A Retrospective Review of Anaesthetic Records of Patients with Osteogenesis Imperfecta Undergoing Orthopaedic Procedures over 10 years. A single-center study

Osteogenesis imperfecta (OI) is a rare, autosomal dominant, inherited connective tissue disorder that affects bones*,* inner ear and the sclera. Mutations in the collagen type I (COLIA 1) and type II (COLIA 2) genes lead to the development of this disorder. The incidence is about 1 in every 20,000 births. The most common features of this disease include fragile bones, hyperextensible joints, blue sclera, improper dentition and progressive hearing loss it is also associated with conditions like kyphoscoliosis that can lead to cardiopulmonary compromise. These patients mainly present with features that are either skeletal or non-skeletal. Perioperative anesthetic considerations that are of primary concern include: airway challenges, intravenous catheter placement challenges, difficulty performing neuraxial and/or peripheral nerve blockade, significant intraoperative blood loss, hypermetabolism with concern for malignant hyperthermia, and perioperative fracture. Airway management may be difficult.

We aim to access the records of OI patients who have undergone major orthopaedic procedures at our center over the last 10 years. Being a rare condition, this study will help throw insight on the Indian scenario and the most common variants of OI, the anaesthetic risks and perioperative challenges for better management.