"Maxillary lateral incisor partial anodontia sequence": a clinical entity with epigenetic origin

ABSTRACT The relationship between maxillary lateral incisor anodontia and the palatal displacement of unerupted maxillary canines cannot be considered as a multiple tooth abnormality with defined genetic etiology in order to be regarded as a "syndrome". Neither were the involved genes identified and located in the human genome, nor was it presumed on which chromosome the responsible gene would be located. The palatal maxillary canine displacement in cases of partial anodontia of the maxillary lateral incisor is potentially associated with environmental changes caused by its absence in its place of formation and eruption, which would characterize an epigenetic etiology. The lack of the maxillary lateral incisor in the canine region means removing one of the reference guides for the eruptive trajectory of the maxillary canine, which would therefore, not erupt and /or impact on the palate. Consequently, and in sequence, it would lead to malocclusion, maxillary atresia, transposition, prolonged retention of the deciduous canine and resorption in the neighboring teeth. Thus, we can say that we are dealing with a set of anomalies and multiple sequential changes known as sequential development anomalies or, simply, sequence. Once the epigenetics and sequential condition is accepted for this clinical picture, it could be called "Maxillary Lateral Incisor Partial Anodontia Sequence."

RESUMO A relação entre a anodontia parcial do incisivo lateral e o deslocamento palatino do canino superior não irrompido não pode ser considerada uma anomalia dentária múltipla com etiopatogenia genética definida, a ponto de ser considerada como uma "síndrome". Os genes envolvidos sequer foram identificados e localizados no genoma humano, e nem mesmo presumiu-se em qual cromossomo se localizaria o gene responsável. O deslocamento palatino do canino superior em casos de anodontia parcial do incisivo lateral superior está potencialmente associado às mudanças ambientais provocadas pela sua ausência no local de formação e erupção, o que caracterizaria uma etiologia epigenética para essa associação. A falta do incisivo lateral superior na região canina implica em tirar um dos guias referenciais da trajetória eruptiva do canino superior, que ficaria, assim, não irrompido e/ou impactado no palato. Como consequência, e em sequência, promove-se uma má oclusão, atresia maxilar, transposição, retenção prolongada do canino decíduo e reabsorções nos dentes vizinhos. Dessa forma, pode-se afirmar que estamos frente a um conjunto de anomalias e alterações múltiplas sequenciais conhecido como anomalias de desenvolvimento sequencial ou, simplesmente, sequência. Uma vez aceita a condição epigenética e sequencial para esse quadro clínico, ele poderia ser chamado de "Sequência da Anodontia Parcial do Incisivo Lateral Superior".

"Maxillary lateral incisor partial anodontia sequence": a clinical entity with epigenetic origin.

The relationship between maxillary lateral incisor anodontia and the palatal displacement of unerupted maxillary canines cannot be considered as a multiple tooth abnormality with defined genetic etiology in order to be regarded as a "syndrome". Neither were the involved genes identified and located in the human genome, nor was it presumed on which chromosome the responsible gene would be located. The palatal maxillary canine displacement in cases of partial anodontia of the maxillary lateral incisor is potentially associated with environmental changes caused by its absence in its place of formation and eruption, which would characterize an epigenetic etiology. The lack of the maxillary lateral incisor in the canine region means removing one of the reference guides for the eruptive trajectory of the maxillary canine, which would therefore, not erupt and /or impact on the palate. Consequently, and in sequence, it would lead to malocclusion, maxillary atresia, transposition, prolonged retention of the deciduous canine and resorption in the neighboring teeth. Thus, we can say that we are dealing with a set of anomalies and multiple sequential changes known as sequential development anomalies or, simply, sequence. Once the epigenetics and sequential condition is accepted for this clinical picture, it could be called "Maxillary Lateral Incisor Partial Anodontia Sequence."

Orthodontic-restorative treatment as an option for biologic replacement of a maxillary central incisor: 5-year follow-up.

The maxillary central incisor is the tooth most often affected by trauma, especially in the age range of 7 to 10 years, when high-impact sports are prevalent. The options for conservative treatment should be prioritized in these patients, aiming to achieve a biologic response that might provide continuity of growth of the alveolus, to provide functional and esthetic development of the affected region. This case report describes a patient with a history of trauma during the deciduous dentition with consequent intrusion, root dilaceration, and retention of the maxillary left central incisor. The treatment involved extraction of the traumatized tooth and mesial movement of the lateral incisor and posterior segments.

Cleidocranial dysostosis: a case report / Disostosis cleidocraneal: reporte de caso

Cleidocranial dysostosis is a congenital condition that results from faulty development of membranous bones, mainly the clavicles and skull. The clavicular abnormality may range from a small defect in one clavicle to complete absence of both, but most frequently an absence of the central clavicular segment, as was seen in our patient. A review of the literature has revealed only one previous report a cleidocranial dysostosis syndrome...

La disostosis cleidocraneal es una condición congénita que resulta de un desarrollo defectuoso de los huesos con osificación membranosa, principalmente las clavículas y el cráneo. La anormalidad clavicular puede ir desde un pequeño defecto en una clavícula hasta la total ausencia de ambas, pero con mayor frecuencia se observa la ausencia del segmento clavicular central, como se observó en nuestro paciente. Una revisión de la literatura reveló sólo un caso previo con un síndrome de disostosis cleidocraneal de características similares...

Maxillary ameloblastic fibroma: a case report.

Ameloblastic fibroma is a relatively rare benign odontogenic tumor in which both the epithelial and ectomesenchymal components are neoplastic. An 8-year-old Caucasian boy was referred to the dentist for evaluation of failed eruption of the maxillary left first molar. The panoramic radiograph showed a well-circumscribed unilocular radiolucency involving an unerupted maxillary left first permanent molar. The lesion was enucleated and the material was sent for histopathologic examination. Microscopically, it was composed by cords and islands of odontogenic epithelium in a myxoid cell-rich stroma that closely resemble the dental papilla with histopathological diagnosis of ameloblastic fibroma. After 24 months of follow-up no recurrence was observed and the maxillary left first molar erupted spontaneously through the buccal mucosa and was aligned with a fixed orthodontic appliance. This case emphasized the importance of careful differential diagnosis of intraosseous oral lesions and reported a rarity of the lesion and its atypical location.

Maxillary ameloblastic fibroma: a case report

Ameloblastic fibroma is a relatively rare benign odontogenic tumor in which both the epithelial and ectomesenchymal components are neoplastic. An 8-year-old Caucasian boy was referred to the dentist for evaluation of failed eruption of the maxillary left first molar. The panoramic radiograph showed a well-circumscribed unilocular radiolucency involving an unerupted maxillary left first permanent molar. The lesion was enucleated and the material was sent for histopathologic examination. Microscopically, it was composed by cords and islands of odontogenic epithelium in a myxoid cell-rich stroma that closely resemble the dental papilla with histopathological diagnosis of ameloblastic fibroma. After 24 months of follow-up no recurrence was observed and the maxillary left first molar erupted spontaneously through the buccal mucosa and was aligned with a fixed orthodontic appliance. This case emphasized the importance of careful differential diagnosis of intraosseous oral lesions and reported a rarity of the lesion and its atypical location.

Fibroma ameloblástico é um tumor odontogênico benigno relativamente raro, em que ambos os componentes epiteliais e ectomesenquimais são neoplásicos. Menino de oito anos de idade, branco, foi encaminhado ao dentista para avaliar a falha na erupção do primeiro molar maxilar do lado esquerdo. A radiografia panorâmica revelou imagem radiolúcida, unilocular, bem circunscrita, envolvendo o primeiro molar permanente maxilar esquerdo incluso. A lesão foi enucleada e o material encaminhado para avaliação histopatológica. Microscopicamente, era composta de ilhas e cordões de epitélio odontogênico num estroma mixóide rico em células, que se assemelhava à papila dentária, com diagnóstico histopatológico de fibroma amelobástico. Após 24 meses de acompanhamento, nenhuma recorrência foi observada e o primeiro molar permanente maxilar irrompeu espontaneamente através da mucosa bucal e foi alinhado com aparelho ortodôntico fixo. Esse caso enfatiza a importância do cuidadoso diagnóstico diferencial das lesões orais intra-ósseas e relato de lesão rara e sua localização atípica.

Valoración de la ecuación de Tanaka-Johnston en estudiantes cubanos con oclusión normal / Assessment of Tanaka-Johnston equation in Cuban students presenting normal occlusion

El análisis de Tanaka-Johnston, mundialmente empleado para la predicción del espacio de caninos y premolares no brotados, ha sido muy cuestionado por la variabilidad en el tamaño dentario entre las diferentes poblaciones, y por haber sido realizado en un grupo étnico no aplicable a todos. Se realizó un estudio descriptivo transversal, con el objetivo de determinar el ancho mesiodistal de incisivos, caninos y premolares mandibulares, así como de caninos y premolares maxilares, y comparar la suma real de estos dientes con los estimados por Tanaka-Johnston, según el sexo y la arcada. El universo de esta investigación estuvo constituido por 50 estudiantes de la Facultad de Estomatología de La Habana, 25 del sexo femenino y 25 del sexo masculino, con oclusión normal, sin antecedentes de tratamiento ortodóncico; todos descendientes de cubanos. En los resultados se observó que los anchos mesiodistales de los incisivos, caninos y primeros premolares inferiores y de caninos y segundo premolar izquierdo superiores fueron significativamente mayores en el sexo masculino que en el femenino. La suma real de caninos y premolares superiores e inferiores fue mayor en el sexo masculino que en el femenino. Cuando se comparó la suma real de caninos y premolares, con lo estimado según la ecuación de Tanaka-Johnston, se halló que la esta sobrestimó los valores de caninos y premolares, ya que los resultados fueron significativamente menores(AU)

The Tanaka-Johnston analysis used at worldwide to predict the space of non-erupted canines and premolars has been questioned due to the variability in the teeth size among different populations and also to be carried out in non-applicable whole ethnic group. A cross-sectional and descriptive study was conducted to determine the mesiodistal width of mandibular incisives, canines and premolars, as well as of danines and premolar-maxillary and to compare the real sum of these theeth with the estimates by Tanaka-Johnston, according to sex and the arcade. Universe of this research included 50 students of Stomatology Faculty of havana (25 males and 25 females) presenting with normal occlusion, without a history of Orthodontics treatment all of them descendants of Cubans. In results it was noted that the mesiodistal widths of incisives, canines and first lower premolars and of canines and second left premolars upper are greater in male sex than in the female one. The real sum of upper and lower canines and premolars was greter in male sex than in the female one. Comparing the real sum of canines and premolars with that estimated according Tanaka-Johnston, it was noted that it overestimated the values of canines and premolar since the results were significantly lower(AU)

Maroteaux-Lamy syndrome: orofacial features after treatment by bone marrow transplant.

PURPOSE: The objective of the present study was to emphasise the oral and dental findings of a male patient with the Maroteaux-Lamy syndrome who successfully underwent bone marrow transplantation (BMT) at the age of 22 months. CASE REPORT: A 15-year-old boy was referred to the Dentistry Division of the Catholic University of Brasília, Brazil, for dental diagnosis. General characteristics of the Maroteaux-Lamy syndrome, such as a large head, a short neck, corneal opacity, an open mouth with macroglossia, enlargement of the skull and a long anteroposterior dimension, were observed. The patient had received the benefit of a BMT at an early stage. Therefore, characteristics were presented in a moderate form, except for the skeletal symptoms. DISCUSSION: Maroteaux-Lamy syndrome, also known as mucopolysaccharidosis type VI, is a lysosomal storage disorder that is caused by a deficiency of arylsulphatase B, which leads to an accumulation of dermatan sulphate in tissues and its increased excretion in urine. The deposition of mucopolysaccharides leads to a progressive disorder involving multiple organs. It is a rare condition that is inherited as an autosomal recessive trait. The characteristic features of this disease include retardation in growth, a large head, a short neck, corneal opacity, typical facies and spinal abnormalities. The main dental findings of this syndrome include gingival hyperplasia, hypertrophy of the maxillary alveolar ridge, macroglossia, unerupted dentition, malocclusions and dentigerous cyst-like follicles. BMT is a therapeutic treatment that is given to permanently replace any disorder caused due to the deficiency of enzymes in patients with storage diseases.

Síndrome de ectrodactilia, displasia ectodérmica y labio-paladar hendidos / Syndrome of Ectrodactylism, ectodermic dysplasia and lip-cleft palate

El presente trabajo se propone reportar a un paciente masculino de 12 años de edad con presencia de la tríada completa del síndrome ectrodactilia, displasia ectodérmica y labio-paladar hendidos (EEC); señalar los hallazgos clínicos encontrados en las valoraciones, así como los tratamientos realizados. Es un síndrome autonómico dominante, con penetración incompleta y expresividad variable. Esta conjunción de signos y síntomas puede llevar a la confusión con otro tipo de entidades clínicas, y ocasionar un diagnóstico erróneo del paciente. La hendidura de labio y ausencia del conducto lagrimal no es una combinación usual en otras condiciones. Se hace mención de las manifestaciones clínicas reportadas en la literatura, y se enfatiza en las manifestaciones maxilofaciales y dentales. Los dos hermanos del paciente se consideraron normales o exentos de este síndrome(AU)

In present paper is reported the case of a patient presenting with the complete triad of Ectrodactylism syndrome, ectodermic dysplasia and lip-cleft palate, to mention the clinical findings present in assessments as well as the treatments applied. The syndrome is characterized by the triad Ectrodactylism, ectodermic dysplasia and cleft (EEC) and it is autosomal dominant with incomplete penetration and a variable expressiveness. This group of signs and symptoms may be cofounded with other clinical entities, leading to a mistake diagnosis of patient. Lip cleft and lack of lacrimal duct is not a usual combination in other conditions. This is the clinical case of a male patient aged 12 with the syndrome of: Ectrodactylism, ectodermic dysplasia and lip-cleft palate. Clinical manifestations reported in literature are mentioned emphasizing the maxillofacial and dental manifestations. The two brothers of patient were normal or free of this syndrome. It is very important that the professionals charged of oral and maxillofacial health, as well the Stomatology students of Bachelor degree of postgraduate level, be able to identify, diagnose and to work in a multidisciplinary way to arrive to definite diagnosis of patients presenting with EEC syndrome and to apply the appropriate treatments(AU)

Cessation of dental development in a child with idiopathic hypoparathyroidism: a 5-year follow-up.

Idiopathic hypoparathyroidism is a rare endocrinopathy whose origin is unknown, and which is characterized by a deficiency of parathyroid hormone causing low serum calcium and high serum phosphorus concentrations. Disorders of ectodermal tissues are common in this pathology, and enamel hypoplasia is the most frequently found dental pathology. This article describes the case of a 9-year-old child with hypoparathyroidism that had gone undiagnosed for 6 years. The permanent dentition showed enamel hypoplasia, delayed eruption, and cessation of root formation (i.e., shortened roots), resulting from nontreated hypocalcemia during the developmental phase of the dentition.

Primary failure of tooth eruption associated with secondarily inflamed dental follicle: inflammatory follicular cyst?

Primary failure of eruption represents a cessation of tooth eruption before emergence not due to a physical barrier or abnormal position and has no systemic cause. This failure of tooth eruption is rare phenomenon and it has not been associated with alterations of dental follicle. This case report describes a permanent right first molar with primary failure of eruption at the inferior border of the mandible presenting microscopic, clinical and radiographic findings compatible with follicular cystic lesion. The follicular tissues of this molar showed microscopic aspects of inflammation. Thus, pericoronal tissues surrounding long-term unerupted teeth with primary failure of eruption has potential to develop into odontogenic cysts. The lesion presented herein probably arose in association with a periodontal pocket mesial to the adjacent second molar, representing an atypical inflammatory follicular cyst. This case shares etiological, radiological and microscopic features with the usual form of the inflammatory follicular cyst associated with infected primary teeth, except for the infection pathways reaching the tooth follicle. The controversies regarding the terminology for this cyst are discussed.

Unicystic ameloblastoma in a child: a differential diagnosis from the dentigerous cyst and the inflammatory follicular cyst.

Unicystic ameloblastoma (UA) is a benign epithelial odontogenic tumor of the jaws with an aggressive potential that commonly occurs in children. This cystic odontogenic neoplasm is generally asymptomatic and found during routine radiographs. The purposes of this report were to describe a case of UA involving the crown of the unerupted right mandibular second premolar in an 11-year-old girl under orthodontic treatment, and discuss its diagnosis and radiographic and microscopic findings, emphasizing its distinction from the dentigerous cyst and the inflammatory follicular cyst.

Primary failure of tooth eruption associated with secondarily inflamed dental follicle: inflammatory follicular cyst?

Primary failure of eruption represents a cessation of tooth eruption before emergence not due to a physical barrier or abnormal position and has no systemic cause. This failure of tooth eruption is rare phenomenon and it has not been associated with alterations of dental follicle. This case report describes a permanent right first molar with primary failure of eruption at the inferior border of the mandible presenting microscopic, clinical and radiographic findings compatible with follicular cystic lesion. The follicular tissues of this molar showed microscopic aspects of inflammation. Thus, pericoronal tissues surrounding long-term unerupted teeth with primary failure of eruption has potential to develop into odontogenic cysts. The lesion presented herein probably arose in association with a periodontal pocket mesial to the adjacent second molar, representing an atypical inflammatory follicular cyst. This case shares etiological, radiological and microscopic features with the usual form of the inflammatory follicular cyst associated with infected primary teeth, except for the infection pathways reaching the tooth follicle. The controversies regarding the terminology for this cyst are discussed.

A falha primária de erupção é caracterizada pelo não irrompimento de um dente sem que haja impedimento mecânico ou que o dente esteja em posição anormal ou devido à causas sistêmicas. É um fenômeno raro e não foi associado com alterações do folículo dentário. Este relato descreve um caso de falha primária de erupção de um primeiro molar inferior direito permanente, localizado na base da mandíbula, que apresenta achados clínicos, radiográficos e microscópicos compatíveis com cisto folicular. O folículo pericoronário deste molar apresentou aspectos microscópicos de inflamação. Assim como os tecidos pericoronários dos dentes não irrompidos, a longo prazo, os folículos pericoronários dos dentes com falha primária de irrompimento têm o potencial para desenvolver cistos odontogênicos. Esta lesão provavelmente surgiu em associação com uma bolsa periodontal na mesial do segundo molar adjacente, caracterizando um cisto folicular inflamatório atípico. Este caso apresenta características etiológicas, radiográficas e microscópicas comuns nos cistos foliculares inflamatórios associados a dentes decíduos infectados, com exceção das infecções que alcançam o folículo. São discutidas ainda as controvérsias relativas à terminologia deste cisto.

Dental findings in GAPO syndrome: case report.

This article reports the case of a young female adult with GAPO syndrome who presented as a peculiar dental finding unerupted primary and permanent dentitions, which resembled total anodontia on clinical examination. A cephalometric analysis was performed to investigate the alterations in facial bone development. This is the 9th GAPO syndrome case reported in a Brazilian patient.

Dental findings in GAPO syndrome: case report

This article reports the case of a young female adult with GAPO syndrome who presented as a peculiar dental finding unerupted primary and permanent dentitions, which resembled total anodontia on clinical examination. A cephalometric analysis was performed to investigate the alterations in facial bone development. This is the 9th GAPO syndrome case reported in a Brazilian patient.

Este artigo relata o caso de um jovem paciente, gênero feminino, portadora da síndrome de GAPO, apresentando impacções dos dentes decíduos e permanentes, sugerindo anodontia total no exame clínico. Foi realizada uma análise cefalométrica para investigar as alterações no desenvolvimento ósseo facial. Este é o nono caso descrito no Brasil

Osteopetrosis--a review and report of two cases.

We present a brief review of the rare condition of osteopetrosis together with two case reports of this disease in the same family affecting the jaws. The first in a 41-year-old woman, and the second in her 39-year-old brother. Plain films and computed tomography showed marked sclerosis of the affected bones with obliteration of the medullary cavities and thickening of the cortices as well as multiple absent and unerupted teeth. In addition radiographs showed discrete mixed radiopaque/radiolucent areas consistent with the appearance of fibro-cemento-osseous dysplasia, but which may also represent part of the overall spectrum of bone changes in osteopetrosis.

O odontoma como fator etiológico da näo-erupçäo dentária: relato de caso / Odontoma as an etiologic factor of non-erupting tooth: a case report

O odontoma pode se tornar um obstáculo à erupçäo dentária. O diagnóstico precoce de sua presença permite um tratamento cirúrgico preventivo. Apresentamos um caso clínico e procuramos explorar pontos fundamentais no diagnóstico e tratamento da alteraçäo

Odontomas--report of 3 cases.

Three clinical cases of odontoma were detected in 2 children and 1 adolescent. The tumors were surgically removed. Clinical suspicion was based on facial deformity in one of the cases and on the absence of permanent tooth eruption in the other two. Radiographic evidence of odontoma was confirmed through histological study.