A Uncommon Case: Kasabach-Merritt syndrome with VACTERL Association

We present a case of a mature male neonate with Kasabach-Merritt- Syndrome as well as VACTERL association. The VACTERL association describes a group of malformations. Our patient presented with anal atresia combined with tethered cord, and left renal agenesis. The VACTERL association as well as Kasabach-Merritt syndrome were found to be independent pathologies within this patient. A common occurrence or an association with each other has not been described in the literature so far. The challenging coagulation setting due to severe thrombocytopenia complicated the surgical management so far. Finally, mTOR-inhibitor sirolimus was successful in terms of tumor reduction and especially reduction of platelet consumption.PMID:38428835 | DOI:10.1055/a-2262-8607
Source: Zeitschrift fur Geburtshilfe und Neonatologie - Category: Perinatology & Neonatology Authors: Source Type: research