Anaesthetic considerations in paediatric klippel feil syndrome- A case report

Abstract

Klippel feil syndrome is a rare skeletal disorder primarily characterized by abnormal union or fusion of two or more cervical vertebrae which is often associated with cervical canal stenosis. This results in a short neck, a low hairline at the back of the head, a limited range of motion in the neck and infrequently an atlanto-axial instability.Here we report a case of type 3 paediatric Klippel Feil syndrome who underwent Sprengel deformity repair in prone position and its anesthetic implications. A careful assessment of airway and an algorithmic approach towards management is needed in children with KFS. The various spine deformities associated with KFS demand careful positioning and a reconfirmation of appropriate ETT position, airway pressures and lung compliance is recommended in these children.