Amit J. Dwivedi, Fellow in Vascular Surgery Eric Nguyen, Fellow in Vascular Surgery C. Cherukupalli, Fellow in Vascular Surgery K.V. Krishnasastry, Chairman, Department of Vascular Surgery, North Shore University and Long Island Jewish Medical Centers, Manhasset, NY
Amit J. Dwivedi, MD Fellow in Vascular Surgery
Eric Nguyen, MD Fellow in Vascular Surgery
C. Cherukupalli, MD Fellow in Vascular Surgery
K.V. Krishnasastry, MD Chairman
Department of Vascular Surgery
North Shore University and Long Island Jewish Medical Centers
Cystic adventitial disease of the popliteal artery is an unusual condition of uncertain etiology and causes symptoms of intermittent claudication. The authors report the case of a patient who had intermittent claudication for 3 months before his presentation. Arteriography demonstrated a smooth-walled narrowing, and Doppler ultrasonography revealed severe narrowing of the popliteal artery in the proximal segment. These imaging studies proved helpful in establishing the diagnosis. The diseased arterial segment was successfully resected, and a vein bypass interposition was performed. The authors discuss the presentation, radiographic findings, diagnosis, and literature on cystic adventitial disease of the popliteal artery.
Cystic adventitial disease of the popliteal artery results in compression of the vessel lumen, causing localized stenosis or occlusion. The first reported case of adventitial cyst with compression of the popliteal artery was in 1954.1 In cystic adventitial disease, a mucin-containing cystic structure forms in the popliteal artery wall, causing claudication. Adventitial cystic disease remains a rare cause of claudication, probably accounting for 0.1% or fewer of all patients seeking treatment.2 Many cases of popliteal artery adventitial cyst remain undiagnosed. The condition is usually diagnosed using Doppler ultrasonography, contrast arteriography, computed tomography (CT) scanning, or magnetic resonance angiography (MRA). We report the case of a 33-year-old man with cystic adventitial disease of the popliteal artery and review the diagnostic and treatment options pertaining to this rare condition.
A 33-year-old man presented with a 3-month history of two-block claudication of the left lower extremity. He did maintenance work, and the pain in his leg was affecting his ability to do his job. The patient reported no history of diabetes mellitus, hypertension, hypercholesterolemia, or previous surgeries, and no significant family history. He acknowledged smoking a pack of cigarettes every 3 to 4 days. On physical examination, he had no bruits in the carotid artery or abdomen and the femoral pulses were equal bilaterally. The right popliteal, dorsalis pedis, and posterior tibial pulses were normal. On the left lower extremity, he had no popliteal pulse, and a dorsalis pedis and posterior tibial pulse were barely palpable. Both lower extremities were warm, and no abnormal changes in his hair growth, skin, or nails were observed. The laboratory studies were all within normal limits, and an electrocardiogram demonstrated a normal cardiac rhythm.
The patient underwent a left lower extremity angiogram using a right common femoral approach, which showed a 2- to 3-cm, smooth, focal, severe stricture within the proximal popliteal artery (Figure 1). The appearance of the stricture was characteristic of external compression. The remainder of the popliteal artery as well as the left lower leg trifurcation and proximal tibial vessels were normal. The distal calf vessels and the pedal vessels could not be visualized because of poor flow through the strictured segment.
The patient underwent Doppler ultrasonography of the left popliteal fossa, which revealed severe narrowing of the popliteal artery in the proximal segment (Figure 2). A 1-cm, hypoechoic, tubular structure was noted to be in intimate association with the popliteal artery at the level of the stenosis. The patient underwent resection of the cyst and repair of the popliteal artery with a saphenous vein patch. During the procedure, an incision was made in the wall of the cyst and the contents were noted to be clear, gelatinous, and loculated into three separate compartments (Figure 3).
Pathology demonstrated a cyst with fibrovascular tissue accompanied by scattered, mucin-rich, palisading granulomata suggestive of granuloma annulare. A completion angiogram revealed the popliteal artery to be widely patent with no defects and good runoff (Figure 4). The patient tolerated the procedure well, his diet was advanced, and he was discharged to home on postoperative day 1, requiring crutches and pain medication. His follow-up has been unremarkable, and he is ambulating normally with no reports of pain.
Cystic adventitial disease is an uncommon condition that was first observed in the external iliac artery.3-5 The disease may occasionally manifest as a mass lesion in other vascular sites, but the popliteal artery is the most frequently involved vessel and accounts for 85% of reported cases.4,6 Men are predominantly affected and usually manifest symptoms in their fourth and fifth decades of life, while women usually present in their fifth or sixth decades.6 Elderly men with risk factors for peripheral vascular disease have also been reported to have the condition.7-9
In the 1970s, Flanigan and colleagues and Schramek and Hashmonai reviewed the four theories regarding the possible etiology and pathogenesis of adventitial cystic disease.6,10 These are referred to as the systemic disorder, traumatic, ganglion, and developmental theories. Some forms of adventitial cystic disease may involve more than one of these theories of etiology. The theory of repeated trauma has had the greatest support.11 The proximity of the popliteal artery to the knee joint results in the artery being unduly prone to stretch and distortion. This repetitive trauma of the popliteal artery has been postulated to cause cystic degeneration and possible destruction of the adventitia of the adjacent vessel.12
Diagnosis starts with a thorough patient history and physical examination. Patients present with sudden onset of claudication, without any evidence of occlusive atherosclerotic disease or manifestations of embolism, as was the case with our patient. On physical examination, they may have normal or reduced popliteal and pedal pulses.13 Differential diagnosis may include atherosclerosis, popliteal artery aneurysm, arterial embolus, trauma, popliteal artery entrapment syndrome, or cystic adventitial disease. Traditionally, this condition is diagnosed based on contrast arteriography demonstrating an eccentric “sword like” stenosis described as a scimitar sign.14 Noninvasive diagnostic studies include color Doppler ultrasonography, CT scanning, and MRA, which can aid in correctly recognizing the disease in most cases.15-17 Color Doppler ultrasonography shows popliteal narrowing, with increased peak systolic velocity in the stenosis. The intramural cyst lies eccentric to the artery and contains low-level echoes, as demonstrated in our patient. CT scanning and MRA can reveal the popliteal stenosis.
There are various options for treating cystic adventitial disease of the popliteal artery. Aspiration of the cystic collection has been performed successfully, although there is a higher incidence of recurrence with this procedure than with others.18 Partial or complete excision without opening the artery has been performed with good results.19 Most cases have been treated with resection of the affected segment and an autogenous vein graft reconstruction or a patch, such as was used in our patient.20
Cystic adventitial disease of the popliteal artery is an uncommon cause of vascular insufficiency, but recognizing it is important because early diagnosis and therapy are curative and prevent the onset of severe vascular complications. The diagnosis should be considered in young men who present with vascular insufficiency but have no other evidence of atherosclerotic disease. Angiography, ultrasonography, CT scanning, or MRA may be used to evaluate popliteal fossa anatomy and vascular comprise.
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