Diffuse Tortuosity and Lengthening of the Arteries

Abstract

This article describes the case of a 10-year-old girl with generalized tortuosity and lengthening of all her major arteries-the carotid arteries, the aorta from the arcus to the bifurcation, and the iliac, femoral, splenic, hepatic, renal, and intercostal arteries. The condition appears to be a new entity. Pertinent abnormalities were as follows: gross pulsation of the arteries in the neck, telangiectasis of both cheeks, high palate, tortuosity of all the arteries, aortic valvular insufficiency, 1+ to 2+ capillary fragility on tourniquet test (lacet), 1+ Wassermann reaction, 4+ cephalin flocculation, and histological fragmentation of the internal elastic membrane of the arteries. Syphilis, arteriosclerosis, hypertension, mucopolysaccharidosis, and defects of amino acid metabolism were ruled out and the author concludes that the condition was most probably caused by a congenital defect of the elastic tissues of the arterial system.