Coexistence of Kasabach–Merritt Syndrome and placental chorioangioma in a premature infant

Gültekin, N. Dilay*, Yilmaz, F. Hilal, Altunhan, H., Findik, S., Tokgöz, H., Çalişkan, Ü. | JNPM 2018;

Kasabach-Merritt syndrome is a rare life-threatening clinical presentation in neonatal period. it is characterized by giant hemangioma and serious thrombocytopenia. The diagnostic criteria include: 1) hemangiomas on skin, 2) thrombocytopenia or coagulopathy, 3) hemangioma on internal organs diagnosed by ultrasonography, computed tomography or magnetic resonance imaging, and 4) excluding reasons, such as idiopathic thrombocytopenic purpura or hypersplenism. Placental chorioangiomas are the most widespread non-trophoblastic benign tumor-like lesions of placenta. The clinical signs are associated with tumor size. Chorioangiomas larger than 4-5 cm may lead to various maternal and fetal complications. Here, a female premature infant was diagnosed with placental chorioangioma and liver hemangioma during antenatal period. She developed heart failure secondary to non-immune hydrops fetalis in the neonatal period. The atypical giant hemangioma and coagulopathy suggested the diagnosis of Kasabach-Merritt syndrome. The macroscopic and histopathological examination of the placenta confirmed the diagnosis of chorioangioma. The patient died due to purpura fulminans despite the treatment with prednisolone and propranolol that was started on the second day of life. We are presenting this rare case where placental chorioangioma leading to non-immune hydrops fetalis co-existed with Kasabach-Merritt syndrome

*Corresponding Author: 

Nazli Dilay Gültekin, Yunus Emre Mahallesi, Beyşehir Caddesi, Necmettin Erbakan University, Meram Medical Faculty, Department of Neonatology, 6. Kat, S blok Konya, Turkey. Tel.: +90 0332 223 66 97; Fax: +90 0332 223 61 82; E-mail: