Abstract
Purpose
A case of a patient associated with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis is presented.
Clinical features
Williams syndrome is a rare disease associated with a characteristic facies, supravalvular aortic stenosis, and mental retardation. A 15-yr-old girl with Williams syndrome underwent aortoplasty for supravalvular aortic stenosis. Anaesthesia was induced with fentanyl and thiamylal, and maintained with nitrous oxide, oxygen, sevoflurane, and continuous intravenous 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 management in a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis.
Objectif
Présenter le cas d’une patiente atteinte du syndrome de Williams et Beuren devant subir une aortoplastie pour une sténose aortique supravalvulaire.
Aspects cliniques
Le syndrome de Williams et Beuren est une maladie rare associée à un faciès caractéristique, une sténose aortique supravalvulaire et un retard mental. Une jeune patiente de 15 ans atteinte du syndrome de Williams et Beuren a subi une aortoplastie pour une sténose aortique supravalvulaire. L’anesthésie a été induite avec du fentanyl et du thiamylal et maintenue avec du protoxyde d’azote, de l’oxygène, du sévoflurane et une perfusion continue de fentanyl. Avant et après la chirurgie, on a évalué la sténose aortique supravalvulaire à l’aide d’une sonde d’échocardiographie transoesophagienne (ETO) multiplan.
Conclusion
LETO multiplan a été jugée très utile pour la gestion de l’anesthésie chez une patiente atteinte du syndrome de Williams et Beuren qui devait subir une aortoplastie pour une sténose aortique supravalvulaire.
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References
Williams JCP, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation 1961; 24: 1311–8.
Black JA, Bonham Carter RE. Association between aortic stenosis and facies of severe infantile hypercalcaemia. Lancet 1963; 2: 745–8.
Garabédian M, Jacqz E, Guillozo H, et al. Elevated plasma 1,25-dihydroxyvitamin D concentrations in infants with hypercalcemia and an elfin facies. N Engl J Med 1985; 312: 948–52.
Patel J, Harrison MJ. Williams syndrome: masseter spasm during anaesthesia. Anaesthesia 1991; 46: 115–6.
Pandian NG, Hsu T-L, Schwartz SL, et al. Multiplane transesophageal echocardiography. Imaging planes, echocardiographic anatomy, and clinical experience with a prototype phased array omniplane probe. Echocardiography 1992; 9: 649–66.
Putnam TC, Harris PD, Bernhard WF, Gross RE. The surgical management of congenital aortic stenosis. J Thorac Cardiovasc Surg 1964; 48: 540–55.
Wu Y-Q Sutton VR, Nickerson E, et al. Delineation of the common critical region in Williams syndrome and clinical correlation of growth, heart defects, ethnicity, and parental origin. Am J Med Genet 1998; 78: 82–9.
Mammi I, Iles DE, Smeets D, Clementi M, Tenconi R. Anesthesiologic problems in Williams syndrome: the CACNL2A locus is not involved. Hum Genet 1996; 98: 317–20.
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Kawahito, S., Kitahata, H., Kimura, H. et al. Anaesthetic management of a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis. Can J Anaesth 45, 1203–1206 (1998). https://doi.org/10.1007/BF03012465
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DOI: https://doi.org/10.1007/BF03012465