Kasabach-Merritt syndrome arising from an Enteroatmospheric Fistula

TL;DR

This paper reports a novel case of Kasabach-Merritt syndrome associated with a vascular malformation in an infant with an enteroatmospheric fistula, expanding understanding of the syndrome's possible causes.

Contribution

It introduces a new association between Kasabach-Merritt syndrome and a vascular malformation in the context of an enteroatmospheric fistula, which has not been previously documented.

Findings

Kasabach-Merritt syndrome can occur with vascular malformations, not just tumors.

The syndrome was observed in an infant with a vascular enteroatmospheric fistula.

Proper diagnosis and management can resolve the syndrome.

Abstract

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.