June 6th, 2020

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Elevated Maternal Shock Index Associated with Nonimmune Hydrops Fetalis, Fetal Hepatic Hemangioendothelioma, and Kasabach-Merritt Syndrome: A Case Report
Authors:  Matthew J. Blitz, M.D., M.B.A., and Burton Rochelson, M.D.
BACKGROUND: Fetal hydrops may be associated with adverse effects on maternal physiology, as in mirror syndrome, in which maternal edema and anemia are observed. In the absence of clinically recognized maternal edema, a significant maternal response to fetal pathology is rarely reported.

CASE: A 25-year-old multiparous woman at 31 weeks of gestational age was found to have a hydropic fetus with a hepatic hemangioendothelioma. Both the mother and fetus developed severe anemia. The newborn was postnatally diagnosed with Kasabach-Merritt syndrome, a rare disease in which a vascular tumor is associated with severe thrombocytopenia and consumptive coagulopathy. The mother had no appreciable edema. However, she had an elevated shock index of 1.3, suggesting hemodynamic instability.

CONCLUSION: Significant aberrations in maternal physiology may occur in association with fetal hydrops even in the absence of clinically recognized maternal edema. Therefore, narrowly defined diagnostic criteria for mirror syndrome may fail to identify all cases in which a hydropic fetus exerts a deleterious effect on the mother.
Keywords:  anemia, fetal hepatic tumor, fetal hydrops, Kasabach-Merritt syndrome, maternal anemia, mirror syndrome, placental edema, shock index
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