Anaesthetic management of a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis

Purpose: A case of a patient associated with Williams syndrome undergoing a ortoplasty for supravalvular aortic stenosis is presented. Clinical features: Williams syndrome is a rare disease associated with a ch aracteristic facies, supravalvular aortic stenosis, and mental retardation. A 15-yr-old girl with Williams syndrome underwent aortoplasty for supraval vular aortic stenosis. Anaesthesia was induced with fentanyl and thiamylal, and maintained with nitrous oxide, oxy gen, sevoflurane, and continuous in travenous infusion of fentanyl. Supravalvular aortic stenosis was evaluated using a multiplane transesophageal echocardiography (TEE) probe before and after repair. Conclusion: Multiplane TEE was found to be very useful for anaesthetic mana gement in a patient with Williams syndrome undergoing aortoplasty for supra valvular aortic stenosis.