Chemotherapy and Surgical Approach with Repeated Endovascular Embolizations: Safe Interdisciplinary Treatment for Kasabach-Merritt Syndrome in a Small Baby
A 40-day-old female baby was referred to our division for an abdominal mass that had been diagnosed in another institution. Upon physical examination, she presented a pelvic mass extending to the gluteus and left thigh. Left lower-extremity paralysis was noted. Blood tests showed severe anemia and thrombocytopenia. In the following hours, her hematologic status worsened due to consumptive coagulopathy and the increasing volume of the mass. The CT scan done on admission showed an infiltrating pelvic mass (12 × 8 × 6 cm) extending through the sciatic foramen to the left gluteus and thigh (fig. ). The clinical aspect was pathognomonic of KMS.\nTransfusion, steroids and chemotherapy (vincristine, prednisone, rapamycin and cyclophosphamide) were started immediately. Coagulation parameters were normalized and the patient stabilized. Three months later, regression of the tumor volume (8 × 4 × 3 cm) was observed and interventional treatment became possible. Surgical dissection and ligature of the left internal iliac artery and sacral artery were performed. The main arterial supply of the pelvic mass was then embolized using a gelatin sponge (Spongostan Haemostatic®) under the fluoroscopy guidance of a portable C-arm tube in the operating room. At the control, complete regression of the pelvic mass and its vascularization were seen on the CT scan (fig. ). A clear sensitive-motor improvement was observed.\nThree months later, a second, similar, combined approach for the tumor occupying the left thigh was adopted. Surgical dissection of the left femoral artery and then partial embolization of the remaining tumor using a gelatin sponge (Spongostan Haemostatic) were successfully performed (fig. ). A third selective embolization of the arterial supply of the remaining tumor localized at the left gluteus region was planned for 2 months afterwards.\nA hybrid approach to the contralateral femoral artery was achieved in the angiographic room using Philips Allura flat-panel angiography. The right femoral artery was dissected and a 4-french, valved introducer was placed in the right common femoral artery. The contralateral, left lower-limb angiography done with a 4-french, shaped Cobra catheter (Glidecath, Terumo, Tokyo, Japan) showed the main supply of the hypervascular tumor in the gluteus (fig. ). Using a 2.7-french Echelon (EV3) microcatheter, selective catheterization of the profunda femoris artery feeders supplying the tumor was performed. Angiographic devascularization was achieved under fluoroscopy after injection of 0.8 ml of a nonadhesive, liquid glue, Onyx-18 (ethylene vinyl alcohol copolymer, Micro Therapeutics, Irvine, Calif., USA) (fig. ). Four months after treatment, the patient was stable.