RESUMO
La picnodisostosis es una enfermedad poco común que pertenece a las displasias esqueléticas que presentan fragilidad ósea y fracturas frecuentes. Radiológicamente se caracteriza por incremento de la densidad y fragilidad óseas. OBJETIVO: Presentar el caso de un escolar con displasia esquelética con fracturas en hueso patológico y manejo quirúrgico. CASO CLÍNICO: Escolar de sexo femenino, con antecedente de picnodisostosis detectado en etapa preescolar. Consulta posterior a caída de bicicleta con fractura de ambos fémures que se manejan quirúrgicamente con placa de compresión bloqueada.
Pycnodysostosis is a rare condition within skeletal dysplasias presenting with brittle bones and frequent fractures. Radiologically, it is characterised by increased bone density and fragility. OBJECTIVE: To present the case of a primary schoolchild with skeletal dysplasia with pathological bone fractures and their surgical management. CASE REPORT: A female primary schoolchild with a history of pycnodysostosis detected during the pre-school period. She was seen after bicycle fall that resulted in the fracture of both femurs, that were surgically managed with a locking compression plate.
Assuntos
Humanos , Feminino , Criança , Fraturas do Fêmur/cirurgia , Fraturas do Fêmur/etiologia , Picnodisostose/complicações , Fixação Interna de Fraturas/métodos , Radiografia , Procedimentos Cirúrgicos Minimamente Invasivos , Fraturas do Fêmur/diagnóstico por imagem , Picnodisostose/diagnóstico por imagemRESUMO
Oral cavity very often has been described as reflection of our body as most of the initial clinical symptoms occur in oral cavity. The current paper also focuses on the oral presentation of the systemic condition called Pyknodysostosis (MIM 265800) which is a rare, autosomal recessive skeletal dysplasia characterized by short stature, wide cranial sutures, and increased bone density and fragility was first described in 1962 by Maroteaux and Lamy under the heading of diastrophic dwarfism.