Díaz FLA, Sarmiento L, Barrera FM, Asz SJ

Language: Spanish
References: 7
Page: 227-231
PDF size: 278.73 Kb.

ABSTRACT

Williams-Beuren syndrome is a congenital disease characterized by craniofacial, cardiovascular and metabolic disorders. It occurs as a result of a deletion of chromosome 7q11.23, alteration affecting the gene responsible for elastin synthesis, leading to cardiovascular system diseases such as aortic supravalvular stenosis, stenosis of pulmonary vessels and coronary diseases. The latest publications in the literature are reports of cases in which patients with this diagnosis have suffered sudden death, most of them in anesthetic events. We present a pediatric patient with a diagnosis of Williams-Beuren syndrome who underwent urgent inguinal hernia repair under regional anesthesia with epidural block and sedation with propofol infusion, achieving hemodynamic stability without impact on coronary perfusion. The choice of anesthetic technique was proposed based on the cardiovascular physiology and the surgical procedure. Although there are insufficient data to recommend a specific anesthetic technique in these patients, a prudent approach would involve special attention to the balance between supply and demand of myocardial oxygen.

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