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.