Lymphatic malformations

Lymphatic malformations are abnormal cystic dilatations of lymphatic vessels. They are present at birth, but often can only be detected later in life. Simple lymphatic malformations are limited to certain anatomical regions, while complex diffuse abnormaliti- es can affect the entire organism. Simple lymphatic malformations are divided into macrocystic (cysts >1cm) and microcystic (cysts <1cm). According to the ISSVA classification from 2015, the term lymphangioma is abandoned and the term lymphatic malforma- tion is introduced, which is intended to clearly indicate that it is not a tumor formation. The clinical course is characterized by long asymptomatic periods with short-term inflammatory or infectious episodes and episodes of intracystic bleeding. In the case of volu- minous changes, the diagnosis can be made at the transition from the second to the third trimester of pregnancy by ultrasound examination or fetal magnetic resonance. Ultrasound and magnetic resonance imaging are diagnostic methods of choice in the postpartum period as well. The purpose of the treatment is not to completely remove the formation, but to limit the symptoms to the extent that would preserve functionality and provide an appropriate appearance. Treatment methods are the expectant method for asymptomatic disease, sclerotherapy, surgical resection, drug treatment, laser treatment and physical therapy measures. When the- re is no life-threatening airway obstruction, sclerosing is the method of choice. Bleomycin as a sclerosant provides good results with mild side effects. Therapy with rapamycin (sirolimus) allows for a large percentage of reduction in formation, but it is important to monitor possible side effects.

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