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Abstract
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Background and Aims Osteogenesis imperfecta (OI) is a rare genetic disorder affecting connective tissue, particularly characterized by fragile bones due to a defect in type I collagen production. Challenges in airway management, positioning, increased bleeding tendency, and a high risk of malignant hyperthermia (MH) complicate anesthesia management. In this study, we aimed to retrospectively examine our experience in terms of anesthesia in pediatric patients diagnosed with OI operated in the orthopedic clinic of our university hospital.
Methods We retrospectively reviewed files of patients with OI who underwent orthopedic surgery at our hospital between 2011 and 2022. We screened for airway management issues, intravenous access difficulties, surgical blood loss, peripheral nerve blockade/neuraxial techniques, perioperative fracture, and intraoperative peak temperature.
Results In our study, 40 patients with OI and 101 orthopedic operations were evaluated. Difficult airway was encountered in 1/101 (1%) and perioperative fracture in 1/101 (1%) of the cases. Neuraxial anesthesia was attempted in 32/101 cases with a success rate of 87%. All peripheral nerve block attempts (16/101 cases) were successful. Difficult intravenous catheter placement was noted in 3/101 (3%) of cases. An estimated blood loss of more than 10% of the estimated blood volume was considered significant and occurred in 17/101 (17%) cases.
Conclusions Although patients with osteogenesis imperfecta are rare, the need for orthopedic surgery frequently requires anesthesia. OI is a serious disease associated with multiple complications and it is necessary to determine the severity of the disease, perform a complete preoperative evaluation and develop an appropriate, individualized anesthesia management plan for patients requiring surgery.
