Anaesthetic management of a case of osteogenesis imperfecta with bladder outlet obstruction: a case report

Abstract

Osteogenesis Imperfecta (OI) is a fibro-osseous disorder of the collagen tissue which leads to defects in the skeletal growth and short stature. OI poses various anaesthetic challenges, which include difficult intubation, problems with positioning and a tendency to develop malignant hyperthermia, coagulopathy and cardiovascular abnormalities. We describe the anaesthetic management of an 18 year old boy, a known case of OI with kyphoscoliosis, who presented with acute retention of urine due to urethral stone and was successfully managed for emergency suprapubic cystolithotomy with caudal anaesthesia. He had characteristic features like short stature, short neck, blue sclera, a receding mandible, pectus carinatum (pigeon chest), hyper mobile joints, kyphoscoliosis and a history of recurrent hospitalization for long bone fractures. Since our patient presented for an emergency surgery, we had only little time to evaluate and plan out the management, which posed significant challenges. A successful outcome was ensured by the carefully obtained history, examination and gentle care, with the application of basic principles in managing this patient