Total intravenous anaesthesia and the use of an intubating laryngeal mask in a patient with osteogenesis imperfecta.

Abstract

Osteogenesis imperfecta is a genetically determined rare disease of the connective tissue, associated with abnormalities of type 1 collagen. The primary bone lesion is the lack of normal ossification of the endochondrial bone. Patients with osteogenesis imperfecta present several problems for anaesthetists. They have a tendency to develop malignant or non-malignant hyperthermia. During laryngoscopy and tracheal intubation, the mandible, teeth and cervical spine may be fractured or injured, and mucosal bruising or bleeding may occur. Renal or ureteral stones are common. The main problems are thus with airway control and intubation, and the risk of anaesthetic agents triggering malignant hyperthermia. We describe the successful anaesthetic management of a patient with osteogenesis imperfecta, undergoing nephrolithotomy and ureterolithotomy with total intravenous anaesthesia including propofol, remifentanil and cisatracurium, using an intubating laryngeal mask.