Sildenafil for Severe Lymphatic Malformations

Congenital chylothorax treated with oral sildenafil: a case report and review of the literature

“…3 The trial was conducted after a serendipitous observation in a child with an intrathoracic lymphatic malformation and idiopathic pulmonary hypertension. 4 The pulmonary hypertension was treated with sildenafil and during the course of treatment the lymphatic malformation reduced in size, then enlarged with discontinuation of sildenafil.…”

“…2 Recently, oral sildenafil, a specific inhibitor of phosphodiesterase-5, was reported to be effective in resolving non-pulmonary lymphatic malformations in infants and young children. 3,4 We report a case of CC in a late preterm infant with congenital pulmonary lymphangiectasia where octreotide was not effective, but management with oral sildenafil was successful.…”

Orbital lymphaticovenous malformations: Current and future treatments

“…170 Swetman et al reported the use of sildenafil for the treatment of a severe microcystic lymphatic malformation with intrathoracic extension in a 10-year-old girl. 175 They hypothesized that the therapeutic effect of sildenafil is through relaxation of smooth muscle in the malformation followed by cystic decompression. Alternatively, relaxation may cause secondary lymphatic spaces to open, or sildenafil may normalize lymphatic endothelial dysfunction.…”

Vascular Anomalies in Pediatrics

“…Many pharmacotherapies have been proposed for the treatment of vascular malformations [87][88][89]. Sirolimus (rapamycin) has emerged at the forefront.…”