1Aliyya Rifki, M.D., 1Prijo Sidipratomo, 1Jacub Pandelaki

1Division of Interventional and Cardiovascular Radiology, Department of Radiology, Cipto Mangunkusumo National Central General Hospital, Universitas Indonesia, Central Jakarta, Dki Jakarta, Indonesia

Background:

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor according to ISSVA Classification, with a prevalence of fewer than 0,9 cases per 100.000 children. When presented with life-threatening thrombocytopenic coagulopathy, known as Kasabach – Merritt Phenomenon (KMP), the mortality rate increased up to 24% due to massive hemorrhage. Its rarity causing the hemorrhagic management guidelines to remain limited. We hereby report a case of extensive KHE bleeding in a patient with KMP, successfully managed by an Interventional Radiologist through a transarterial embolization procedure.

Case Report:

A 3-month-old infant was referred due to a spontaneous massive hemorrhage on a known KHE of the left lower leg. Laboratory tests revealed anemia (Hb 8.1 g/dL) and profound thrombocytopenia (thrombocyte 4000/uL), suggestive of KMP. Left cruris Doppler ultrasound revealed a hypervascular mass with dilatation of the left femoral artery, femoral vein, and its superficial branches. An emergency selective angiography through the anterior tibia artery was performed prior to embolization using Gelfoam. Vascularization of the tumor was markedly reduced and the bleeding was deceased. The coagulation profile was rapidly improved following embolization (Hb 11.0 g/dL, thrombocyte 58,000/uL) and there were no complications reported until the patient was discharged.

Discussion:

In KHE with KMP, radical surgery is unfavorable due to the high possibility of worsening coagulopathy, causing an increased risk of hemorrhage, both locally and systemically. An emergency angiography followed by transarterial embolization has shown effectiveness in  KHE bleeding control by decreasing blood flow from the tumor while also producing a rise in platelet level and a decrease in the size of the mass. However, data on its clinical success and long term complications are scarce and need further study

Conclusion(s):

Transarterial embolization was safe and effective management in the acute setting of KHE bleeding presented with KMP.