Anaesthetic Management in a Patient with osteogenesis imperfecta

Abstract

Osteogenesis Imperfecta (OI) (Brittle Bone disease) is a rare, autosomal inherited disorder due to a defect in collagen I synthesis leading to skeletal deformities with a characteristic tendency to fracture bones easily, and ocular, otologic, cutaneous and dental abnormalities ¹ . Anaesthetic management of such patient is a real challenge to an Anaesthesiologist due to multipal skeleton deformities,risk of odonto axial dislocation during laryengosopy and intubation and difficulty in achivenment of reagional anaesthesia due to spastic posture .Again there is a unusual response to muscle relaxants and chances of malignant hyperthermia intraoperative. A case of 11 year old patient with osteogenesis imperfect with cerebral palsy and mental retardation was posted for bone shortning and K wire fixation managed successfully under epidural anaesthesia after proper pre operative assessment and workup.