The most common congenital lymphatic malformation is lymphangioma or cystic hygroma with an incidence of one in 6000 pregnancies. Multiple dilated cysts of lymphatic fluid due to absence of adequate vein drainage develop in the head and neck region, followed in order of preference with the axilla. Lymphangiomas cause cosmetic and functional complications. Management of lymphatic malformations can include observation, sclerotherapy (using K-432, Bleomycin or Doxycycline), or surgical excision. An oral herbal medicine called Eppikajutsuto (TJ-28) is utilized to reduce and eliminate excessive fluid in patient with inflammatory joint disorders and edema caused by nephritis and nephrotic syndrome. The ephedra herb is the main ingredient in Eppikajutsuto and is known to induce pharmacologic effects beyond its sympathomimetics activities such as antiinflammatory, antianaphylactic, antimicrobial and antihistamine effects. Pseudoephredine, a component of the ephedra herb, has inhibitory effects on acute inflammation. TJ-28 produces an inhibitory effect on fibroblast proliferation along with inhibition of prostaglandin E2 biosynthesis as antiinflammatory effect. The main mechanism of action of TJ-28 is by suppressing the activity of vascular endothelial growth factor (VEGF) by inhibiting the synthesis of prostaglandin E2 and cyclooxygenase. Ephedra herbal treatment should not be used in elderly patients or individuals with ischemic heart disease or low appetite because of the risk of tachycardia and hypertension. TJ-28 reduces the accumulation of lymphatic fluid in lymphatic malformations. TJ-28 treatment has led to regression of lymphangiomas in the head, neck, shoulder, retroperitoneum and mediastinum with a response rate above 80% after six months of treatment. TJ-28 can be combined with sclerotherapy and/or surgery. The microcystic or combined variant of the lymphangioma seems to respond better than the macrocystic type-lesions. Difficult located lymphatic lesions can also be managed with TJ-28.
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