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Il Giornale Italiano di Radiologia Medica 2018 Novembre-Dicembre;5(6):789-92

DOI: 10.23736/S2283-8376.18.00128-6

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: Italian

A rare case of neonatal Kasabach-Merritt Syndrome

Anna FAVIA , Mauro D’ADDATO, Angelica DRAGO, Laura MAPPA, Amato A. STABILE IANORA, Veronica FAVIA

Sezione di Diagnostica per Immagini, Dipartimento Interdisciplinare di Medicina, Policlinico di Bari, Università degli Studi di Bari, Bari, Italia


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The Kasabach-Merritt Syndrome (KMS) is a rare clinical entity characterized by severe consumption coagulopathy in association with highly aggressive neonatal vascular lesions such as kaposiform hemangioendothelioma or tufted angioma; this clinical condition is burdened by a mortality rate between 10-30%. We describe a case of vascular neoformation of the neck, found in prenatal age, clinically confirmed at birth and complicated by the onset of KMS. The instrumental diagnosis of vascular injury was initially performed with ultrasound of the neck. The newborn has been treated with propanolol and prednisone, without any benefit. Although the evidence of clinical features suggestive for the KMS, the severe hematological condition did not allow to proceed with the biopsy examination. We then proceeded to MR of the neck which confirmed the presence of a voluminous vascular lesion starting from the subcutaneous plan, in correspondence of the right laterocervical region; new therapy was then set with sirolimus (mTOR protein kinase inhibitor, involved in lymphocyte proliferation) and subsequent 2-month MR re-evaluation. After about 60 days, the dimensional stability of the neoformation and the remission of the serious hematological picture were demonstrated. The objective of our work is to underline the decisive role of imaging in KMS associated with vascular lesion of the subcutaneous tissue, responsive only to therapy with inhibitors of lymphangiogenesis.


KEY WORDS: Kasabach-Merritt Syndrome - Sirolimus - Vascular neoplasms

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