Pycnodysostosis: Anaesthetic Challenges

A rare autosomal recessive disorder Pycnodysostosis is to mutation of CTSK gene. This group of patients have fragile bone,craniofacial abnormalities with difficult airway. We are describing the anaesthetic management of a 12 yr male with pycnodosostosis for lower limb fracture fixation in combined spinal epidural anaesthesia.