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CASE REPORT
Year : 2016  |  Volume : 17  |  Issue : 2  |  Page : 62-64

A child of Williams-Beuren syndrome for inguinal hernia repair: Perioperative management concerns


1 Department of General Anaesthesia, Institute of Critical Care and Anaesthesiology, Medanta - The Medicity, Gurgaon, Haryana, India
2 Department of Pediatric Surgery, Medanta - The Medicity, Gurgaon, Haryana, India

Correspondence Address:
Jyotirmoy Das
403, Aero View Heights, Sector 22, Dwarka, New Delhi - 110 077
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-0311.194268

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Williams-Beuren syndrome, commonly known as Williams syndrome (WS), is a multi-organ disorder. The principal anomalies of the syndrome are developmental delay, unusual craniofacial dysmorphic features, and cardiovascular anomalies such as valvular or supravalvular aortic stenosis, pulmonary artery stenosis, and coronary insufficiency. Sudden cardiac death during minor procedures even in the absence of gross cardiovascular pathology is the most dreaded complication in these patients. A 7-year-old child with WS was posted for left-sided inguinal hernia repair under general anesthesia. Our article describes the uneventful perioperative course of the patient and highlights the concerns and complications that may be an integral part with the syndrome.


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