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  1. M. J. Lyons,
  2. L. Hudgins
  1. Division of Medical Genetics, Stanford University


Hydrops fetalis is defined as gross edema of the entire body of a fetus or newborn infant The majority of cases are nonimmune with a myriad of causes. Machin (1989) estimated that an etiology for hydrops fetalis can be found in up to 80-85% of cases. However, diagnosis can be difficult if initial testing does not lead to an underlying cause The purpose of this report is to present hepatic hemangioendothelioma as a rare cause of hydrops fetalis and indicate skin findings which may aide in diagnosis. We describe a 5 month old female with nonimmune hydrops fetalis detected at 31 weeks gestational age by prenatal ultrasound. An amniocentesis revealed a normal 46,XX karyotype. A C-section was performed due to worsening hydrops. She had a normal echocardiogram and abdominal ultrasound during a prolonged NICU stay. At three weeks of age, a hemangioma was noted over her right eyelid. Her hydrops gradually resolved and she was discharged to home at six weeks of age. At two months of age, she was noted to have three violaceous skin lesions over her back. At five months of age, a renal ultrasound was done due to a family history of vesicoureteral reflux. The ultrasound revealed multiple enhancing lesions in both hepatic lobes. An abdominal MRI confirmed the presence of the hepatic lesions which were diagnosed as hemangioendotheliomas. Infantile hemangioendothelioma is the most common vascular hepatic tumor in children. The tumor is seen almost exclusively in the first six months of life with girls more frequently affected (1.7:1). Only six cases of hepatic hemangioendothelioma associated with hydrops fetalis have been reported in the literature. We report an additional example of hydrops fetalis due to hepatic hemangioendothelioma. Vascular tumors, such as hemangioendotheliomas, may cause hydrops fetalis by functioning as shunts leading to high output cardiac failure. Our case demonstrates that hepatic hemangioendothelioma should be a diagnostic consideration in idiopathic hydrops fetalis. Skin hemangiomas can be found in 11-40% of hepatic hemangioendothelioma cases. Our patient developed unusual skin lesions over her back and a hemangioma over her right eyelid prior to the diagnosis of hepatic hemangioendothelioma. These skin lesions may serve as a diagnostic clue to the presence of hepatic hemangioendothelioma, especially in the setting of hydrops fetalis. By recognizing this association, an earlier diagnosis and more efficacious treatment may be possible.

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