[Kasabach-Merritt syndrome with pancreatic hemangioma in an infant].

Abstract

Background: Kasabach-Merritt syndrome is characterized by one or more large hemangiomas associated with thrombocytopenia due to platelet trapping. The hemangiomas may be located in the abdominal viscera, in which case, treatment may be difficult.

Case report: A 2 1/2 month-old boy was admitted because of the discovery of an abdominal mass. He was anemic (hemoglobin: 6.8 g%; reticulocytosis: 288,000/mm3), thrombocytopenic (50,000/mm3), hypofibrinogenemic (0.95 g/l), with fibrin split products in the blood. Ultrasonography showed that this mass was retroperitoneal and heterogeneous. X-rays delineated the mass, which distorted the duodenal loop and pressed forward against the stomach. Surgery showed that the mass included the pancreas, the root of mesenterium and the extrahepatic bile duct. This mass was biopsied. Histological examination showed infantile type hemangioendothelioma. Abdominal angiography showed that the mass was not very hypervascularized, and there were no dilated supplying blood vessels. An aortogram showed two other small vascularized areas. The patient was given methylprednisolone, but the volume of the mass remained unchanged, while hepatosplenomegaly and jaundice developed and ultrasound showed dilated extra- and intrahepatic biliary ducts. The patient was then given interferon alpha 2b for 1 month. Embolization of the small supplying arteries was performed because of a lack of improvement after 1 week of treatment. This procedure was followed by rapid disappearance of the signs of consumption coagulopathy, a progressive improvement in cholestasis, and decrease in the mass.

Conclusion: Improvement in manifestations of this Kasabach-Merritt syndrome is difficult to assign to one of the several therapies. The rapid disappearance of consumption coagulopathy after arterial embolization suggests that this treatment was successful in our patient.