Syphilitic aortitis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Lakshmi Gopalakrishnan, M.B.B.S. [2]

Synonyms and keywords: Heller-Döhle syndrome

Overview

A pathological state of the aorta associated with the tertiary stage of syphilis infection. Syphilitic aortitis begins as inflammation of the adventia, including the vessels that supply the aorta itself with blood, the vasa vasorum. As the disease progression worsens, the vasa vasorum demonstrate hyperplastic thickening of their walls that restricts blood flow and causes ischemia of the outer 2/3's of the aortic wall. Starved for oxygen and nutrients, elastic fibers become patchy and smooth muscle cells necrose. The wall weakens and scars. If the disease progresses, syphilitic aoritis may lead to an aortic aneurysm.

Pathophysiology

Inflammatory involvement of tertiary syphilis begins at the adventitia of the aortic arch which progressively causes obliterative endarteritis of the vaso vasorum. This then causes narrowing of the lumen of the vaso vasorum, causing ischemic injury of the medial aortic arch and then finally loss of elastic support and dilation of the vessel. Dissection of the aortic arch is rare due to medial scarring.

Diagnosis

Physical Examination

• Syphilitic aortitis can cause de Musset's sign, a bobbing of the head that de Musset first noted in Parisian prostitutes.

Imaging

Chest X Ray

• Aortic dilatation
• Linear calcification of the ascending aorta

Angiography

• Helps to distinguish syphilitic aortitis from abdominal aortic aneurysm

Treatment

Surgery

• Aortic valve replacement