405 Kasabach-Merrit Syndrome in Giant Hemangioendothelioma of the Liver in a Preterm Infant

Abstract

Kasabach-Merrit syndrome, the association of a vascular lesion and consumptive coagulopathy represent a diagnostic and therapeutic challenge to clinicians. We report a case of an infant with a large mass of the liver diagnosed at 25 weeks of amenorrhea that started to bleed at 33 weeks of gestational age. A male newborn, 33 GW, 3170 gr of weight, was admitted to the neonatal intensive care unit for severe consumption coagulopathy caused by hepatic mass prenatally diagnosed. At the admission the clinical conditions were severe and so we decided for surgical treatment. He was then operated on at 3 hours of life. The operation was a total excision of the tumor with a left-side hemihepatectomy. Macroscopically the tumor was brown, encapsulated, elastic in consistency. The weight was 200 gr and the diameter 9 cm. Histological examination revealed an infantile hemangioendothelioma of the liver. The infant received a pre and postoperatively supportive care with transfusions of fresh frozen plasma and red blood cells and infusion protein C i.v. To stop the activation of coagulopathy. The postoperative course was uneventful. At 6 months after surgery the patient had no evidence of residual tumor or further coagulopathy. In 1940, Kasabach and Merrit first described the association of a large vascular tumor and trombocytopenia and termed this Kasabach-Merrit syndrome. This is a potentially life-threatening condition, with mortality estimates ranging from 20 to 30% as a result of severe sepsis, coagulopathy, or invasion of vital organs. Treatment modality have included corticosteroids, interferon alfa 2a or 2b, vincristine, cyclophosphamide, ticlopidine, aspirin, amicar, radiation therapy, and surgical excision. In our case the treatment of this life-threatening condition was aggressive surgery at birth associated to medical treatment of coagulopathy. To our knownledge our patient is the younger one in literature who underwent surgery for this lesion.