Consumptive coagulopathy in utero associated with multiple vascular malformations

We report a postmature male infant who died at age 2 h of asphyxiating pulmonary hemorrhage. Autopsy found edema, serous ascites, and vascular malformations within myocardium, lung, mediastinal soft tissue, thoracic-wall skeletal muscle, spleen, thyroid and adrenal glands, and pancreas. The pulmonary malformations had arteriovenous features, while those elsewhere were predominantly capillary; many of the latter contained fibrinous thrombi. Circulating nucleated erythrocytes, siderosis of proximal renal tubular epithelium, and intrahepatocytic and intracanalicular cholestasis were present. We believe that these findings represent an unusual instance of hemangioma-associated consumptive coagulopathy and intravascular hemolysis (Kasabach-Merritt syndrome) of antenatal onset, associated with features suggestive of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).