Hypoplastic Aortoiliac Syndrome in the Arseniasis Area (Southwestern Coast) of Taiwan

Abstract

Objective: The hypoplastic aortoiliac syndrome (HAIS) has been well documented in the literature and it is unusually found in the population of atheroslerosis with aortoiliac occlusive disease. In the arseniasis area, so-called Blackfoot Disease (BFD) endemic area in the southwestern coast of Taiwan, we encountered HAIS patients in whom the patho-etiology was not been clear, but the surgical revascularization for limb salvage was feasible. Methods: From Jan. 2001 through Jan. 2007, 8 cases of HAIS were recognized among 288 patients in the BFD endemic area. The image study showed hypoplasia of the infrarenal aorta and iliofemoral system with mild to moderate degree of atherosclerotic change in the distal runoff. Clinically, all patients presented with severe intermittent hip or thigh claudication associated with ischemic changes of the foot or toes. The area ratio of the sum of cross-sectional areas of common iliac arteries/cross-sectional area of aorta above the bifurcation ranged between 0.39 and 0.7 (normal range 0.78 to 1.2). Results: Of the 8 HAIS patients, 1 patient underwent an aorto-bifemoral bypass with lumbar sympathectomy (LS), 2 received an aorto-unifemoral bypasses with LS, 1 had an iliofemoral bypass with LS, 3 had profundaplasty with LS, and the remaining one had a unilateral thoracic sympathectomy for finger ischemia and constant poikilothermia of both hands, but declined vascular reconstruction. There was no operative or perioperative mortality. The operative complications included minor wound infection, wound hematoma, paralytic ileus, and atelectasis. The preexistent pregangrenous toes needed amputation in the post-operative days when demarcation became clear. Conclusions: The hypoplastic aortoiliac syndrome (HAIS) associated with ischemia of toes should be treated as aortoiliac occlusive disease without delay. We believe the bypass grafting through a retroperitoneal approach with adjunct lumbar sympathectomy is the treatment of choice if patients' general condition permits.