ABSTRACT
El papel que desempeñan las alteraciones genéticas en el desarrollo dental es fundamental. Se ha descubierto que si no existe una correcta expresión del gen o se da una mutación de este, el individuo podría presentar ausencias o malformaciones de estructuras de la boca. Por esta razón se describe la forma en que se da el desarrollo de las estructuras dentales, teniendo en cuenta cómo las interacciones genética y ambiental influyen en su correcto desarrollo. Entre los genes involucrados se encuentran el PAX9 y el MSX1, que según recientes investigaciones son los implicados en las ausencias congénitas de estructuras dentarias o sus posibles alteraciones, teniendo en cuenta que la delación de estos genes o su mutación son factores hereditarios. Los genes odontogénicos PAX9 y MSX1, son genes homeóticos (homebox) que codifican para factores de transcripción y son responsables, durante la odontogénesis, de la expresión de genes asociados con la regulación espacial y temporal dentro del primer arco braquial. En determinado momento de la organogénesis pueden darse fallas en la expresión de los factores necesarios para la formación y buen desarrollo dental, causando anomalías como la Odontodisplasia Regional (OR), también denominada diente fantasma o detención localizada del desarrollo dental, la cual es una anomalía estructural del desarrollo, compleja y rara; parece ser el resultado de una o más mutaciones puntuales en el cromosoma 4 y 14. Se reportan dos casos donde se describen las características clínicas, radiográficas, y el seguimiento clínico.
The role of genetic alterations in tooth development is essential. It has been discovered that if there is a correct expression of the gene or there is a mutation of this, the individual could present absence or malformations of structures of the oral cavity or other body parts. For this reason we describe how given the development of dental structures, taking into account how genetic and environmental interactions influence their proper development. Among the genes involved are in the PAX9 and MSX1, which according to recent research are involved in congenital absence of dental structures or alterations, considering that the denunciation of these genes or the mutation is inherited. The odontogenic PAX9 and MSX1 genes are homeotic genes (homebox) that encode for transcription factors and are responsible, during odontogenesis, the expression of genes associated with spatial and temporal regulation within the first brachial arch. At some point during organogenesis may be flaws in the expression of the factors necessary for the formation and tooth development, causing among other abnormalities Regional Odontodysplasia (RO), also called ghost tooth or detention tooth dental development located, which is a structural anomaly development complex and rare; seems to be the result of one or more point mutations in the chromosome 4 and 14. In this report we show two cases with odontodysplasia where there are clinical and radiographic features of two patients with this anomaly, one of them has been made up and treatment, and the other begins his analysis stage.
Subject(s)
Humans , Male , Female , Child , Adolescent , Odontodysplasia/pathology , Maxilla/abnormalities , Orthodontics , Radiography, Panoramic , Odontodysplasia/diagnosis , Endodontics , Anodontia , Morphogenesis , OdontogenesisABSTRACT
Regional odontodysplasia is a rare and significant dental malformation. The present report describes an unexpected radiographic finding of rare tooth developmental anomaly and conservative management of primary teeth with in a child in mixed dentition. A 5-year-old healthy girl was referred to the Pediatric Dental Clinic due to pain and periapical radiograph exam showed teeth with abnormal large pulp chambers and a thin enamel and dentin of the primary incisors and canine and abnormal germs of the permanent successors teeth, with 'ghost-like' appearance. In addition, no root formation or only an insignificant amount was visible, characterizing regional odontodysplasia. At 10 years old, the treatment plan consisted of rehabilitation with a provisory partial resinous fixed prosthesis until implant surgery. The girl is still being followed up periodically.
A odontodisplasia regional é uma importante e rara malformação dental. O presente relato descreve um achado radiográfico inesperado de uma anomalia de desenvolvimento rara e seu manejo conservador em uma criança em dentição mista. Uma menina de 5 anos de idade, saudável, foi encaminhada à Clínica de Odontopediatria devido à dor. O exame radiográfico periapical mostrou os elementos dentários com câmaras pulpares amplas e uma fina camada de esmalte e dentina nos incisivos decíduos e anomalia nos germes dentários dos caninos permanentes sucessores, com aparência de 'dente-fantasma'. Além disso, não havia formação de raízes ou apenas uma quantidade insignificante era visível, caracterizando a odontodisplasia regional. Aos 10 anos, o plano de tratamento consistiu na reabilitação provisória por meio de uma prótese parcial fixa de resina até a cirurgia de implante ser possível. A criança, ainda, se encontra em acompanhamento periódico.
ABSTRACT
PURPOSE: Regional odontodysplasia (RO) is a rare condition characterized by distinctive clinical, radiographic and microscopic findings. It is presumed to be the result of a developmental disturbance that locally affects the odontogenic ectodermal and mesodermal tissues. This report describes the clinical and radiographic findings of RO and its treatment. CASE DESCRIPTION: A 15-year-old boy presented with an uncommon case of RO involving two quadrants of the jaws on the right side of his face. CONCLUSION: A complex multidisciplinary team is required to fully rehabilitate a patient affected by RO. The treatment of RO is controversial and varies according to the individual patient.
OBJETIVO: Odontodisplasia regional (OR) é uma condição rara caracterizada por achados clínicos, radiográficos e microscópicos bem distintos. É o resultado de um distúrbio de desenvolvimento bem localizado afetando tanto o ectoderma quanto o mesoderma odontogênico. Este trabalho descreveu os achados clínicos e radiográficos de um caso de OR e os tratamentos. DESCRIÇÃO DO CASO: Um menino de 15 anos de idade apresentou um caso incomum de OR envolvendo dois quadrantes dos maxilares no lado direito da face. CONCLUSÃO: Há necessidade de uma equipe multidisciplinar complexa para reabilitar inteiramente um paciente com OR. O tratamento de OR é controverso e varia de acordo com as características e resposta do paciente.
Subject(s)
Humans , Male , Adolescent , General Surgery/methods , Dentin Dysplasia/diagnosis , OdontodysplasiaABSTRACT
Regional odontodysplasia (RO) is a rare developmental anomaly involving both mesodermal and ectodermal dental components in a group of contiguous teeth. It affects the primary and permanent dentitions in the maxilla and mandible or both jaws. Generally it is localized in only one arch. Clinically, affected teeth have an abnormal morphology, are soft on probing and typically discolored, yellow or yellowish-brown. Radiographically, the affected teeth show a “ghostlike” appearance. This paper reports the case of an 11-year-old girl presenting this rare anomaly on the right side of the maxillary arch. The primary maxillary right central, the lateral and the canine were found missing. The permanent teeth had a “ghostlike” appearance radio graphically. The treatment performed was rehabilitation with temporary partial acrylic denture and periodic controls. The presentation of this case adds valuable information to dentists to review special clinical and radiographic features of RO, which will facilitate the diagnosis and treatment of patients with this condition. Since the etiology of this dental anomaly is uncertain, a review of its probable etiologic factors is summarized to get a better understanding of its cause of occurrence.
ABSTRACT
La odontodisplasia Regional es una anomalía del desarrollo poco frecuente de los tejidos dentarios duros, que involucra ambos tipos de denticiones y generalmente en un solo cuadrante. Lascaracterísticas clínicas son variables e incluyen decoloración dental amarilla o amarilla café, esmalte y dentina hipomineralizados, acompañadas de gingivitis, inflamación o abscesos; radiográficamente presentan cámaras pulpares amplias, y radiodensidad disminuida de esmalte y dentina dando la apariencia de dientes fantasma.
The Regional odontodysplasia is a rare developmental anomaly of dental hard tissues, involving both types of dentition and usually in one quadrant. Clinical features are variable and include dentaldecoloration yellow or yellowish-brown, enamel and dentin hypomineralised, accompanied by gingivitis, swelling or abscesses. Radiographically large pulp chambers are present and decreasedradiodensity of enamel and dentin giving the appearance of "ghost teeth".
Subject(s)
Humans , Dentin Dysplasia , Esthetics, Dental , OdontodysplasiaABSTRACT
Regional odontodysplasia (RO) is a rare developmental anomaly involving both mesodermal and ectodermal dental components in a group of contiguous teeth. It affects the primary and permanent dentitions in the maxilla and mandible or both jaws. Generally it is localized in only one arch. The etiology of this dental anomaly is uncertain. Clinically, affected teeth have an abnormal morphology, are soft on probing and typically discolored, yellow or yellowish-brown. Radiographically, the affected teeth show a "ghostlike" appearance. This paper reports the case of a 5-year-old girl presenting this rare anomaly on the left side of the maxillary arch, which crossed the midline. The primary maxillary left teeth (except for the canine) and the primary maxillary right central incisor were missing due to previous extractions. The permanent teeth had a "ghostlike" appearance radiographically. The treatment performed was rehabilitation with temporary partial acrylic denture and periodic controls. In the future, the extraction of affected permanent teeth and rehabilitation with dental implants will be evaluated. The presentation of this case adds valuable information to pediatric dentists to review special clinical and radiographic features of RO, which will facilitate the diagnosis and treatment of patients with this condition.