Medical Management of Vascular Anomalies

Stefanos Intez, MD
Pediatric Oncologist
Sacred Heart Children’s Hospital

The field of vascular anomalies has greatly expanded in the past few years, and management of such complex patients relies on the implementation of a multi-disciplinary approach.  Vascular anomalies may be broadly classified as tumors or malformations.  Malformations may then be divided further in low or high flow lesions to encompass arterial, capillary, venous, lymphatic or combined.  Tumors include hemangiomas (infantile, congenital), which may be simple or complex based on their location, interference with vital structures, or links to syndromes.  A type of tumor of particular importance includes Kaposiform hemangioendothelioma, along with a fairly broad differential diagnosis.

It has become evident that many lesions behave aggressively and may lead to a number of complications, including death, chronic pain, disfigurement, coagulopathy and infectious risks.  The use of medical therapies, such as propranolol (2- 3 mg/kg/day) in complex hemangiomas and sirolimus (0.8 mg/m^2/dose twice a day) in venous and lymphatic malformations,  supportive care that includes anti-coagulation and antibiotics, and traditional surgical and interventional radiology approaches has led to an improved functional outcome for patients.

New research insights in pathogenesis have allowed us to better refine the classification of these lesions, which in turn is leading to additional targeted therapies.  The utilization of multiple fields of expertise within the Sacred Heart Children’s Hospital community allows for improved characterization and management of such patients.