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