Korean J Anesthesiol.  2005 Mar;48(3):311-314. 10.4097/kjae.2005.48.3.311.

Anesthetic Management of Two Patients with Williams Syndrome: Two cases reports

• Kwak HJ
• Kim JY
• Lee KC
• Kim JC
• Lee KH
• Kwak YL

• KMID: 2091981
• DOI: http://doi.org/10.4097/kjae.2005.48.3.311

Abstract

Williams syndrome is a rare genetic disorder with a frequency of one per 20,000-50,000 live births. It is caused by a deletion of one elastin allele located within chromosome subunit 7q11.23 (long arm). This syndrome is frequently accompanied by congenital heart disease, facial anomalies, and mental retardation and as a result, anesthetic management may be complicated by hemodynamic instability and difficult intubation. Also, as it was reported that this disease is associated with malignant hyperthermia, careful anesthetic selection is required. We experienced the anesthetic management of a 3-year-old girl with Williams syndrome who received dental treatment for severe dental caries under general anesthesia, and of a 4-year-old girl underwent aortoplasty for supravalvular aortic stenosis. Airway management and tracheal intubation was accomplished successfully in both patients. For anesthetic management, propofol, fentanyl and rocuronium, and fentanyl, isoflurane and vecuronium were used in the first and second patients, respectively. Surgeries were performed uneventfully in both cases, and the patients were discharged without complication.

Keyword

anesthesia; Williams syndrome

MeSH Terms

Airway Management
Alleles
Anesthesia
Anesthesia, General
Aortic Stenosis, Supravalvular
Child, Preschool
Dental Caries
Elastin
Female
Fentanyl
Heart Defects, Congenital
Hemodynamics
Humans
Intellectual Disability
Intubation
Isoflurane
Live Birth
Malignant Hyperthermia
Propofol
Vecuronium Bromide
Williams Syndrome*
Elastin
Fentanyl
Isoflurane
Propofol
Vecuronium Bromide