Perioperative management of a parturient with VACTERL association for a caesarean section

A parturient with VACTERL association (vertebral defects, anal atresia, cardiac defects, trachea-oesophageal fistula, renal abnormalities and limb abnormalities) was listed for an elective caesarean section. She had a short neck with reduced cervical extension and flexion. Magnetic resonance imaging of her whole spine was performed which showed failure of cervical spine segmentation and cervical stenosis. Neuraxial blockade could have resulted in unpredictable spread of local anaesthetic due to the low volume of the spinal canal, and could have caused myelopathic changes within the spinal cord due to cerebrospinal fluid pressure changes. A general anaesthetic using a rapid sequence induction was also predicted to be challenging due to her fixed, unstable neck and severe cervical spine stenosis. After a multidisciplinary discussion Including neurosurgeons, we planned for awake tracheal intubation followed by general anaesthesia. However, before the date of her planned delivery, she required an urgent caesarean section due to severe preeclampsia. This was performed under general anaesthesia following uncomplicated awake tracheal intubation.