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Case Reports in Anesthesiology
Volume 2016, Article ID 1371095, 4 pages
http://dx.doi.org/10.1155/2016/1371095
Case Report

Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery

1Clinica di Rianimazione Generale, Respiratoria e del Trauma Maggiore, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, Italy
2Anestesia e Rianimazione dei Trapianti e della Chirurgia Maggiore, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, Italy
3Clinica di Neurologia, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, Italy
4Anestesia e Rianimazione Pediatrica, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, Italy

Received 16 December 2015; Accepted 1 March 2016

Academic Editor: Maria Jose C. Carmona

Copyright © 2016 Federico Boncagni et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Williams Syndrome (WS) is a complex neurodevelopmental disorder associated with a mutation on chromosome 7. Patients with WS usually display dysmorphic facial and musculoskeletal features, congenital heart diseases, metabolic disturbances and cognitive impairment. Structural cardiovascular abnormalities are present in the majority of the children and may provide a substrate for perioperative Sudden Cardiac Death, as presented by several reports, something that creates a great challenge to the anesthetic conduct. We present the case of a 12-year old girl who required anesthetic care for surgical correction of an acquired kyphoscoliosis. Potential anesthesiological implications of WS are subsequently reviewed.