Sirolimus for pediatric cervicofacial lymphatic malformation: A systematic review and meta-analysis

Kamhieh, Yasmine, Mitra, Robin, Burnett, Thomas, Jones, Hugh, Roblin, Graham and Hall, Andrew 2024. Sirolimus for pediatric cervicofacial lymphatic malformation: A systematic review and meta-analysis. The Laryngoscope 134 (5) , pp. 2038-2047. 10.1002/lary.31091

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Abstract

Objective This study is a systematic review and meta-analysis of the efficacy and safety of sirolimus in the management of pediatric cervicofacial lymphatic malformations (LMs). Data Sources EMBASE, Medline, Scopus, and Cochrane databases were searched, along with the reference list of all included articles. Review Methods The study protocol was registered with PROSPERO and a systematic literature search strategy was designed and conducted with the aid of a medical librarian. All studies including case reports were included, with pooled analysis of raw data. A meta-analysis was conducted of magnetic resonance imaging (MRI), clinical, and airway outcomes. Results Thirteen case series and five individual case reports were included. Meta-analysis showed 78% (95% CI 57%–94%) of 62 patients had a reduction in LM volume, on MRI criteria, by 20% or more, and 32% (95% CI 11%–57%) had a reduction of 50% or more. Further meta-analysis showed 97% (95% CI 88%–100%) of 78 patients reported some clinical improvement on sirolimus. Sirolimus may be of particular value in management of airway LMs; out of 27 tracheostomy-dependent patients, meta-analysis showed 33% (95% CI 1%–78%) were decannulated after starting sirolimus. Individual patient meta-analysis on 24 individuals showed a statistically significant better response to sirolimus when initiated under the age of 2 years. Conclusion This review and meta-analysis support the efficacy of sirolimus in pediatric LMs of the head, neck, and airway. A large multi-center trial is needed to further explore its role and limitations. Laryngoscope, 134:2038–2047, 2024

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Medicine
Publisher:	Wiley-Blackwell
ISSN:	0023-852X
Date of First Compliant Deposit:	26 April 2024
Date of Acceptance:	11 September 2023
Last Modified:	29 Apr 2024 10:16
URI:	https://orca.cardiff.ac.uk/id/eprint/167949

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