KASABACH-MERRITT-SYNDROME OF THE LEG ASSO CIATED WITH OSTEOLYSIS (SIGN OF GORHAM)

Introduction. Kasabach-Meritt syndrome and Gorham's sign are two uncom mon and severe, sometimes life-threatening, complications in infants w ith vascular lesions. Their association has been described in rare cas es. Case report. An infant with a vast congenital angiomatous lesion i ncluding an extensive lymphatic component, developed active regional o steolysis then suddenly suffered disseminated intravascular coagulatio n of the leg. Medical treatment was unsatisfactory. After unsuccessful use of low molecular weight heparin, pentoxifyllin and alpha interfer on. amputation of the leg was required to avoid a fatal outcome. Discu ssion. Kasabach-Merritt syndrome does not develop on classic immature hemangiomas, despite some contradictory statements in the literature. In our case, a complex tumor developed in association with a lymphatic malformation. The association of Kasabach-Merritt syndrome with osteo lysis (Gorham's sign) does not appear to be fortuitous. Therapeutic ma nagement of these severe complications is difficult and requires case by case analysis.