematic review of case reports and case series.

OBJECTIVES: Molar-root incisor malformation (MRIM) is a seldom reported condition characterised by disturbances in root development of first permanent molars. This systematic review aimed to collate the clinical characteristics of individuals diagnosed with MRIM. MATERIALS AND METHODS: A systematic search strategy using PubMed, Embase, Web of Science, and SCOPUS databases was performed through to March 2023. Inclusion criteria were case reports or case series including a diagnosis consistent with MRIM. Critical appraisal for all included studies utilised the Joanna Briggs Institute (JBI) critical appraisal checklist for case reports and case series and collation of clinical characteristics was performed in JBI System for the Unified Management, Assessment and Review of Information program. RESULTS: The search identified 157 studies from which 35 satisfied the inclusion criteria. After full-text review, a total of 23 papers described the MRIM dental anomaly and were included in this paper. A total of 130 reported cases were retrieved, with age ranging 3-32 years, and males affected 1.16:1 females. Presence of neurological conditions, premature birth history, medication, and surgery within first years of life were synthesised and described. CONCLUSIONS: The aetiology of MRIM is yet to be determined but epigenetic changes from significant medical history in the first years of life are likely to influence the development of this root malformation. First permanent molars were most commonly affected, but clinicians should be aware that permanent central incisors, primary teeth and other permanent teeth may also be affected.

Multidisciplinary management of a tooth with severe root dilaceration and enamel hypoplasia: a case report.

Dental trauma in primary teeth can cause irreversible changes in the development of permanent tooth germs, including enamel hypoplasia, crown dilaceration, and root dilaceration. This article discusses multidisciplinary treatment of enamel hypoplasia and root dilaceration in the maxillary left central incisor of an 11-year-old girl. A 10-year follow-up is reported to demonstrate the long-term clinical outcomes. At the initial presentation, the patient's mother reported that the child had an accident at the age of 2 years, resulting in intrusive luxation of the primary maxillary left central incisor. After the accident, the patient was monitored for eruption of the permanent successor tooth, and different approaches were proposed during each period of the patient's development on the basis of the clinical diagnosis of root dilaceration and enamel hypoplasia. The crown was restored with composite resin, and the root defect was restored with resin-modified glass ionomer cement. After 10 years, the clinical outcomes highlight that the multidisciplinary approach was successful in preserving the natural tooth with good periodontal health conditions.

Radix em molares decíduos inferiores: revisão sistemática com metanálise / Radix in lower primary molars: systematic review with meta-analysis

Objective: To estimate the prevalence of three roots in deciduous mandibular molars. Methodology: Electronic searches were carried out in PubMed and Scopus to identify cross-sectional studies published up to September 2023. The Joanna Briggs Institute tool was used to critically appraise the studies. STATA 16.0 was used to generate risk of bias figures and perform the meta-analysis. Results: Eighteen studies evaluating 9,067 patients (8,969 first molars and 10,765 second molars) were included in this review. The overall prevalence of radix in mandibular deciduous molars was 9.61% (3.67% for first molars and 18.72% for second molars). The prevalence rate of teeth diagnosed using Cone Beam Computed Tomography (CBCT) was similar to the diagnoses made using conventional radiographic techniques together (periapical, interproximal and panoramic). Final considerations: Lower deciduous molars with three roots have a prevalence of almost 10%, with a higher prevalence in second molars. The diagnosis of this morphological alteration can be made using conventional radiographic techniques, but the use of CBCT is recommended.(AU)

Objetivo: estimar a prevalência de três raízes em molares inferiores decíduos. Metodologia: foram realizadas buscas eletrônicas na PubMed e Scopus para identificar estudos transversais publicados até setembro/2023. Para a avaliação crítica dos estudos foi utilizada a ferramenta do Instituto Joanna Briggs. STATA 16.0 foi usado para gerar figura do risco de viés e realizar a metanálise. Resultados: dezoito estudos que avaliaram 9.067 pacientes (8.969 primeiros molares e 10.765 segundos molares) foram incluídos nesta revisão. A prevalência global de radix em molares decíduos inferiores foi de 9,61% (3,67% para primeiros molares e 18,72% para segundo molares). A taxa de prevalência de dentes com diagnóstico através de Tomografia Computadorizada Cone Beam (TCCB) foi semelhante aos diagnósticos realizados pelas técnicas radiográficas convencionais em conjunto (periapical, interproximal e panorâmica). Considerações finais: os molares decíduos inferiores com três raízes têm uma prevalência de quase 10%, com maior prevalência em segundo molares. O diagnóstico desta alteração morfológica pode ser feito através das técnicas radiográficas convencionais, porém recomenda-se a utilização de TCCB.(AU)

The design and clinical application of a new appliance to treat impacted maxillary central incisors.

Maxillary central incisor impaction is one of the most common types of dental anomalies in children. Treatment of impacted central incisors is complicated and challenging given the position of the impacted central incisors, root development, and the complexity of the crown eruption direction. This study aimed to describe the use of a new multifunctional appliance for the treatment of impacted maxillary central incisors. This article reports the use of a novel appliance for the treatment of impacted maxillary central incisors. We describe the cases of two young patients with labial horizontally impacted maxillary central incisors. Both patients were treated using this novel appliance. Therapeutic effects were evaluated by comparing the pretreatment results, posttreatment cone-beam computed tomography images, and posttreatment clinical examination results. At the end of the treatment period using the novel appliance, the impacted central incisors had successfully been properly aligned in the dental arch, and the tooth roots had not resorbed. Both patients exhibited good dental alignment, with restored function and acceptable aesthetics. This article demonstrates that the new appliance was comfortable, convenient, safe, and effective in the treatment of impacted maxillary central incisors and that its clinical use should be promoted in the future.

Multi-disciplinary treatment of severe palatal radicular groove of maxillary lateral incisor: A case report and literature review. / 上颌侧切牙重度畸形舌侧沟多学科联合治疗1ä¾‹å¹¶æ–‡çŒ®å¤ä¹ .

Palatal radicular groove is a developmental malformation of maxillary incisors, lateral incisors in particular, which often causes periodontal destruction. This paper reports a case of combined periodontal-endodontic lesions induced by palatal radicular groove, which was initially misdiagnosed as a simple periapical cyst. After root canal therapy and periapical cyst curettage, the course of disease was prolonged, resulting in the absence of buccal and maxillary bone plates in the affected tooth area. After the etiology was determined, the affected tooth was extracted and guide bone tissue regeneration was performed at the same time, followed by implantation and restoration at the later stage, leading to clinical cure. The palatal radicular groove is highly occult, and the clinical symptoms are not typical. If the abscess of the maxillary lateral incisor occurs repeatedly, and the abscess of the maxillary lateral incisor has not been cured after periodontal and root canal treatment, cone-beam computed tomographic and periodontal flap surgery should be considered.

Multi-disciplinary treatment of severe palatal radicular groove of maxillary lateral incisor: A case report and literature review / 中南大学学报(医学版)

Palatal radicular groove is a developmental malformation of maxillary incisors, lateral incisors in particular, which often causes periodontal destruction. This paper reports a case of combined periodontal-endodontic lesions induced by palatal radicular groove, which was initially misdiagnosed as a simple periapical cyst. After root canal therapy and periapical cyst curettage, the course of disease was prolonged, resulting in the absence of buccal and maxillary bone plates in the affected tooth area. After the etiology was determined, the affected tooth was extracted and guide bone tissue regeneration was performed at the same time, followed by implantation and restoration at the later stage, leading to clinical cure. The palatal radicular groove is highly occult, and the clinical symptoms are not typical. If the abscess of the maxillary lateral incisor occurs repeatedly, and the abscess of the maxillary lateral incisor has not been cured after periodontal and root canal treatment, cone-beam computed tomographic and periodontal flap surgery should be considered.

Cicatrização e prognóstico no longo prazo de fraturas radiculares transversais pós-traumáticas: estudo clínico longitudinal / Healing and long-term prognosis of root fractured permanent teeth: a retrospective longitudinal study

Fraturas radiculares são lesões traumáticas dento-alveiolares (LTDA) complexas que afetam dentina, cemento, polpa e o ligamento periodontal, sendo frequente a ocorrência de lesões concomitantes ao fragmento coronário. São lesões relativamente raras com frequências relatadas variando entre 1,2% e 7% das LTDA na dentição permanente, sendo mais comuns em dentes com completo desenvolvimento radicular. O presente estudo consistiu em um levantamento clínico longitudinal retrospectivo para avaliar dados de 87 pacientes, portadores de 104 dentes permanentes com fraturas radiculares transversais, atendidos na Clínica de Traumatismos Dentários da Faculdade de Odontologia da UFMG, durante o período de 1995 a 2022. Com base na análise dos prontuários, avaliou-se o padrão radiográfico de cicatrização na linha de fratura, seguindo os critérios estabelecidos por Andreasen e Hjorting-Hansen (1967), em dois momentos: no período inicial após o trauma e ao final do período de acompanhamento. Uma análise de sobrevivência de risco competitivo foi realizada para estimar as taxas dos eventos de cicatrização no período inicial após o trauma, bem como o efeito das variáveis demográficas, clínicas e relacionadas ao tratamento. Os resultados demonstraram um percentual de 51,9% de cicatrização no curto prazo, diagnosticada em até 5 meses após o trauma. A não cicatrização com interposição de tecido de granulação na linha de fratura foi fortemente influenciada pela presença e tipo de lesão concomitante no fragmento coronário. Dentes com fraturas de esmalte e dentina (sHR 24,38, IC 95% [3,16 - 188,3], p = 0,0022), luxações com deslocamentos (sHR 10,58, IC 95% [1,37 - 81,9], p = 0,0240) e subluxações (HR 9,66, IC 95% [1,14 - 81,7], p = 0,0370) apresentaram as maiores taxas de não cicatrização das fraturas radiculares no curto prazo. A taxa de cicatrização no longo prazo foi de 70,1%, a maioria delas com interposição de osso e tecido conjuntivo. A estatística Kappa demonstrou uma concordância global de 67,1% entre as modalidades de cicatrização no período inicial e no longo prazo, especialmente para os padrões tecido mineralizado e tecido conjuntivo/osso.

Root fractures are complex traumatic dental injuries (TDI) affecting dentin, cementum, pulp and supporting tissues, generally associated to concurrent injuries to the coronal fragment. They are uncommon injuries with reported frequencies varying between 1.2% and 7% of all TDI in permanent teeth, being more frequent in teeth with complete root development. In the present study we retrospectively evaluated data from 87 patients with104 root-fractured permanent teeth treated at the Dental Trauma Clinic from the Federal University of Minas Gerais, during the period from 1995 to 2022. Clinical dan radiographic data was collected from patient's records to establish healing patterns in the fracture line, following the criteria established by Andreasen & Hjorting-Hansen (1967), at two moments: in the initial period after the trauma and at the end of the follow-up period. A competing risk survival analysis was performed to estimate rates of healing events in the early period after trauma as well as the effect of demographic, clinical, and treatment-related variables. The results showed a rate of 51.9% of healing in the short term, diagnosed in a maximum median period of 5 months after trauma. Non-healing with interposition of granulation tissue at the fracture line was strongly influenced by the presence and type of concomitant lesion in the coronal fragment. Teeth with associated enamel -dentin fractures (sHR 24.38, 95% CI [3.16 - 188.3], p = 0.0022), luxations with dislocations (sHR 10.58, 95% CI [1.37 - 81.9], p = 0.0240) and subluxations (HR 9.66, 95% CI [1.14 - 81.7], p = 0.0370) had the highest rates of non-healing of root fractures in the short term. The long-term healing rate was 70.1%, most of them with interposition of bone and connective tissue. Kappa statistics demonstrated an overall agreement of 67.1% between short and long-term healing patterns, in special HT and CT+ Bone modalities.

The incidence of radicular groove on maxillary lateral incisors of Saudi population: CBCT evaluation.

BACKGROUND: The radicular groove (RG) is one of the developmental anomalies that is commonly found in maxillary incisors. The formation of radicular groove is initiated around the cingulum and can reach the root at different levels. The incidence of radicular grooves was reported in different countries but there was no published data about the incidence of RG in Saudi Arabia. Therefore, this study aimed to evaluate the incidence of radicular grooves on maxillary lateral incisors in the Saudi population using cone-beam computed tomography (CBCT). METHODS: The dental records of 490 patients (N = 490) with CBCT images of maxillary anterior teeth were screened for inclusion criteria. Then 264 included cases were evaluated independently by two Endodontists. The evaluation was performed on CBCT images in the axial, sagittal, and coronal sections using Planmeca Romexis® software. The following data were recorded for each patient: Patients' age and gender, radicular groove presence or absence, and if it is bilateral or unilateral. The type of radicular groove was recorded according to Gu's classification (type I, II, or III). RESULTS: The incidence rate of radicular grooves in maxillary lateral incisors was 4.9%. RG was found to be unilateral in 61.5% and bilateral in 38.5%. The majority of RG were classified as type I in 69.2%, followed by type II in 15.4%, and type III was found in 15.4%. CONCLUSION: 4.9% of the Saudi population has RG in the upper lateral incisor. This anatomical variation is mostly present as type I on one side only (unilateral).

Endodontic Management of a Fused Mandibular Third Molar with Supernumerary Tooth Using Cone-Beam Computed Tomography: A Case Report.

BACKGROUND Dental fusion is a developmental dental anomaly that clinicians must be aware of and able to identify. It results from the union of 2 adjacent tooth germs affecting the normal dentition and leads to a decrease in the number of permanent teeth. Clinically, the fused teeth show excessive width and irregular anatomy and can complicate any endodontic intervention. CASE REPORT A 26-year-old male patient presented to the clinic with severe pain related to the lower right mandibular area. The dental examination revealed a large tilted and irregular fused third molar with the fourth molar. The endodontic findings suggested a diagnosis of symptomatic irreversible pulpitis with symptomatic apical periodontitis. An intraoral periapical radiograph revealed fused molars with an irregular morphology and a wide mesiodistal width. Cone beam computed tomography (CBCT) was performed after obtaining consent from the patient. The morphology of the involved tooth was obtained in sagittal, coronal, and axial CBCT slices of 0.13 mm. The pulp chamber was continuous, and the number of canals was 5 in the 3-dimensional view before initiating endodontic treatment. After we discussed the options with the patient, he decided to proceed with nonsurgical root canal treatment for the fused molar. A successful management of a fused third molar with a supernumerary tooth was performed, and a 6-month follow-up radiograph showed normal apical tissue with absence of tenderness on percussion or palpation. No clinical swelling or sinus tract swelling was observed. CONCLUSIONS This case report indicates that a proper treatment plan ensures predictable outcomes by the use of all available diagnostic tools.

Multidisciplinary management of a type III palatoradicular groove: an atypical presentation.

The palatoradicular groove is an endodontic anomaly that complicates diagnosis, treatment planning, and prognosis. The extent of this developmental defect dictates the treatment plan. Treatment approaches may vary from simple restoration of the groove to surgical exploration. A palatoradicular groove may lead to pulpoperiodontal complications if not treated precisely. This case report describes a rare presentation of a type III palatoradicular groove in a maxillary lateral incisor that also had an accessory root and periodontal complications.

The critical role of nuclear factor I-C in tooth development.

OBJECTIVES: Nuclear factor I-C (NFIC) plays a critical role in regulating epithelial-mesenchymal crosstalk during tooth development. However, it remains largely unknown about how NFIC functions in dentin and enamel formation. In the present review, we aim to summarize the most recent discoveries in the field and gain a better understanding of the roles NFIC performs during tooth formation. SUBJECTS AND METHODS: Nfic-/- mice exhibit human dentin dysplasia type I (DDI)-like phenotypes signified by enlarged pulp chambers, the presence of short-root anomaly, and failure of odontoblast differentiation. Although loss of NFIC has little effect on molar crown morphology, researchers have detected aberrant microstructures of enamel in the incisors. Recently, accumulating evidence has further uncovered the novel function of NFIC in the process of enamel and dentin formation. RESULTS: During epithelial-mesenchyme crosstalk, the expression of NFIC is under the control of SHH-PTCH-SMO-GLI1 pathway. NFIC is closely involved in odontoblast lineage cells proliferation and differentiation, and the maintenance of NFIC protein level in cytoplasm is negatively regulated by TGF-ß signaling pathway. In addition, NFIC has mild effect on ameloblast differentiation, enamel mineralization and cementum formation. CONCLUSIONS: NFIC plays an important role in tooth development and is required for the formation of dentin, enamel as well as cementum.

Prevalence of Short Root Anomaly in a Group of Puerto Ricans.

OBJECTIVE: To estimate the prevalence of short root anomaly (SRA) in a group of Puerto Ricans at the Advanced Education Program in Orthodontics of the University of Puerto Rico. METHODS: A cross-sectional study was conducted with digital panoramic x-rays of 203 patients (112 females and 91 males; mean age: 17 years) in Puerto Rico. Utilizing the modified Lind's method, 406 maxillary right and left central incisors (1) were measured and evaluated for SRA. Root and crown length and R/C ratio were compared by sex and side. Linear regression was used to evaluate the associations between the R/C ratio and age, sex, and side (right/left). All the statistical analyses were evaluated using significance level of .05 (2). RESULTS: The prevalence of SRA was 0.49%. The average root and crown lengths for the maxillary right and left central incisors were 19.47 and 10.28 mm, respectively. Sex was associated with root and crown length, not with the R/C ratio. No differences were observed in the R/C ratio by the side. There were no significant associations between age, sex, and side and R/C ratio in the multivariate analysis. CONCLUSION: The prevalence of SRA in our population is lower than any reported in the literature. The R/C ratio in our Hispanic population was the highest compared with those of other ethnicities. Neither the sex nor the side of the incisor was associated with the R/C ratio. These findings are contrary to those of prior reports stating that males have higher R/C ratios than females.

Bilateral occurrence of bimaxillary bi-rooted primary canines: a rare case report with review of the literature.

Multiple roots in anterior teeth are very rarely reported in the literature. The occurrence of multi-rooted canine in primary dentition is an infrequent entity. This unusual abnormality is an accidental finding on routine examination. Radiographic examination plays a significant role in the identification of this anomaly. This rare anomaly has been reported very infrequently reported and has not been published in a Saudi Arabia population. Therefore, the purpose of the present case report is to describe a case of an 8-year old Saudi boy presented with the bimaxillary occurrence of bi-rooted primary canine. This report discusses this unique occurrence in the context of the published literature.

Molar-incisor malformation: a narrative review

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.

Characteristic dental pattern with hypodontia and short roots in Fraser syndrome.

Fraser syndrome (FS) is a rare autosomal recessive multiple congenital malformation syndrome characterized by cryptophthalmos, cutaneous syndactyly, renal agenesis, ambiguous genitalia, and laryngotracheal anomalies. It is caused by biallelic mutations of FRAS1, FREM2, and GRIP1 genes, encoding components of a protein complex that mediates embryonic epithelial-mesenchymal interactions. Anecdotal reports have described abnormal orodental findings in FS, but no study has as yet addressed the orodental findings of FS systematically. We reviewed dental radiographs of 10 unrelated patients with FS of different genetic etiologies. Dental anomalies were present in all patients with FS and included hypodontia, dental crowding, medial diastema, and retained teeth. A very consistent pattern of shortened dental roots of most permanent teeth as well as altered length/width ratio with shortened dental crowns of upper incisors was also identified. These findings suggest that the FRAS1-FREM complex mediates critical mesenchymal-epithelial interactions during dental crown and root development. The orodental findings of FS reported herein represent a previously underestimated manifestation of the disorder with significant impact on orodental health for affected individuals. Integration of dentists and orthodontists into the multidisciplinary team for management of FS is therefore recommended.

Dental Manifestations of Ehlers-Danlos Syndromes: A Systematic Review.

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and variable tissue fragility. However, there are limited published data on the dental manifestations of EDS. This review systematically assessed the spectrum of published dental anomalies in various types of EDS. Twenty-four individual case reports/series and 3 longer case-control studies, reporting on a total of 84 individuals with a clinical diagnosis of EDS, were included in the data analysis. The main dental features listed in classical EDS were pulp calcification and localized root hypoplasia. Common dental abnormalities observed in vascular EDS were pulp shape modifications (52.2%), exceeding root length (34.8%), and molar root fusion (47.8%). Dentinogenesis imperfecta is a consistent finding in osteogenesis imperfecta/EDS overlap syndrome. Data on dental manifestations in other types of EDS are both rare and generally inconclusive.

Treatment of peri-invagination lesion and vitality preservation in an immature type III dens invaginatus: a case report.

BACKGROUND: To report a case of type III dens invaginatus associated with peri-invagination periodontitis in an immature permanent mandibular central incisor with open apex, in which only the invagination area was treated and vitality was preserved. CASE PRESENTATION: A 9-year-old boy was referred complaining of pain in the mandibular left central incisor. After radiographic examination, an invagination into the pulp chamber of the tooth associated with periapical radiolucency was detected. Endodontic access was performed and the orifice was identified under a dental operating microscope. The invagination area was chemo-mechanically cleaned. After 1 week, the invagination was obturated with mineral trioxide aggregate. During the 2-year follow up period, the tooth was asymptomatic. Radiographic examination revealed significant progression of periapical healing and root development in the main root canal of the tooth. CONCLUSION: Non-surgical root canal treatment of the invagination may preserve pulp vitality, and continuous root development of the tooth.