分析总结外科手术联合口服西罗莫司治疗儿童口腔颌面部淋巴管畸形（LM）的治疗经验。

收集2019年1月至2022年12月广州市妇女儿童医疗中心口腔颌面外科病房收治的外科手术联合口服西罗莫司治疗口腔颌面部LM患儿共15例，对患儿基本情况、首次治疗方案疗效、组织分型与疗效和不良反应进行分析，并通过文献检索，探索并总结本中心在儿童口腔颌面部LM的序贯治疗的经验。

共14例患儿首次治疗方案选择西罗莫司，疗效评价无病例完全缓解（CR）；部分缓解（PR）9例（64.29%），均为混合型LM；无反应（AR）5例（35.71%），其中混合型LM 2例、微囊型LM 3例。1例首次治疗方案选择外科切除，随诊可见患儿舌及口底基本恢复正常形态及功能，疗效评价为CR。15例患儿中，1例可疑西罗莫司药物不良反应，表现为反复发热及肺部感染。

西罗莫司可作为气道高风险的头颈部LM患儿的治疗选择；该药物治疗LM有局限性，需要联合其他治疗以提高治疗效果；对于无明显症状的微囊型LM，西罗莫司不作为首选推荐；西罗莫司所致不良反应与年龄无关，呈现剂量相关性，年龄越小剂量越低，应密切监测血药浓度，及时调整药物剂量，以减少不良反应的发生。LM的治疗仍然需要临床医师根据患儿的实际情况制定个性化的序列治疗方案。

To analyze and summarize the treatment experiences of surgical treatment combined with oral sirolimus for lymphatic malformations (LM) in children′s oral and maxillofacial region.

Medical records and imaging data of children diagnosed with LM in oral and maxillofacial region admitted to the oral and maxillofacial surgery ward of Guangzhou Women and Children′s Medical Center from January 2019 to December 2022 were collected. The patients′ information, therapeutic effect of the first treatment, therapeutic effect between different types of classification, and sirolimus-induced adverse drug reactions in pediatric patients were analyzed, and a literature search was conducted.

A total of 14 patients were treated with sirolimus for the first time, and none of them had complete remission (CR) . Partial response (PR) accounted for 64.29% (nine cases) , all of them were mixed LM, and none were microencapsulated LM. No response (AR) accounted for 35.71% (five cases) , and all the three microencapsulated LM cases were AR. Surgical resection was selected for the first treatment of one case, and basically returned to the normal form and function, and it was evaluated as CR. One of the 15 patients was diagnosed as suspected sirolimus-induced adverse drug reactions of sirolimus with repeated fever and pulmonary infection.

Sirolimus can be used as an option in the management of LM patients with life-threatening upper airway. The drug has limitations in the treatment of LM and needs to be combined with other treatments to improve the therapeutic effect. Sirolimus is not recommended as the first choice for microencapsulated LM without obvious symptoms. The sirolimus-induced adverse drug reactions caused by sirolimus was not related to age, but it showed dose correlation. The younger the age, the lower the dose. Close monitoring of blood concentration and timely dose of sirolimus could reduce the occurrence of sirolimus-induced adverse drug reactions. The treatment of LM still requires surgeons to develop individualized sequential treatment regimens for each patient.