Angiomegaly

Abstract

The authors report a clinical and ultrastructural study on a group of patients with angiomegaly, a vascular disorder characterized by elongated and distended blood vessels affecting the arterial (arteriomegaly) and/or venous system (venomegaly). The arterial group, drawn from a large arteriographic series, focuses on a comparison between atherosclerotic arteriopathy and arteriomegaly. The venous group, drawn from a large ultrasound series of vein disorders, is made up of patients with venomegaly. Venomegaly gives rise to few or no symptoms and it appears to be less frequent than arteriomegaly but as the latter proved to be associated in the majority of cases studied. Based on ultrastructural findings, the chief abnormality of angiomegaly seems to lie in a specific alteration of the elastic component of the vessel wall. We found slightly osmiophil amorphous elastic material neighbouring the basement membrane of the myocytes of the vessel walls. In the superficial parts of these myocytes occurred a great number of pinocytotic vesicles indicating for a rich creation of the new elastic material. Middle or highly osmiophil thick elastic fibers with irregular side protrusions were also found among myocytes remembering the moth-eaten picture. Results from a large ultrasonographic study on patients' relatives suggest an inheritability of this vascular disorder.