Sirolimus for Pediatric Cervicofacial Lymphatic Malformation : A Systematic Review and Meta-Analysis
© 2023 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:134 |
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| Enthalten in: |
The Laryngoscope - 134(2024), 5 vom: 29. Apr., Seite 2038-2047 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Kamhieh, Yasmine [VerfasserIn] |
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| Links: |
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| Themen: |
Cystic hygroma |
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| Anmerkungen: |
Date Completed 11.04.2024 Date Revised 11.04.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1002/lary.31091 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
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| 520 | |a © 2023 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc. | ||
| 520 | |a 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) | ||
| 520 | |a DATA SOURCES: EMBASE, Medline, Scopus, and Cochrane databases were searched, along with the reference list of all included articles | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
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