Experience in the Treatment of Lymphatic Vascular Malformations with Sirolimus, A Prospective Cohort Study in a Tertiary Hospital

Abstract

Introduction: Lymphatic vascular malformations (LVM), previously known as lymphangiomas, are rare congenital malformations, generally diagnosed in the first months of life. Although they are benign, they produce significant morbidity and sometimes mortality from compression. They have a poor response to existing treatments, so Sirolimus began to be used. The objective of this work is to describe the response of LVM patients treated with Sirolimus
in a 3rd level hospital in Mexico.

Methodology: A prospective cohort-type study was carried out. Patients from birth to 5 years -old with LVM were included. Patients with immunosuppressive diseases or who received other prior treatment were excluded. Those who completed less than 6 months of treatment due to loss of follow-up were eliminated. Of the total of 32 patients: 62.5% were male and 37.5% female. LVMs were located in the neck 46.8%, head: 21.8%, thorax: 25%, abdomen: 3.1% and extremities: 3.13%. They were macrocystic 59.3%, mixed: 31.2% and microcystic 9.3%. Average age of diagnosis: 1.3 years -old, and size: 101 cc. All received treatment for at least 6 months and up to 1 year. Favorable responses were observed in 12 (63%) patients with macrocystic LVM, 3 (30%) mixed
and 1 (33%) microcystic. There were no significant differences between the type of LVM, nor its anatomical location. 31% of the LVM had minor adverse events. The Sirolimus response was similar to a previous study with OK-432.

We find 50% of the lesions presented a decrease greater than 90%, and 84.4% of all lesions presented a decrease in size. Sirolimus is a tolerable treatment