Anaesthetic considerations in Klippel-Feil syndrome

Anaesthesia for the repair of a large occipito cervical encephalomyelocele in a neonate with Kippel-Feil syndrome is described. The fusion of the cervical spines, a short neck, low posterior hair line and Sprengel's deformity, which were present in this patient, collectively indicated Klippel-Feil syndrome. In addition to the usual stigmata of the syndrome, this patient had a large encephalomyelocele and persistent patent ductus arteriosus complicated by congestive heart failure. Patients with this syndrome are vulnerable to cervical spinal cord injury and are at high risk for neurological injury not only during laryngoscopy and intubation but thereafter. Implications of Kippel-Feil syndrome for the anaesthetist are reviewed and discussed.