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All studies including case reports were included, with pooled analysis of raw data. A meta\u2010analysis was conducted of magnetic resonance imaging (MRI), clinical, and airway outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirteen case series and five individual case reports were included. Meta\u2010analysis showed 78% (95% CI 57%\u201394%) of 62 patients had a reduction in LM volume, on MRI criteria, by 20% or more, and 32% (95% CI 11%\u201357%) had a reduction of 50% or more. Further meta\u2010analysis showed 97% (95% CI 88%\u2013100%) of 78 patients reported some clinical improvement on sirolimus. Sirolimus may be of particular value in management of airway LMs; out of 27 tracheostomy\u2010dependent patients, meta\u2010analysis showed 33% (95% CI 1%\u201378%) were decannulated after starting sirolimus. Individual patient meta\u2010analysis on 24 individuals showed a statistically significant better response to sirolimus when initiated under the age of 2\u2009years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This review and meta\u2010analysis support the efficacy of sirolimus in pediatric LMs of the head, neck, and airway. A large multi\u2010center trial is needed to further explore its role and limitations. <jats:italic>Laryngoscope<\/jats:italic>, 134:2038\u20132047, 2024<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.31091","type":"journal-article","created":{"date-parts":[[2023,10,9]],"date-time":"2023-10-09T18:50:09Z","timestamp":1696877409000},"page":"2038-2047","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Sirolimus for Pediatric Cervicofacial Lymphatic Malformation: A Systematic Review and Meta\u2010Analysis"],"prefix":"10.1002","volume":"134","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1219-8531","authenticated-orcid":false,"given":"Yasmine","family":"Kamhieh","sequence":"first","affiliation":[{"name":"ENT Department Noah's Ark Children's Hospital  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin","family":"Mitra","sequence":"additional","affiliation":[{"name":"Department of Statistics University College of London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Burnett","sequence":"additional","affiliation":[{"name":"Department of Mathematical Sciences University of Bath  Bath BA2 7AY UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugh","family":"Jones","sequence":"additional","affiliation":[{"name":"Cardiff University  Cardiff CF10 3AT UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graham","family":"Roblin","sequence":"additional","affiliation":[{"name":"ENT Department Noah's Ark Children's Hospital  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Hall","sequence":"additional","affiliation":[{"name":"ENT Department Noah's Ark Children's Hospital  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-016-3867-4"},{"key":"e_1_2_6_3_1","unstructured":"ISSVA classification for vascular anomalies \u00a92018 International Society for the Study of Vascular.Anomalies. 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