Aortic regurgitation and false aortic aneurysm formation in Behçet’s disease

Abstract

SummaryBehqet's disease is associated with vascular complications. Aortic regurgitation associated with this condition has never previously been described. A case is now reported in which this occurred and which was followed postoperatively by a false aneurysm of the ascending aorta. Case reportA 26-year-old man was referred to out-patients with severe leg ulceration in December 1974. He had had mouth and scrotal ulceration since the neonatal period and had observed that minor skin trauma was invariably followed by sepsis. He had no symptoms referable to the cardiovascular system and there was no past history of rheumatic fever.There was a positive family history in that his mother, brother and nephew all had episodes of mucocutaneous ulceration.He was thin and pale. There were no skeletal features of Marfan's syndrome. He had a single aphthous ulcer on his tongue and a symmetrical eruption over the extensor aspect of his lower limbs which comprised bluish papules and nodules; some hyperkeratotic and some pustular. Annular lichen planus was present at the base of the glans penis. In addition he had aortic regurgitation. BP was 140/50 mmHg.Investigations were: Hb, 14-7 g/dl; WBC, 9.9 x 109/1; ESR, 10 mm/hr; urea and electrolytes, normal; serum proteins and electrophoresis, normal; serological tests for syphilis, negative; chest X-ray, normal; metacarpo-phalangeal index, normal; sacroiliac joints, normal. Blood group, AB Rh-negative; tissue type: HLA A2.XB15.27/CW1. Skin biopsy showed non-specific epidermal reaction, dermal downgrowth and some degree of scarring. Immunofluorescence was negative.

Diffuse Aortitis Complicating Behcet’s Disease Leading to Severe Aortic Regurgitation

“…Intrinsic valvular abnormalities have been documented in Behcet's disease, often leading to torrential mitral or aortic regurgi tation with hemodynamic decompensation [10][11][12][13], Chikamori et al [13] have recently reviewed the reports of aortic regurgitation complicating Behcet's disease in the English (6 cases) and Japanese (15 cases) literature. Intrinsic aortic valve disease was the com monest etiological factor.…”

Papilledema as an Initial Manifestation of Behcet's Syndrome: Report of Four Cases with Cerebral Venous Sinus Thrombosis

“…3 This propensity for aneurysm formation may be augmented by arterial punctures as shown in one of our patients and two previous reports. 23,24 The result of surgery in such cases is uncertain, and the course is usually complicated, as demonstrated in our patient and in the literature. [23][24][25] Hence, we suggest that arterial punctures are to be avoided, and for visualization of the venous sinuses of the brain, the transvenous route for DSA is recommended.…”

“…23,24 The result of surgery in such cases is uncertain, and the course is usually complicated, as demonstrated in our patient and in the literature. [23][24][25] Hence, we suggest that arterial punctures are to be avoided, and for visualization of the venous sinuses of the brain, the transvenous route for DSA is recommended. Recently, MRI has been proposed as a procedure of choice for the initial evaluation of dural sinus thrombosis, 26 which should be ideal in Behcet's syndrome where thrombophlebitis may develop after venous punctures.…”

Valved conduit operation for aortic regurgitation associated with Behçet’s disease

“…This lesion is rare in the ascending aorta. 2 Behget's disease causes lesions not only in the aorta and arteries branching from the aorta, but also in the aortic valve. 3-2 AR is primarily due to valuvulitis.…”