[Anesthetic treatment of patients with osteogenesis imperfecta].

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.