Hypoplastic aortoiliac syndrome: An entity peculiar to women

Hypoplastic aortoiliac syndrome (HAIS) is a unique disorder that occurs almost exclusively in women. Because reports in the literature are scant, we reviewed our own experience. The criteria for definition were high bifurcation of the abdominal aorta, straight course of the iliac arteries without the normal characteristic bowing, acute angle of the aortic bifurcation, aortic diameter of 14 mm or less, and iliac artery diameter of 7 mm or less. Nineteen patients with HAIS were identified in a 14-year period. All 19 patients were heavy smokers. Most had significant associated arterial disease, and all were women. Fifteen patients were selected as candidates for operation. Two were treated conservatively, one refused surgery, and one died of a myocardial infarction before operation. Reconstruction was performed by aortoiliac thromboendarterectomy with vein patch for segmental disease (four patients) and Dacron bifurcating graft for diffuse disease (11 patients). There were no deaths during operation. Four patients required reoperations for graft failure and three patients eventually required amputations. The four patients who underwent reconstructions by thromboendarterectomy with vein patch had uniformly good results. From this experience, the following approach and management are recommended: conservative nonoperative exercise program with close follow-up if symptoms are tolerable, aortoiliac thromboendartectomy and vein patch if disease is segmental, aortofemoral bifurcating graft for patients with diffuse disease with graft size closely approximating vessel size, and concomitant femoral-to-popliteal graft to ensure adequate runoff and graft patency if there is significant femoral disease. The etiology of HAIS remains obscure. Early onset of symptoms seems to be due to small vessels with superimposed atherosclerotic plaques. Addiction to smoking in these women is extreme (paralleling that seen in Buerger's disease) and may be of etiologic significance.