Transarterial Embolization for Medically Refractory Kasabach-Merritt Syndrome: A Case Report

Kasabach-Merritt syndrome (KMS) is a life-threatening disorder characterized by severe thrombocytopenia and consumptive coagulopathy associated with vascular tumors, typically Kaposiform hemangioendothelioma (KHE) or tufted angioma. Drug therapy is the first-line treatment, but some cases are refractory, and no established salvage therapy exists. This report describes a case of KMS resistant to multiple drug therapies in which transarterial embolization (TAE) was successfully performed.

A 2-month-old male infant presented with a left posterior neck mass. Examination revealed severe thrombocytopenia (5000/μL) and coagulation abnormalities. Imaging studies diagnosed KMS with KHE. Platelet counts did not improve despite sequential treatment with corticosteroids, beta-blockers, and sirolimus. TAE was indicated due to the high risk of fatal bleeding. Angiography identified feeding vessels from the posterior muscular branch of the vertebral artery and the ascending cervical artery. TAE was performed using 20% n-butyl-2-cyanoacrylate (NBCA) for the vertebral artery branch and gelatin sponge particles for the ascending cervical artery branch. Post-embolization, platelet counts normalized, and the tumor shrank.

In a case of drug-resistant KMS, TAE effectively restored platelet counts and prevented fatal complications. When performed with appropriate embolic selection and careful complication management, arterial embolization can be a useful adjunctive treatment.