• Pediatric dermatology · Jul 2011

    Case Reports

    Capillary-lymphatic malformation, kaposiform hemangioendothelioma and delayed Kasabach-Merritt phenomenon.

    • Jesús del Pozo, Juan Carlos López-Gutiérrez, Manuel Gómez-Tellado, Manuel Nistal, Rafaela Soler, Felipe Sacristán, and J A Tovar.
    • Unit of Pediatric Vascular Anomalies, Department of Dermatology, CHU A Coruña, Spain. jesus.del.pozo.losada@sergas.es
    • Pediatr Dermatol. 2011 Jul 1; 28 (4): 439-43.

    AbstractAccording to International Society for the Study of Vascular Anomalies classification, vascular anomalies are mainly divided into two groups: vascular tumors and vascular malformations. Nevertheless, a small group of patients present clinical and/or histopathologic overlapping features. We report a case of a 4-month-old boy that presented a vascular lesion on his right buttock with involvement of abdominal wall muscles, abdominal cavity and drainage to primitive iliac by a tortuous drainage vein. Surgery was performed and histopathology demonstrated a combined vascular malformation. Six months later he developed a thrombocytopenia and repeat magnetic resonance imaging revealed a new solid mass involving the majority of the abdominal cavity. At 18 months of age the patient developed a Kasabach-Merrit phenomenon and treatment with vincristine, interferon and then acetyl-salicilic acid and dypiridamol was started. No response in platelet counts was obtained and one more surgery was perfomed. On this occasion a histopathologic study revealed vascular malformation areas intermingled with areas of kaposiform hemangioendothelioma. This patient demonstrates the Kasabach-Merritt phenomenon with kaposiform hemangioendothelioma arising within a pre-existing combined vascular malformation.© 2010 Wiley Periodicals, Inc.

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