Successful treatment of steroid-resistant Kasabach-Merritt syndrome in infants with transcatheter arterial scleroembolization and vincristine

Abstract

Objective To evaluate the therapeutic effect and safety of transcather arterial scleroembolization(TASE) and vincristine for steroid-resistant Kasabach-Merritt in infants.Methods The clinical data of 17 infants with steroid-resistant Kasabach-Merritt syndrome treated between June 2007 and June 2013 in our hospital were reviewed retrospectively.Among the 17 cases,5 were located in the head and neck region,7 were located in the trunk and 5 were located in the limbs.The platelet count was all lower than 100× 109/L and the lowest was 3×109/L.After the diagnosis was made,transcather arterial scleroembolization (TASE) was performed in all 17 cases.At the time of angiography,pingyangmycin(PYM) and dexamethasone (DXM) dispersed in iodized oil contrast medium were injected into the lesions with a few minutes through the feeding artery followed by polyvinyl(PVA) pieces or absolute ethanol until the main feeding artery was embolized.Vincristine was injected through the feeding artery at the last.Besides,vincristine was used for systemic chemotherapy through intravenous injection once weekly after TASE.Four weeks after TASE,treatment effects were observed.It was necessary to perform TASE and vincristine therapy once again if the patient was not cured well.All cases were followed-up from 6 months to 2 years.Results Thirty-six courses of TASE+VCR treatment were performed in 17 cases.The success rate of operation was 100％.Fourteen patients were cured,2 improved and 1 died.The common complications were local blister or ulccr in five cases and hemangioma necrosis in one case.There was no bone marrow suppression,neurotoxicithy and cardiovascular and pulmonary complications.Conclusions The therapy of transcather arterial scleroembolization combined with vincristine is a safe,useful and effective method for steroid-resistant Kasabach-Merritt syndrome in infants and it is worthy of clinical application. Key words: Hemangioma; Sclerotherapy; Embolization,therapeutic ; Vincristine