Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion.
We describe an infant presenting with fetal pericardial effusion requiring in utero pericardiocentesis. Important postnatal clinical features included recurrent pericardial effusion, progressive stridor, thrombocytopenia, anemia, and a mediastinal mass surrounding the heart and coronary arteries. Investigations and management consisted of repeat pericardiocentesis, platelet and red blood cell transfusions, laryngoscopy, creation of a pericardial window, and biopsy of the mediastinal mass. Diagnosis was made of Kaposiform hemangioendothelioma surrounding the base of the heart, trachea, and esophagus as well as Kasabach-Merritt phenomenon. The infant responded well to treatment with vincristine and prednisone.
Walsh
Mark A
MA
Department of Pediatrics, Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Canada.
Carcao
Manuel
M
Pope
Elena
E
Lee
Kyong-Jin
KJ
D019468:Disease Management; D006390:Hemangioendothelioma / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007223:Infant; D010490:Pericardial Effusion / Q000209:etiology*; D011241:Prednisone / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
D014750:Vincristine; D011241:Prednisone