Hepatic vascular malformation with secondary heart failure in a two months old infant, endovascularly managed by embolisation using onyx™: presentation of a case and review of the literature

Abstract

DOI: 10.1016/j.rcpe.2015.12.002

Vascular malformations have always been a difficult disease to treat because of its complexity, difficult diagnosis, and poor results with conventional surgery. A multidisciplinary team approach is required to manage these malformations, including a paediatrician, paediatric surgeon, vascular surgeon, plastic surgeon, etc., in order to properly classify them, and use endovascular methods as the first therapeutic option.

Using the current classification by the International Society for the Study of Vascular Anomalies (ISSVA) and diagnostic and therapeutic endovascular techniques, a clearer picture of these disorders is emerging, leading to more opportunities for the patient.

The ISSVA has promulgated several consensus to sort and classify various tumours and vascular malformations. Using the old nomenclature causes confusion, inaccuracy in the diagnosis, and potentially incorrect management. The main objective of this classification is to divide the vascular abnormalities into vascular malformations and vascular tumours.

Hepatic vascular malformations, particularly in very young children are a cause of mortality and significant long-term morbidity, given the size and the amount of vascular flow steal phenomenon generating high output or intravascular coagulation syndrome (Kassabach- Merrit syndrome). The case is presented of a male infant admitted with decompensated heart failure secondary to a liver mass diagnosed using Computed Tomography and treated with endovascular embolisation.