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Kasabach-Merritt syndrome (KMS) is a rare, life-threatening condition that ischaracterized by profound thrombocytopenia, hypofibrinogenemia, elevatedpartial thromboplastin time, and may also be associated with microangiopathichemolytic anemia. It is well established that this phenomenon is notablyassociated with the vascular tumors kaposiform hemangioendothelioma and tuftedangioma; however, recent literature has suggested its presence in the settingsof various vascular malformations (i.e. without neoplastic proliferation ofendothelial cells). This report focuses on a patient in the first year of life,who experienced a chronic, consumptive coagulopathy in the setting of a highlyvascular enteroatmospheric fistula. Sharing many features with theaforementioned syndrome, this anomaly suggests a novel association of theKasabach-Merritt phenomenon with a unique vascular malformation. Althoughpotentially lethal, Kasabach-Merritt syndrome can resolve with appropriatediagnosis and management; uncovering new associations can help to improverecognition and treatment in future cases.

Kasabach-Merritt syndrome arising from an Enteroatmospheric Fistula