General Surgery Resident Physician, PGY-2 DMC Sinai Grace Hospital
Introduction/Purpose: Hypoplastic aortoiliac syndrome (HAS) is a rare condition largely described in young women of small stature with a significant smoking history. Angiography is the gold standard of diagnosis. The treatment of choice remains surgical revascularization.
Methods or Case Description: A 48-year-old woman presented with intermittent claudication in her bilateral lower extremities since 1 year previously with worsening symptomology with extended periods of walking. CT angiography revealed moderate and severe segments of stenosis at the infrarenal abdominal aorta with the worst area of involvement at the aortic bifurcation. Based on the patient’s presentation and radiographic findings, she was diagnosed with HAS. We performed an aortoiliac bypass grafting with a bifurcating graft graft via an open transabdominal approach. After surgery, the patient was administered clopidogrel and aspirin on post operative day 1. She has remained free of claudication since her operation.
Outcomes: In contrast to arteries of aortoiliac occlusive disease, the arteries in HAS may have little if any atherosclerotic changes such as stenosis, kinking or calcification. These arteries, however, have distinct anatomic findings that define the syndrome, including high bifurcation of the abdominal aorta, straight course of the iliac arteries without the normal characteristic bowing, acute angle of the aortic bifurcation (20°–30°), aortic diameter of 14 mm or less, and iliac artery diameter of 7 mm or less. As a result of these stenotic features, patients with HAS present most commonly with intermittent claudication and hypertension. Most patients need to be surgically managed, with aortofemoral bypass being the most commonly used grafting technique.
Conclusion: As HAS increases the risk for aortoiliac occlusive disease and severe complications of hypertension, it is imperative to identify these patients early and treat them accordingly.
Upon completion, participants will be able to clearly define hypoplastic aortoiliac syndrome or small aorta syndrome, identify patients most at risk for this syndrome, and the symptomology associated with this disease process.
Upon completion, participants will be able to effectively work-up a patient suspected of having hypoplastic aortoiliac syndrome, with respect to ordering the appropriate imaging modality.
Upon completion, participants will be able to discuss different treatment approaches to hypoplastic aortoiliac syndrome.
Upon completion, participants will be able to identify key surveillance measures and follow-up requirements in patients with hypoplastic aortoiliac syndrome.