Surgical Management of Takayasu's Arteritis

Takayasu's arteritis (TA) is a nonspecific granulomatous inflammatory arteriopathy of unknown cause that results in occlusion or less common aneurysmal degeneration of large and medium-sized elastic arteries. The disease was first described in 1908 by Takayasu (1), a Japanese ophthalmologist, in a young female patient with retinal neovascularization and absent radial pulse. Subsequent descriptions of the disease have emphasized the 'pulseless' syndrome, with involvement of the brachiocephalic arteries. Its incidence is low, being reported in 1/3,000 autopsies in Japan and as low as 2.6 cases per one million in the United States (2, 3). The female-to-male ratio was reported as 8-10:1 (4).

The clinical feature was dependent on the organ involved. The patients presented with claudication if the lower abdominal aorta was involved, hypertension if the renal arteries were involved, stroke if the cerebral vascular vessels were involved or angina and myocardial infarction symptoms if the coronary arteries were involved.

Arteriography was the best modality for the diagnosis of TA with magnetic resonance angiography and CT scans (5-7). The disease did not just affect the arterial bifurcations but usually involved the entire length of arteries. The disease had a very peculiar distribution with the subclavian, axillary, carotids, renals and infra-abdominal aortas.

The clinical presentations of TA was not the same as atherosclerosis. The patients with atherosclerosis were usually elderly while patients with TA were young, but despite their youth, they still had severe cardiac, renal and pulmonary problems. Therefore these patients should undergo extensive medical evaluation prior to surgery. It was preferable to avoid surgery during the acute phase of the illness. The surgical procedure always consisted of a bypass operation to vessels normal on angiography proximal and distal to the occlusive or stenotic lesion. The aim of this report was to evaluate the effectiveness and safety of the operation performed for symptomatic TA.

Cerebral ischemic symptoms and visual disturbance disappeared in all 3 patients after the ascendo-carotid bypass. One out of these 3 patients complained of migraine-like headache for 3 days after the operation. This symptom was relieved with no specific medication. There was no other postoperative complication. The symptoms of cardiac failure disappeared after thoraco-left iliac bypass. All patients were followed up at an interval of every 6 to 12 months with CT angiography. The patients of aortic endarterectomy showed no restenosis and all grafts were patent during the follow up periods (range; 4-75 months).

TA is an idiopathic, non-specific inflammatory disease involving the aorta and its main branches; histologically it is characterized by fibrosis in the intima and the adventitia, and degeneration and disintegration in the media.

Microscopic finding may reveal a heavy cellular infiltration of the media consisting mainly of lymphocytes and a variable mixture of histiocytes and plasma cells. Giant cells of western body type may be present and are occasionally numerous (8, 9). We could obtain the tissue only in patient who was performed aortoiliac endarterectomy. We could not obtain the tissue in other 4 patients because we performed bypass surgery. The pathologic finding of our patient showed that a heavy cellular infiltration of the media consisted mainly of lymphocyte and intimal thickening with myxoid degeneration. Three types of this entity were classified histologically: granulomatous inflammatory, diffuse productive inflammatory, and fibrosing (2).

The cause of TA remains obscure, but autoimmune and allergic responses to previous infections have been considered. Franklin and Morrison demonstrated a reaction between lymphocytes and bacterial protein antigen and considered that the inflammatory cells that were produced may destroy the surrounding fragile elastic fibers via their enzymes (11). Several studies demonstrated various human leucocyte antigen associations with TA, suggesting a genetic predisposition for the disease (11-14). The strong predilection for women, geographical incidence, and occasional familial occurrence may also indicate a role for genetic factors (15).

A large series of patients with this inflammatory arteriopathy have now been reported from Japan and Mexico, and the natural history, pathology, and arterial involvement have been better defined (16, 17). The clinical course of TA includes a prodromal "prepulseless phase," which is manifested by a variety of constitutional symptoms including pyrexia, weight loss, anorexia, night sweats, malaise, arthralgias, and myalgias. Since these symptoms are nonspecific and protean, they are rarely discernible as a discrete prodromal phase (16, 17). A "pulseless phase" follows months to years later and is attended by peripheral pulse deficits and symptomatic occlusive or aneurysmal arterial disease. The arterial involvement may be confined to the aortic arch as in Takayasu's original patient, or involve multiple aortic segments, or the entire aorta, its major branches, and the pulmonary artery as well (18). Arterial pathology varies from a fulminant acute inflammatory process to extensive post-inflammatory mural fibrosis (19).

Corticosteroids have been the primary therapy for TA, since dramatic symptomatic improvement and restoration of absent pulses were reported to follow its use (20). However, the efficacy of corticosteroids in TA has not been universally acknowledged, since most patients with symptomatic arterial insufficiency do not manifest dramatic clinical improvement. We used corticosteroid in all patients when they were in the acute phase of disease. Certainly, as in temporal arteritis, a reversible phase of this disease exquisitely sensitive to corticosteroid therapy may exist.

Vascular reconstruction in patient in patients with TA was approached with considerable trepidation in the past, particularly during the acute phase because of fear of occlusion of the involved vessel or graft, or anastomotic disruption (21). However, Ahn et al. reported the bypass surgery to the 18 patients with TA, and there was no motality (22). Mwipatayi et al. reported 115 surgical procedure with overall operative mortality in 4% (23). They confirmed that surgery was both safe and effective. There are distinct differences between the lesions of TA and those of atherosclerosis. Atherosclerotic type endarterectomy has not been feasible in this group of patients with TA because of involvement of the long segment of artery in the patients with TA. The choice of operative procedure depends on the number of arteries involved, the extent of arterial occlusive lesions and the clinical manifestations. But endarterectomy is usually indicated in patients with TA involving short segment, high flow artery (i.e., aortoiliac occlusive disease). We had reported 2 cases of abdominal aortic endarterectomy in TA and Leriche's syndrome (24).

In conclusion, surgical treatment of symptomatic TA was highly effective and safe. Symptomatic improvement and excellent long-term graft patency could be expected after arterial reconstruction.