Background Williams Syndrome is a genetic condition occurring in approximately 1 in 10,000 live births. The deletion of genes in chromosome 7 affects tropoelastin, a protein involved in vascular wall formation. This results in a multisystem disorder predominantly affecting connective tissue, the central nervous system, and the cardiovascular system. Some surgical interventions can exacerbate haemodynamic changes and pose challenges for the anaesthetist when a patient with Williams Syndrome requires a general anaesthetic.

Case Report We present the case of a 7-year-old girl with Williams Syndrome undergoing angiography and angioplasty to her only kidney and explore the perioperative challenges for the anaesthetist. Prior to her transfer to Great Ormond Street Hospital, she had a prolonged hospital admission for control of hypertension and underwent neurosurgical intervention following an intracranial haemorrhage. Blood pressure was being controlled with seven antihypertensive medications. Echocardiogram showed left ventricular hypertrophy, severe left atrial dilatation, mitral stenosis and pulmonary hypertension. A cardio-stable induction was achieved with ketamine and total intravenous anaesthesia with propofol. An arterial line was sited in the right radial artery to monitor beat-to-beat changes in systemic blood pressure and non-invasive blood pressure monitoring sited on the leg. Blood pressure was optimised throughout the procedure using phenylephrine. Following a short recovery period, she was transferred back to the ward and was discharged two days after the procedure.

Conclusion Patients with Williams Syndrome undergoing general anaesthesia offer challenges to the anaesthetist. Careful consideration should be given to the cardiovascular implications of such cases and the approach to minimising changes in blood pressure.