Key result
Novel syndrome features generalized arterial tortuosity and elongation linked to congenital elastic tissue defects.
Why the study?
A new clinical entity characterized by generalized tortuosity and lengthening of all major arteries was identified and described.
Case Report (n=1)
Describes a novel congenital defect of the elastic tissues of the arterial system characterized by diffuse tortuosity and lengthening of major arteries.
Alerts clinicians to consider rare diffuse arteriopathy in children with pulsatile vessels; leaves open its etiology, prevalence, and management.
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.
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Ali Ertuğrul (1967) conducted a case report in Generalized tortuosity and lengthening of all major arteries (n=1). A 10-year-old girl presented with a novel condition characterized by generalized tortuosity and lengthening of all major arteries, likely caused by a congenital defect of the elastic tissues.
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