Citation Information :
Krishnaswami S, Sharma V, Giri P, Rajappa D, Teja P. Williams–Beuren Syndrome in Adulthood for Hernia Repair: Anesthetic Challenges and Tutorial on Atrial Fibrillation. 2023; 2 (2):80-86.
Williams–Beuren syndrome (WBS) is characterized by the deletion of the long arm of chromosome 7q11.231, which encodes the elastin gene. This chromosomal abnormality leads to a complex neurodevelopmental disorder associated with dysmorphic facial and musculoskeletal features, congenital heart diseases, metabolic disturbances, and cognitive impairment. Structural cardiovascular abnormalities present in a majority of the cases may provide a potential risk of perioperative sudden cardiac death. A meticulous anesthetic technique is tailor-made to avoid increased myocardial oxygen demand with a focus on airway management, intravenous access, and anesthetic induction is employed to decrease the perioperative morbidity and mortality in WBS. Here, we present a case of the anesthetic management of a 35-year-old male with WBS who was posted for bilateral laparoscopic hernioplasty.
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