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Diffuse neonatal haemangiomatosis with a placental chorangioma
  1. A Lording,
  2. M Saito-Benz
  1. Neonatal Intensive Care Unit, Portsmouth Hospitals Trust, Portsmouth, UK

Abstract

Introduction Single haemangiomas are common in neonates. Rarely they present as multiple, widely spread lesions also known as haemangiomatosis. In 50% of these there is visceral involvement, which can affect the liver, GI system and CNS. There are reports of haemangiomatosis being associated placental chorangioma.

Case report A term male infant, BW 2280 g, was admitted to our neonatal unit with hypoglycaemia and a non-blanching rash. At delivery, a mass was found in the placenta, which was found to be a chorangioma.

On admission, he was stable but had polycythaemia, thrombocytopenia and coagulopathy with a prolonged INR, APTR and decreased fibrinogen. He was managed with intravenous Vitamin K, a platelet transfusion and fresh frozen plasma. He remained clinically stable and he received one further dose of IV vitamin K before his thrombocytopenia and coagulopathy completely resolved. An abdominal ultrasound scan was performed to look for visceral involvement. This showed a hepatic haemangioma.

Discussion Two types of neonatal haemangiomatosis are described. Benign neonatal haemangiomatosis affects the skin only and diffuse neonatal haemangiomatosis affects both the skin and viscera. Our case falls into the second group with both skin and liver involved. The most common sites affected by diffuse haemangiomatosis are the liver, GI system, CNS and lungs. Infants can be ill at birth or present later with high output cardiac failure, GI haemorrhage or CNS compression causing hydrocephalus. Mortality rates of up to 50% is reported.2. Large chorangiomas have an incidence of 1:9000 births and are often associated with antenatal complications and 30% fetal mortality. There is a known link between large chorangiomas and neonatal haemangiomatosis. The mechanisms of this link are still being established.

In our case the neonate presented with thrombocytopenia and coagulopathy, which quickly resolved with treatment. This suggests that this was secondary to the placental chorangioma rather than the hepatic haemangioma, which would have caused a more prolonged consumptive coagulopathy.

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