Definition
Definition
THEChervinsky congenital hepatic hemangiomatosis is a rare pathology corresponding to the presence of liver malformations and described in newborns or male infants. This condition appears very early and is characterized by the presence of a hepatomegaly and insufficient functioning of the heart pump causing a poor outcome before the age of 6 months. It is sometimes possible to make the diagnosis thanks to the presence of cutaneous angiomas. These are dilation permanent and visible, star-shaped, small vessels of the skin of the face, generally appearing between the ages of 30 and 50. This term was first used by Besnier and Doyon.
The liver contains numerous nodules whose perimeter is made up of small capillaries joined together and whose caliber has increased (tiny vessels) organized in the form of networks.
Another characteristic of this reconstituted tissue is the presence of anastomosis (abnormal communication) between the venous and arterial networks; we speak of arteriovenous fistulas, which cause heart failure. This is a type of angiohamartoma of the liver (see hamartoma).