Management of patients with complex vascular anomalies (VAs) is often associated with significant morbidity and mortality because of the lack of effective treatment modalities that may lead to significant improvement of the disease and/or healing. Recently, reports of treatment of patients with complex VAs with sirolimus revealed encouraging results. Sirolimus inhibits the mammalian target of rapamycin, which acts as a master switch of numerous cellular processes. We report a successful use of sirolimus for the treatment of a patient with a complex CLVM of the trunk and the right lower extremity believed to be untreatable. Our patient had 44 hospitalizations during the 10-year period, with various unsuccessful treatments and continuous deterioration of his clinical condition, ending up in a wheelchair. His condition reversed to normal everyday activities 9 months after initiation of sirolimus therapy. We conclude that sirolimus is a very promising therapeutic option for children with complex VAs of capillary-lymphatico-venous type.

Le malformazioni linfatiche (LMS) sono masse cistiche benigne derivanti dallo sviluppo anormale dei canali linfatici. Le malformazioni linfatiche interessano principalmente il distretto testa-collo. L’asportazione chirurgica di malformazioni linfatiche è seguita da un alto tasso di recidiva e vi è un elevato rischio di complicanze. La bleomicina è un farmaco antineoplastico approvato. Tale farmaco può essere usato come agente sclerosante nelle anomalie vascolari. Presentiamo il caso di un bambino sottoposto a quattro interventi chirurgici senza successo, complicati da una paralisi periferica del nervo faciale. La malformazione linfatica è stata da noi trattata con successo con la somministrazione intralesionale di bleomicina.

Mirror foot is a very rare congenital anomaly, with only a few papers presenting definitive treatment for this entity. There are limited management recommendations. Most cases are treated before walking age. In our case, there were no associated developmental defects of the leg. The child underwent complex rays resection with medial foot reconstruction. After 7.5 years of followup, definitive surgical treatment was performed with satisfactory cosmetic and functional outcome.