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- Title
Sirolimus for Pediatric Cervicofacial Lymphatic Malformation: A Systematic Review and Meta‐Analysis.
- Authors
Kamhieh, Yasmine; Mitra, Robin; Burnett, Thomas; Jones, Hugh; Roblin, Graham; Hall, Andrew
- 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
- Subjects
LYMPHATIC abnormalities; RAPAMYCIN; MEDICAL librarians; MAGNETIC resonance imaging; LYMPHANGIOMAS
- Publication
Laryngoscope, 2024, Vol 134, Issue 5, p2038
- ISSN
0023-852X
- Publication type
Article
- DOI
10.1002/lary.31091