Kamhieh, Yasmine;
Mitra, Robin;
Burnett, Thomas;
Jones, Hugh;
Roblin, Graham;
Hall, Andrew;
(2023)
Sirolimus for Pediatric Cervicofacial Lymphatic Malformation: A Systematic Review and Meta-Analysis.
The Laryngoscope
10.1002/lary.31091.
(In press).
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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, 2023.
| Type: | Article |
|---|---|
| Title: | Sirolimus for Pediatric Cervicofacial Lymphatic Malformation: A Systematic Review and Meta-Analysis |
| Location: | United States |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1002/lary.31091 |
| Publisher version: | http://dx.doi.org/10.1002/lary.31091 |
| Language: | English |
| Additional information: | © 2023 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Medicine, Research & Experimental, Otorhinolaryngology, Research & Experimental Medicine, cystic hygroma, lymphatic malformations, pediatric, rapamycin, sirolimus, tracheostomy, EXPERIENCE, MANAGEMENT, THERAPY, HEAD |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > UCL BEAMS UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences > Dept of Statistical Science |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10188899 |
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