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Kaposiform Hemangioendothelioma Complicated by a Kasabach-Merritt Syndrome: Report of a Case

Journal: Pediatrics & Neonatal Biology Open Access (PNBOA) (Vol.3, No. 1)

Publication Date:

Authors : ; ;

Page : 1-6

Keywords : Infantile hemangiomas; Kaposiform Hemangioendothelioma; Kasabach Merrit; New born; Vascular tumor;

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Abstract

Kaposiform hemangioendothelioma (KHE) is a very rare form of vascular tumor, which is complicated by intra-tumor coagulopathy and requiring urgent management. Objective: we report a case of a KHE, complicated by a kasabach merrit syndrom compiled in the neonatology department of Mohamed VI university hospital, Marrakech and we discuss the different therapeutic modalities. Observation: a newborn was admitted, on the third day of life, for jaundice associated with a purplish red swelling of the left hemi face. He had anemia and thrombocytopenia requiring repeated transfusions. In view of the instrumental delivery, a hematoma was initially evoked. But in the absence of improvement, the eventuality of a hemangioma or complicated tumor became more likely. The patient was treated by propranolol in addition to high dose of oral corticosteroids and a skin biopsy was realized. Despite treatment, the patient died 10 days later due to a disseminated intravascular coagulation. A posteriori the histological study confirmed the KHE. Conclusion: a codified and early therapeutic protocol is necessary to improve the prognosis of patients with KEH, especially since other therapeutics has proved their effectiveness in the literature.

Last modified: 2018-11-17 15:35:28