For which syndrome is giant liver hemangioma most notably associated in the pediatric population?

Giant liver hemangiomas are most notably associated with Kasabach-Merritt syndrome in the pediatric population. This syndrome is characterized by the presence of a hemangioma that can lead to significant thrombocytopenia and coagulopathy. In Kasabach-Merritt syndrome, the hemangioma consumes platelets and coagulation factors, causing a significant bleeding risk and requiring close medical management.

Understanding the association of giant liver hemangiomas with Kasabach-Merritt syndrome is crucial for pediatricians and surgeons, as it highlights the importance of monitoring for complications related to coagulation abnormalities that can arise from large vascular malformations. In contrast, while Beckwith-Wiedemann syndrome, Wiskott-Aldrich syndrome, and Alagille syndrome have their own distinct clinical features and complications, they do not have the same strong association with giant liver hemangiomas as seen in Kasabach-Merritt syndrome.