RESUMO
BACKGROUND AND OBJECTIVE: Although the prevalence of osteogenesis imperfecta is low, the effect of this hereditary disease on patients' quality of life is considerable. We report our experience in the perioperative management of patients with this condition in our hospital. PATIENTS AND METHODS: Retrospective study describing the interventions on patients with this disease in our hospital from 1991 to 2009. We analyzed demographic data, disease variants, concomitant disorders, surgical procedures, type of anesthesia, and intraoperative and postoperative complications. RESULTS: From 1991 to 2009, 105 procedures were performed on 29 patients (ages 1 to 25 years) with osteogenesis imperfecta (37.9% women and 62.1% men). The most common type of osteogenesis imperfecta was type III (65.5%). Most patients (93%) had no associated diseases. Two patients were allergic to latex. No complications occurred in 62% of interventions. Reported complications during surgery were 1 case of non-malignant hyperthermia and 1 contralateral femur fracture. CONCLUSIONS: The prevalence of osteogenesis imperfecta is low. Treatment requires a multidisciplinary approach, in which appropriate perioperative management must be based on a proper understanding of the skeletal and extraskeletal abnormalities associated with this disease.
Assuntos
Anestesia , Osteogênese Imperfeita , Adolescente , Adulto , Anestesia/métodos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
Objetivos: La osteogénesis imperfecta es una enfermedad hereditaria con una baja prevalencia, pero gran impacto en la calidad de vida de los pacientes. Se presenta la experiencia en el manejo perioperatorio de estos pacientes en nuestro hospital. Pacientes y métodos: Estudio descriptivo retrospectivo de las intervenciones realizadas en nuestro centro durante los años 1991 a 2009, en los pacientes con esta enfermedad. Se analizan los datos demográficos, variante de la enfermedad, alteraciones asociadas, procedimientos quirúrgicos, tipo de anestesia y complicaciones intraoperatorias y postoperatorias. Resultados: Desde el año 1991 hasta el 2009 se han realizado un total de 105 intervenciones en 29 pacientes con osteogénesis imperfecta (37,9% mujeres, 62,1% varones), con una edad que oscila entre 1 a 25 años. El tipo más frecuente de osteogénesis imperfecta fue el tipo III (65,5%). El 93% de los pacientes no presentaron enfermedades asociadas. Dos pacientes eran alérgicos al látex. El 62% de las intervenciones transcurrieron sin complicaciones. Se registró un caso de hipertermia no maligna y una fractura de fémur contralateral en el quirófano. Conclusiones: La osteogénesis imperfecta es una enfermedad de baja prevalencia que precisa un tratamiento multidisciplinar. Es necesario un adecuado conocimiento de las alteraciones tanto esqueléticas como extraesqueléticas de esta enfermedad para un correcto tratamiento perioperatorio(AU)
Background and objective: Although the prevalence of osteogenesis imperfecta is low, the effect of this hereditary disease on patients quality of life is considerable. We report our experience in the perioperative management of patients with this condition in our hospital. Patients and methods: Retrospective study describing the interventions on patients with this disease in our hospital from 1991 to 2009. We analyzed demographic data, disease variants, concomitant disorders, surgical procedures, type of anesthesia, and intraoperative and postoperative complications. Results: From 1991 to 2009, 105 procedures were performed on 29 patients (ages 1 to 25 years) with osteogenesis imperfecta (37.9% women and 62.1% men). The most common type of osteogenesis imperfecta was type III (65.5%). Most patients (93%) had no associated diseases. Two patients were allergic to latex. No complications occurred in 62% of interventions. Reported complications during surgery were 1 case of non-malignant hyperthermia and 1 contralateral femur fracture. Conclusions: The prevalence of osteogenesis imperfecta is low. Treatment requires a multidisciplinary approach, in which appropriate perioperative management must be based on a proper understanding of the skeletal and extraskeletal abnormalities associated with this disease(AU)
