after signing the informed consent, the patient becomes a participant in the study. the study is identified as an exploratory phase ii trial that will last up to five years.

the patient undergoes an initial assessment that records the size of the port‑wine macrocheilia, the colour of the port‑wine stain, and the quality of life using the ovama face and neck score.

measurements are taken with a caliper in three dimensions and the colour is classified into one of four categories: light red/pink, deep red, bluish red, or purple.

the patient begins oral therapy with sirolimus (commercial name rapamune). the prescribed dose is 5 mg per day.

the medication can be taken as a 1 mg/mL oral solution or as 2 mg coated tablets, according to the patient’s preference.

the treatment is taken once daily, by mouth, and will continue for the entire study period, up to 60 months.

if surgery is indicated, the patient undergoes the procedure while continuing sirolimus therapy.

the timing of the surgery is decided by the clinical team and is performed after the baseline assessment.

when appropriate, the patient receives laser treatment to the port‑wine stain, also while continuing sirolimus therapy.

laser sessions are scheduled according to standard clinical practice.

the patient attends scheduled visits, typically every three months, to monitor safety and adherence to the medication.

during each visit the clinician records any adverse events and checks that the patient continues the daily dose of sirolimus.

at 12, 24, 36, 48 and 60 months the patient undergoes the same measurements performed at baseline: volume of the macrocheilia, colour of the stain, and quality‑of‑life questionnaire.

the change from baseline is calculated for each time point to evaluate the effect of the treatment.

after the 60‑month visit the patient’s participation in the study ends.

the final data on volume reduction, colour change, and quality of life are compiled for analysis.