Sedation for Magnetic Resonance Imaging in a child affected with Williams syndrome

Williams syndrome (WS) is a rare genetical disorder with an incidence of 20-50 000 live births. It is caused by a delation of 1 elastin allele located within chromosome subunit 7q11.23 (long arm). It is characterized by: supravalvular aortic stenosis, multiple peripheral pulmonary artery stenosis, a typical face (elfine face), mental and statural deficiency, characteristic dental malformation, transient hypercalcemia that occurs during the 1(st) year of life. We present the case of a 7-month-old infant affected with WS. In order to clarify the cardiac findings, the baby under-went a MRI investigation, requiring an anesthesiological assistance. In this case a deep sedation approach was carried out by giving chloral hydrate 10% per os (80 mg/kg). We did not perform a general anesthesia in order to avoid the risk related to it. No other drugs were used. During the procedure SpO(2), HR, RR, and ETCO(2) were in normal range; ST tract analysis did not reveal any pathological change The examination lasted 95 minutes; at the end the baby was kept under surveillance in the recovery room for 30 minutes, he could be precociously fed. All the procedure was uneventful.