Anesthetic considerations in children with Hutchinson‐Gilford progeria syndrome: A narrative review. Issue 5 (17th March 2020)

• Record Type: Journal Article
• Authors: Nijs, Kristof; Van de Velde, Marc; Hoogma, Danny
• Editors: Veyckemans, Francis
• Is Part Of: Paediatric anaesthesia. Volume 30:Issue 5(2020:May)
• Page Start: 537
• Page End: 543
• Publication Date: 2020-03-17
• Subjects: anesthesia -- Hutchinson‐Gilford progeria syndrome -- progeria; Pediatric anesthesia -- Periodicals; 617.96798
• DOI: 10.1111/pan.13847
• Languages: English
• ISSNs: 1155-5645

Abstract

Abstract: Background: Hutchinson‐Gilford progeria syndrome is a rare disease in childhood that results in premature aging. The presence of multisystem derangements including skin, bone, and joint diseases and possibly a difficult airway makes the anesthetic management challenging. Because of the extremely low prevalence, experience is limited even for experienced pediatric anesthesiologists. Objective: To review the available literature on anesthesia for patients with Hutchinson‐Gilford progeria syndrome and to give recommendations for establishing the best practice for patients with Hutchinson‐Gilford progeria syndrome. Design: A narrative review of the rare existing literature. Date sources: CENTRAL (Cochrane), EMBASE, Google Scholar, MEDLINE and PubMed. Eligibility criteria: Articles addressing anesthesia in patients with Hutchinson‐Gilford progeria syndrome were included. Results: An overview of the current literature was made on anesthesia care for patients with Hutchinson‐Gilford progeria syndrome. After screening the literature, only ten articles were found to be of interest and include some case reports and a correspondence. The focus points on how to perform anesthesia care in patients with Hutchinson‐Gilford progeria syndrome and the entire perioperative care are suggested. The available data are limited and results need to be interpreted with caution. Conclusion: The patients with Hutchinson‐Gilford progeria syndrome are not just "frail" patients. Awareness concerning intubation difficulties is mandatory, and airway strategies must be addressed in advance. Although these patients present with the physiology of an elderly with accompanying comorbidities, emotionally they are only children and should be approached as such.