1. Anesthetic Management Of A Child With William’s Syndrome Anesthetic Management Of A Child With William’s Syndrome Benjamin Salter, DO, Anuja Thakkar, DO, Ramez Salem, MD Department of Anesthesiology, Advocate Illinois Masonic Medical Center, Chicago, IL 60657 USA Discussion Abstract References Introduction This is the case of a 6 year-old male with Williams Syndrome who presented to our institution for eye muscle surgery. Williams Syndrome (WS) is a neuro-developmental disorder resulting from a deletion of a segment of chromosome 7 which results in abnormal expression and decreased quantity of arterial elastin in the vessel walls. The defining feature of the syndrome is an aortic narrowing at the level of the sinotubular junction; approximately 80% of patients with WS also have pulmonary artery stenosis. 1,4 The cardiovascular abnormalities associated with WS increase the patient’s risk of undergoing sedation and general anesthesia. Therefore, in preparation for the surgical procedure, we met with our patient and his family in the pre-anesthesia clinic. On the basis of this information, an anesthesia plan was formulated and while this anesthesia plan was not unusual, we exercised increased vigilance because of the patient’s condition. With intensive preparation and review, and consideration of the potential cardiac risk, general anesthesia was administered safely and effectively in a 6-year old male with Williams Syndrome. A thorough preoperative evaluation and consultation with the patient is essential in ensuring safe and effective anesthetic management. This case report describes the process as it relates to a pediatric patient with WS who presented to our institution for an ophthalmologic procedure. WS is a disorder with an incidence of 1:20,000 live births. 1 The syndrome is characterized by the presence of congenital supravalvular aortic stenosis and other significant cardiovascular, pulmonary, and metabolic implications. These patients are inherently at risk for developing myocardial ischemia and sudden cardiac death, particularly under sedation and general anesthesia. 2,3 WS is a rare neuro-developmental disorder resulting from a deletion of a segment of chromosome 7, which results in abnormal expression and decreased quantity of arterial elastin in the vessel walls. 1,2 The defining feature of the syndrome is an aortic narrowing at the level of the sinotubular junction; approximately 80% of patients with WS also have pulmonary artery stenosis. WS patients also possess other significant cardiovascular, pulmonary, and metabolic derangements. These patients are inherently at risk for developing myocardial ischemia and sudden cardiac death, particularly under sedation and general anesthesia. 1,4 Thus, WS patients requiring sedation or general anesthesia should not be treated as routine. The characteristic features of WS, such as mental retardation, elfin facies and a “cocktail personality,” should alert the anesthesiologist to its presence. 5 The pre- operative evaluation should be thorough and detailed and include a history of previous surgeries, including corrective operations of the aorta and pulmonary artery. A review of the cardiac history of the patient is necessary. Preoperative meetings with the WS patient and his family are helpful in gaining a more complete picture of the patient’s medical history. Prior to the operation, the entire anesthesia team should be cognizant of the major cardiac and pulmonary sequelae of WS and prepared for any complication. Intraoperative monitoring of vital organ function should be carried out with vigilance. The present case illustrates that with intensive preparation and review, as described above, and with consideration of the potential cardiac risks, general anesthesia can be administered safely and effectively in a pediatric patient with WS. 1.Burch TM, et al. Anesth Analg. 2008 Dec;107(6):1848-54. 2.Kussman, Barry. Journal of Pediatric Anesthesia. 2004. V. 17, N.3. 3.S. Sahin, et al. Internet Journal of Anesthes. 2007 V. 14 N. 1. 4.Stamm C, et al. Europ J Cardiothorac Surgery. 2001;19:195-202. 5.Lake, C, et al. Pediatric Cardiac Anesthesia. 2005 Image Taken From: Backer, C. Cardiothoracic Surgery Network. 2005 Image Taken From: Baylor College of Medicine. Pediatric Radiology Cases. A 6 year-old male with WS presented to our institution for resection of the right lateral rectus and bilateral medial recti muscles. Prior to the date of his operation, we met with the patient and his family for a preoperative evaluation. The patient was quite friendly and outgoing, which is a common characteristic of WS patients. We discussed the patient’s medical, surgical and anesthetic history thoroughly with his parents. Our interview revealed a history of pulmonary stenosis with surgical repair and two cardiac catheterizations under general anesthesia without any complications. A review of the patient’s medical documents confirmed his parent’s narrative. To obtain a more detailed medical history, we consulted the current and past evaluations of the patient’s cardiologist. Findings from pulmonary and cardiac catheterizations demonstrated gradients within the normal range and adequately functioning right and left-sided heart valves. The remainder of the patient's history was unremarkable. On physical exam, the patient weighed twenty kilograms and appeared healthy. A thorough head-and-neck examination revealed a Mallampati class II airway with full range of motion of his cervical spine and normally developed jaw and mandible with adequate mouth opening. A mild systolic murmur was auscultated and heart rate was regular in rate and rhythm. On the day of the operation, the patient’s medical history was again reviewed and, on the basis of an interview with his family, it was determined that his health had not changed since our previous meeting. The patient was mask induced with nitrous oxide, oxygen and sevoflurane. An IV was started and a size 2.5 flexible LMA was inserted. The following variables were monitored with vigilance: oxygen saturation, blood pressure, end tidal carbon dioxide, anesthetic agent, and electrocardiogram. These variables remained adequate throughout the case and the surgery proceeded uneventfully. The patient received 3 milligrams ondansetron, 3 milligrams dexamethasone, and 15 milligrams ketorolac. At the end of the surgery, the anesthetics were discontinued and the LMA was removed without incident. After thorough post-operative monitoring and assessment, the patient was discharged home. Case Report (Continued) Case Report