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Arteriomegaly and Aneurysmosis
Arteriomegaly is defined as a diffuse ectasia involving multiple arterial segments including the aorta, iliofemoral, and femoropopliteal vessels. It is characterized by significant tortuosity, elongation, and luminal irregularities often resulting in diminished blood flow. It was originally described in 1971 by Thomas, with reference given to two cases described by Leriche in 1942 and 1943. These patients were noted to have “extraordinary elongation and dilatation of the pelvic and common femoral arteries.” Leriche termed this condition “arteria magna et dolicho.”
In itself alone, arteriomegaly is considered to be a benign condition; however, aneurysmal involvement can and commonly does affect these arteries. It can be quite extensive. This process is distinct from general atherosclerotic aneurysms and is termed aneurysmosis or arteriomegaly with aneurysms. An increased morbidity and mortality is seen with aneurysmosis, and given the extent of disease, it can necessitate extensive and atypical surgical treatment.
Etiology
The cause of arteriomegaly and aneurysmosis is not well understood. Histological studies of arteries of patients with this disease have shown changes consistent with a connective tissue disease, specifically degradation of elastin in the medial layer, as well as arteriosclerosis. Although no distinct genetic component has been identified to date, reports have suggested a strong familial link with regard to aneurysm development and arteriomegaly.
Risk factors in patients with this disease are similar to those commonly seen in most atherosclerotic vascular patients. In a review by Hollier and colleagues, of 91 patients having diffuse aneurysmal disease or aneurysmosis, all were male with a mean age of 67.5 years (range, 36.5 to 87 years). Sixty-nine patients were current or former smokers (76%), 54 patients were hypertensive (59%), and 16 patients had diabetes mellitus (18%). Significant cardiovascular involvement was noted as well, with 56 patients (61%) having coronary artery disease or congestive heart failure and 30 patients having had a prior myocardial infarction. Cerebrovascular disease affected 13 patients who had a previous transient ischemic attack or stroke.
Classification
Arteriomegaly with aneurysms or aneurysmosis can be classified into three types based on the location and degree of arterial involvement. This classification, based on a review of 5771 aortofemoral angiograms performed at the Mayo Clinic from 1986 to 1982, revealed 300 patients with radiographic findings of arteriomegaly, including 91 (30.3%) with diffuse aneurysmal involvement or aneurysmosis ( Figure 1 ).
Type I includes aneurysms of the aorta, iliac, and common femoral arteries with arteriomegaly of the superficial femoral and popliteal arteries. Type II includes aneurysms of the common femoral, superficial femoral, and popliteal arteries, with arteriomegaly of the aorta and iliac arteries. Type III includes aneurysms of the aorta, iliac, femoral, and popliteal arteries, with arteriomegaly of the intervening arteries that are not specifically aneurysmal.
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