Anesthesia management for patients with congenital epidermolysis bullosa: A challenge case report for the anesthesiologist.

Autor: Gafsi B; Department of Anesthesia and Intensive Care, Monastir University Hospital, Monastir, Tunisia., Slimene AB; Department of Anesthesia and Intensive Care, Monastir University Hospital, Monastir, Tunisia., Bannour M; Department of Anesthesia and Intensive Care, Monastir University Hospital, Monastir, Tunisia., Bouguila M; Department of Anesthesia and Intensive Care, Monastir University Hospital, Monastir, Tunisia., Chaouch MA; Department of Visceral and Digestive Surgery, Monastir University Hospital, Monastir, Tunisia. Electronic address: docmedalichaouch@gmail.com., Toumi S; Department of Anesthesia and Intensive Care, Monastir University Hospital, Monastir, Tunisia.
Jazyk: angličtina
Zdroj: International journal of surgery case reports [Int J Surg Case Rep] 2024 Sep; Vol. 122, pp. 110091. Date of Electronic Publication: 2024 Jul 27.
DOI: 10.1016/j.ijscr.2024.110091
Abstrakt: Introduction and Importance: Congenital epidermolysis bullosa (EB) is a group of rare genetic conditions characterized by fragile skin that blisters easily. Anaesthesia management in these patients is complex due to mucocutaneous fragility, which can complicate surgical procedures and airway management. Our study aimed to report a case of successful general anaesthesia in two patients with congenital epidermolysis bullosa undergoing oesophagal dilatation.
Case Presentation: The case involved a brother and sister, aged 21 and 15 respectively, both of whom have congenital epidermolysis bullosa and presented with solid dysphagia due to double stenosis of the oesophagus. Anaesthesia management included meticulous preoperative planning, use of non-adhesive monitoring equipment, careful management with video laryngoscopy, and minimisation of skin trauma.
Clinical Discussion: Both patients underwent successful esophageal dilation under general anaesthesia without perioperative complications. Airway management was achieved in the first attempt using video laryngoscopy. Analgesia was effectively treated with paracetamol and tramadol.
Conclusion: This case illustrates the complexities and necessary precautions for anaesthesia management in patients with epidermolysis bullosa. Detailed preoperative evaluation, careful monitoring, and specific handling techniques can mitigate perioperative risks and ensure patient safety.
Competing Interests: Conflict of interest statement No conflict of interest to disclose.
(Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)
Databáze: MEDLINE