  Kasabach-Merritt syndrome (KMS) is a rare, life-threatening condition that is
characterized by profound thrombocytopenia, hypofibrinogenemia, elevated
partial thromboplastin time, and may also be associated with microangiopathic
hemolytic anemia. It is well established that this phenomenon is notably
associated with the vascular tumors kaposiform hemangioendothelioma and tufted
angioma; however, recent literature has suggested its presence in the settings
of various vascular malformations (i.e. without neoplastic proliferation of
endothelial cells). This report focuses on a patient in the first year of life,
who experienced a chronic, consumptive coagulopathy in the setting of a highly
vascular enteroatmospheric fistula. Sharing many features with the
aforementioned syndrome, this anomaly suggests a novel association of the
Kasabach-Merritt phenomenon with a unique vascular malformation. Although
potentially lethal, Kasabach-Merritt syndrome can resolve with appropriate
diagnosis and management; uncovering new associations can help to improve
recognition and treatment in future cases.
