ABSTRACT

Osteogenesis imperfecta is a rare , inherited genetic disorder of connective tissue which is characterized by abnormal Type I collagen production. This causes decrease in bone mass of human body, bones become fragile and brittle, resulting in unreasonable multiple fractures. Other manifestations include hyperextensibility of the joints, blue sclera, hearing loss, short stature and dentinogenesis imperfecta. During the anesthetic management of these patients, intubation difficulties, cervical spine fractures, and malignant hyperthermia triggered by used anesthetic agents may develop. Anatomic deformities and platelet dysfunction influence the choice of anesthetic technique. We aimed to present a series of 7 general anaesthetics in one child patient suffering from this disease along with the literature.