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

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ABSTRACT

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.

How to cite this article

Krishnaswami S, Sharma V, Giri P et al. Williams–Beuren Syndrome in Adulthood for Hernia Repair: Anesthetic Challenges and Tutorial on Atrial Fibrillation. J Acute Care 2023;2(2):80–86.

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Most cited references 13

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OUP accepted manuscript

(2020)

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Projections on the number of individuals with atrial fibrillation in the European Union, from 2000 to 2060.

Bouwe P Krijthe, Anton Kunst, Emelia J Benjamin … (2013)

Since atrial fibrillation (AF) is associated with increased risks of cardiovascular and cerebrovascular complications, estimations on the number of individuals with AF are relevant to healthcare planning. We aimed to project the number of individuals with AF in the Netherlands and in the European Union from 2000 to 2060. Age- and sex-specific AF prevalence estimates were obtained from the prospective community-based Rotterdam Study. Population projections for the Netherlands and the European Union were obtained from the European Union's statistics office. In the age stratum of 55-59 years, the prevalence of AF was 1.3% in men (95% CI: 0.4-3.6%) and 1.7% in women (95% CI: 0.7-4.0%). The prevalence of AF increased to 24.2% in men (95% CI: 18.5-30.7%), and 16.1% in women (95% CI: 13.1-19.4%), for those >85 years of age. This age- and sex-specific prevalence remained stable during the years of follow-up. Furthermore, we estimate that in the European Union, 8.8 million adults over 55 years had AF in 2010 (95% CI: 6.5-12.3 million). We project that this number will double by 2060 to 17.9 million (95% CI: 13.6-23.7 million) if the age- and sex-specific prevalence remains stable. We estimate that from 2010 to 2060, the number of adults 55 years and over with AF in the European Union will more than double. As AF is associated with significant morbidities and mortality, this increasing number of individuals with AF may have major public health implications.

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Williams-Beuren syndrome.

Barbara Pober (2010)

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Author and article information

Journal

Journal ID (publisher-id): JAC

Title: Journal of Acute Care

Abbreviated Title: JAC

Publisher: Jaypee Brothers Medical Publishers

Publication date (Print): May-August 2023

Volume: 2

Issue: 2

Pages: 80-86

Affiliations

[1 ]Department of Neuroanaesthesia, Paras Hospitals, Gurugram, Haryana, India

[2,3,5 ]Department of Anaesthesia, Sakra World Hospital, Bengaluru, Karnataka, India

[4 ]Department of Neuroanaesthesia, Sakra World Hospital, Bengaluru, Karnataka, India

Author notes

Sharath Krishnaswami, Department of Neuroanaesthesia, Paras Hospitals, Gurugram, Haryana, India, Phone: +91 8867362494, e-mail: sharathkrishnaswami@ 123456gmail.com

Article

DOI: 10.5005/jp-journals-10089-0069

SO-VID: 3f695689-9ae0-400c-82e4-eed41226b447

License:

© The Author(s). 2023 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( https://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted use, distribution, and non-commercial reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 04 July 2023

Date accepted : 24 July 2023

Date: 30 October 2023

Custom metadata

file-pdf jac-02-080.pdf

ScienceOpen disciplines: Emergency medicine & Trauma

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

Data availability:

ScienceOpen disciplines: Emergency medicine & Trauma

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

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

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