title: Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery.
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.
authors.affiliation: Clinica di Rianimazione Generale, Respiratoria e del Trauma Maggiore, Azienda Ospedaliero-Universitaria "Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi", 60126 Ancona, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Boncagni
authors.affiliation: Anestesia e Rianimazione dei Trapianti e della Chirurgia Maggiore, Azienda Ospedaliero-Universitaria "Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi", 60126 Ancona, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Pecora
authors.affiliation: Clinica di Neurologia, Azienda Ospedaliero-Universitaria "Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi", 60126 Ancona, Italy.
authors.forename: Vasco
authors.initials: V
authors.lastname: Durazzi
authors.affiliation: Anestesia e Rianimazione Pediatrica, Azienda Ospedaliero-Universitaria "Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi", 60126 Ancona, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Ventrella