{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T21:53:41Z","timestamp":1789682021401,"version":"4.0.1"},"reference-count":27,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2021,2,11]],"date-time":"2021-02-11T00:00:00Z","timestamp":1613001600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:sec><jats:title>Objective\/Hypothesis<\/jats:title><jats:p>To evaluate the efficacy of initial sirolimus therapy in the treatment of intractable head and neck lymphatic malformations (LMs) in children.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>Prospective open\u2010label study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this study, Twenty\u2010seven children diagnosed with LMs were given oral sirolimus as primary treatment over a minimum 6\u2010month trial. The major parameter to evaluate therapeutic outcome was percentage of lesion volume change compared with baseline. Average serum sirolimus concentrations, and adverse side effects, were monitored throughout the study period.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifteen girls and twelve boys, average age 27\u2009months (16\u2009days\u2013171\u2009months), constitute the study group. Treatment was deemed effective for twenty\u2010three participants, judged as fair in seven, good in nine, and excellent in seven. Two patients had minimal improvement, and two had increased volume to some degree. Effectiveness differed among LMs subtypes with responsiveness of macrocystic LMs exceeding that of microcystic LMs (<jats:italic>P<\/jats:italic>\u2009&lt;\u2009.05). Adverse drug reactions totaled 27 events in ten patients, the majority being mild with upper respiratory infections being most common.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Sirolimus as initial therapy is effective in decreasing lesion volume in intractable LMs in head and neck region, especially in macrocystic subtypes. Although most cases cannot be completely cured, side effects are few and tolerable.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4 <jats:italic>Laryngoscope<\/jats:italic>, 131:1902\u20131908, 2021<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.29419","type":"journal-article","created":{"date-parts":[[2021,2,14]],"date-time":"2021-02-14T04:14:42Z","timestamp":1613276082000},"page":"1902-1908","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Efficacy of Initial Sirolimus Therapy for 27 Patients with Intractable Lymphatic Malformations"],"prefix":"10.1002","volume":"131","author":[{"given":"Xuexi","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Otolaryngology, Head and Neck Surgery, Beijing Children's Hospital Capital Medical University, National Center for Children's Health (NCCH)  Beijing 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