A male newborn, with a vascular mass on the right side of the neck and face, rapidly developed severe desaturation at birth. The lesion was already visible in utero and was compatible with a hemangioma.
Moreover, laryngoscopy demonstrated the invasion of the laryngeal structures by this hemorrhagic vascular tumor. The biological assessment also revealed coagulation disorders, anemia and severe thrombocytopenia.
The diagnosis of kaposiform hemangioendothelioma complicated by a Kasabach-Merritt phenomenon (KMP) was made based on this typical association.
First, the treatment consisted of the administration of propanolol, vincristine and systemic corticosteroids. Despite daily platelet transfusion, the platelet count was still very low. On day 8, the addition of sirolimus was followed by the platelet normalization and the rapid vascular mass regression, in one week.
The KMP is a rare complication of Kaposiform hemangioendothelioma. Its early diagnosis is very important given its high mortality.
The diagnosis is based on the presence of an indurated vascular tumor associated with anemia, thrombocytopenia and coagulation disorders.
One of the first therapeutic guidelines is the combination of vincristine and systemic corticosteroids. Moreover, sirolimus is increasingly used, with a rapid efficiency and a certain safety profile.
G. Giet1, MD; S. Cao1, MD; V. Willemaers1, MD; B. Dezfoulian2 MD, PhD; S. Tribolet3, MD; C. Hoyoux3, MD.
Dermatology Departments, Citadelle1 and Liège University2 Hospitals, Department of Pediatrics, Liège University Hospital3, Liège, BELGIUM