Original Articles
Vol. 14 (2019)
https://doi.org/10.3269/1970-5492.2019.14.28
KASABACH-MERRITT SYNDROME IN A NEWBORN: FROM PRENATAL DIAGNOSIS WITH MRI TO TREATMENT WITH SIROLIMUS
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
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Kasabach-Merritt Syndrome (KMS) is a rare clinical entity, found in 1% of angiomatous lesions,
characterized by severe consumption coagulopathy/thrombocytopenia in association with highly
aggressive neonatal vascular lesions. We describe a case of prenatally diagnosed vascular
neoformation of the neck, revealed by obstetric ultrasound and then confirmed by fetal Magnetic
Resonance, with subsequent onset of KMS at birth. The newborn was treated with Propanolol and
Prednisone, without any benefit; Magnetic Resonance (MRI) at day 14 of life confirmed the
hypervascularized lesion of about 6x7x6 cm on the right laterocervical site. Sirolimus therapy was
then initiated leading to rapid improvement in thrombocytopenia and a volumetric reduction of the
vascular lesion on MRI. After 12 months of treatment, MRI scan showed a further reduction in
volume of the vascular lesion; also, the KMS-related thrombocytopenia was completely resolved:
Sirolimus could represent the first line of treatment of vascular lesions in the neonatal age.
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KASABACH-MERRITT SYNDROME IN A NEWBORN: FROM PRENATAL DIAGNOSIS WITH MRI TO TREATMENT WITH SIROLIMUS. (2026). EuroMediterranean Biomedical Journal, 14. https://doi.org/10.3269/1970-5492.2019.14.28
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