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Abstract(s)
Introdução: a acondroplasia é a forma mais comum de nanismo em humanos.(1, 2) Trata-se de uma displasia óssea resultante de uma mutação herdada de forma autossómica dominante que provoca alterações no desenvolvimento da cartilagem por deficiente ossificação endocondral. Torna-se, assim, inevitável relacionar a acondroplasia com as modificações do desenvolvimento crânio-mandibular. De facto, a falha na proliferação normal de cartilagem leva não só, e de uma maneira geral, ao característico encurtamento dos membros, como também ao subdesenvolvimento do terço médio da face.
Objetivos: análise de indivíduos acondroplásicos para verificação das características esqueléticas e cefalométricas da face dos mesmos.
Materiais e métodos: este é um estudo epidemiológico transversal descritivo e observacional, no qual foram avaliados 24 indivíduos acondroplásicos (grupo de estudo) e 50 indivíduos não acondroplásicos (grupo de controlo), com idades compreendidas entre os 4 e os 72 anos de idade, e de raça caucasiana.
Resultados: na avaliação vertical são vários os parâmetros cefalométricos que apresentaram diferenças significativas na população acondroplásica: Altura Facial Inferior, Inclinação do Plano Oclusal, Arco Mandibular, Ângulo do Plano mandibular de Ricketts e de Mcnamara, Overbite, distância do Plano Oclusal ao Ramo Mandibular, Plano Oclusal a S-N e Eixo Facial. Por sua vez, no que respeita a avaliação ântero-posterior, os parâmetros Wits, Profundidade Maxilar, Comprimento do Corpo Mandibular, Comprimento Cranial, Ângulo ANB, Segmente SE, Inclinação dos incisivos superiores e inferiores, Diferença entre a Maxila e a Mandíbula, distância do Côndilo ao Ponto A e as perpendiculares Na-A e Na-Pg, apresentam igualmente diferenças significativas na referida população acondroplásica. Conclusões: as características esqueléticas e cefalométricas preponderantes nos indivíduos acondroplásicos são o biótipo dolicofacial, presença de perfis retos a côncavos, hipoplasia maxilar com retrognatismo, prognatismo mandibular, presença de mordida aberta, protrusão dos incisivos superiores e inferiores e osso nasal de reduzidas dimensões.
Introduction: achondroplasia is the most common form of dwarfism in humans.(1, 2) This is a bone dysplasia resulting from an autosomal dominant inherited mutation that causes alterations in the development of cartilage by deficient endochondral ossification. It is therefore inevitable to relate achondroplasia to changes in craniomandibular development. In fact, the failure of normal cartilage proliferation leads not only to the characteristic shortening of the limbs but also to the underdevelopment of the medial face. Aim: analysis of achondroplastic individuals to verify the skeletal and cephalometric characteristics of the face. Materials and Methods: this is a descriptive and observational cross-sectional epidemiological study in which 24 achondroplastic individuals (study group) and 50 non-achondroplastic individuals (control group), aged between 4 and 72 years were evaluated. Results: in the vertical evaluation there were several cephalometric parameters with significant differences in the achondroplasic population: Lower Facial Height, Occlusal Plane Inclination, Mandibular Arc, Ricketts and Mcnamara Mandibular Plane Angle, Overbite, Distance from the Occlusal Plane to the Mandibular Branch, Occlusal Plane a SN and Facial Axis. On the other hand, in relation to the anteroposterior evaluation, the parameters Wits, Maxillary Depth, Mandibular Body Length, Cranial Length, ANB Angle, Segment SE, Inclination of upper and lower incisors, Difference between Maxilla and Jaw, distance from the Condyle to the Point A and the perpendiculars Na-A and Na-Pg, also showed significant differences in said achondroplasic population. Conclusions: the predominant skeletal and cephalometric characteristics in achondroplastic individuals are the dolichofacial biotype, concave/straight profiles, maxillary retrognathism, maxillary hypoplasia, mandibular prognathism, presence of open bite, protrusion of the maxillary and mandibular incisors, reduced nasal bone and enlarged pórion.
Introduction: achondroplasia is the most common form of dwarfism in humans.(1, 2) This is a bone dysplasia resulting from an autosomal dominant inherited mutation that causes alterations in the development of cartilage by deficient endochondral ossification. It is therefore inevitable to relate achondroplasia to changes in craniomandibular development. In fact, the failure of normal cartilage proliferation leads not only to the characteristic shortening of the limbs but also to the underdevelopment of the medial face. Aim: analysis of achondroplastic individuals to verify the skeletal and cephalometric characteristics of the face. Materials and Methods: this is a descriptive and observational cross-sectional epidemiological study in which 24 achondroplastic individuals (study group) and 50 non-achondroplastic individuals (control group), aged between 4 and 72 years were evaluated. Results: in the vertical evaluation there were several cephalometric parameters with significant differences in the achondroplasic population: Lower Facial Height, Occlusal Plane Inclination, Mandibular Arc, Ricketts and Mcnamara Mandibular Plane Angle, Overbite, Distance from the Occlusal Plane to the Mandibular Branch, Occlusal Plane a SN and Facial Axis. On the other hand, in relation to the anteroposterior evaluation, the parameters Wits, Maxillary Depth, Mandibular Body Length, Cranial Length, ANB Angle, Segment SE, Inclination of upper and lower incisors, Difference between Maxilla and Jaw, distance from the Condyle to the Point A and the perpendiculars Na-A and Na-Pg, also showed significant differences in said achondroplasic population. Conclusions: the predominant skeletal and cephalometric characteristics in achondroplastic individuals are the dolichofacial biotype, concave/straight profiles, maxillary retrognathism, maxillary hypoplasia, mandibular prognathism, presence of open bite, protrusion of the maxillary and mandibular incisors, reduced nasal bone and enlarged pórion.
Description
Keywords
Acondroplasia Crescimento crânio-mandibular Ortodontia Cefalometria Achondroplasia Craniomandibular growth Orthodontics Cephalometry