Introduction: Osteogenesis imperfecta (OI) is a genetic disorder characterized by extreme bone fragility, leading to fractures from minimal trauma. OI is rare, with a global incidence of 1 in 10,000 to 20,000 live births. In Chile, 109 cases have been reported. Advances in anesthesia and perioperative care have improved the safety of surgical interventions for these patients, who require special attention due to their complex medical needs. Case Presentation: We present the case of an 8-year-old girl with type III OI, diagnosed at birth, who underwent surgery to correct the deformity of both femurs through multiple osteotomies and telescopic intramedullary osteosynthesis. The anesthetic plan included opioid-free general anesthesia and epidural neuroaxial anesthesia. Induction was performed with sevoflurane, followed by propofol and rocuronium for neuromuscular blockade. Ketadex was administered with a loading dose of 1 ml/kg over 10 minutes, followed by a continuous infusion of 0.3 ml/kg/hr. The patient was invasively monitored, and special care was taken in her mobilization during surgery. The surgical procedure was uneventful, with adequate postoperative pain management, and she was discharged after 4 days without new fractures. Discussion: Type III OI is a severe form of the disease, characterized by significant bone fragility and progressive deformities. Anesthetic challenges include airway management, the risk of perioperative fractures, and pain management. Patients with OI have a higher likelihood of complications due to their bone fragility and associated deformities, requiring a specific anesthetic approach. Literature supports the use of neuroaxial or regional anesthesia techniques for pain management, complemented by general anesthesia. Risks include difficulty in intubation, temperature management, and a higher propensity for fractures and intraoperative bleeding. Current evidence suggests that the incidence of severe complications is low, but patients with type III OI are at increased risk compared to other types. Careful mobilization, the use of adapted anesthetic techniques, and thorough monitoring are crucial to minimizing complications. The administration of bisphosphonates and other medical treatments may influence perioperative management, particularly concerning bleeding. Conclusion: Anesthetic and perioperative management of patients with OI requires meticulous planning to address the risks associated with bone fragility and specific deformities. Although advances in anesthesia have improved the safety of these interventions, continued research and refinement of protocols are essential to reduce complications and optimize long-term outcomes. A multidisciplinary approach, with an emphasis on detailed pre-anesthetic evaluation, safe patient mobilization, and rigorous monitoring, is essential for successful management in these patients.
Figueroa R., P. ., Vera N., B. ., Montecinos M., V. ., & Meneses A., C. . (2025). Anesthetic considerations in osteogenesis imperfecta: A literature review based on a case report. Boletín De Anestesia, (5), pp. 34–43. Retrieved from https://boletinanestesia.uchile.cl/index.php/BA/article/view/81316
