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VOLUME 2 , ISSUE 2 ( May-August, 2023 ) > List of Articles


Williams–Beuren Syndrome in Adulthood for Hernia Repair: Anesthetic Challenges and Tutorial on Atrial Fibrillation

Sharath Krishnaswami, Vaijayanthi Sharma, Pramod Giri, Deepak Rajappa, Pradeep Teja

Keywords : Anticipated difficult airway, Difficult airway, Obstructive sleep apnea, Williams syndrome, Williams–Beuren syndrome

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.

DOI: 10.5005/jp-journals-10089-0069

License: CC BY-NC 4.0

Published Online: 30-10-2023

Copyright Statement:  Copyright © 2023; The Author(s).


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.

  1. Pober BR. Williams-Beuren syndrome. N Engl J Med 2010;362(3):239–252. DOI: 10.1056/NEJMra0903074
  2. Boncagni F, Pecora L, Durazzi V, et al. Anesthesiological management of a patient with Williams syndrome undergoing spine surgery. Case Rep Anesthesiol 2016;2016:1371095. DOI: 10.1155/2016/1371095
  3. Hornik CP, Collins RT 2nd, Jaquiss RD, et al. Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg 2015;149(6):1516–22.e1. DOI: 10.1016/j.jtcvs.2015.02.016
  4. Matisoff AJ, Olivieri L, Schwartz JM, et al. Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review. Paediatr Anaesth 2015;25(12):1207–1215. DOI: 10.1111/pan.12775
  5. Twite MD, Stenquist S, Ing RJ. Williams syndrome. Paediatr Anaesth 2019;29(5):483–490. DOI: 10.1111/pan.13620
  6. Collins RT 2nd. Cardiovascular disease in Williams syndrome. Curr Opin Pediatr 2018;30(5):609–615. DOI: 10.1097/MOP.0000000000000664
  7. Olsen M, Fahy CJ, Costi DA, et al. Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution's experience. Anaesth Intensive Care 2014;42(5):619–624. DOI: 10.1177/0310057X1404200512
  8. Mervis CB, John AE. Cognitive and behavioral characteristics of children with Williams syndrome: implications for intervention approaches. Am J Med Genet C Semin Med Genet 2010;154C(2):229–248. DOI: 10.1002/ajmg.c.30263
  9. Kato S, Mizuno J, Ino K, et al. [Tracheal intubation in patients with Williams syndrome]. Masui 2010;59(5):632–634.
  10. Hindricks G, Potpara T, Dagres N, et al. 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS): The Task Force for the diagnosis and management of atrial fibrillation of the European Society of Cardiology (ESC) Developed with the special contribution of the European Heart Rhythm Association (EHRA) of the ESC. Eur Heart J 2021;42(5):373–498. DOI: 10.1093/eurheartj/ehaa612
  11. Krijthe BP, Kunst A, Benjamin EJ, et al. Projections on the number of individuals with atrial fibrillation in the European Union, from 2000 to 2060. Eur Heart J 2013;34(35):2746–2751. DOI: 10.1093/eurheartj/eht280
  12. Lip GYH. The ABC pathway: an integrated approach to improve AF management. Nat Rev Cardiol 2017;14(11):627–628. DOI: 10.1038/nrcardio.2017.153
  13. Arrigo M, Bettex D, Rudiger A. Management of atrial fibrillation in critically ill patients. Crit Care Res Pract 2014;2014:840615. DOI: 10.1155/2014/840615
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