Cystic adventitial disease (CAD) is a rare vascular condition which most commonly causes localized stenosis or occlusions of the popliteal artery. It is characterized by the presence of unilocular or multilocular mucin-containing cysts localized in the adventitial layer of the affected vessel. The presence of these cysts commonly results in narrowing of the arterial lumen, leading to intermittent claudication with relatively sudden onset and rapid progression. Cystic adventitial disease is most common in otherwise healthy young or middle-aged males without other signs of atherosclerosis or cardiovascular risk factors. Here, we describe a case of right popliteal artery CAD in a 33-year-old male soccer player and its subsequent management.
A 33-year-old man presented with a 1-year history of worsening right lower-extremity pain and right foot numbness with activity. Onset of symptoms would occur after approximately 3 to 5 min of running or while seated for a prolonged period with his knee in a flexed position. He had relief of his symptoms with rest. He is a physically active soccer athlete with no history of significant trauma. He had one isolated right gastrocnemius tear approximately 1.5 years prior to presentation. He had no known risk factors for vascular disease. Initial examination at rest showed a slightly weaker posterior tibial and dorsalis pedis pulse on the right compared to the left, while the remainder of the lower extremity examination was within normal limits bilaterally. Ankle brachial indexes (ABI) obtained at rest and posttreadmill exercise (5-min duration) were 1.09 (R)/1.08 (L) and 0.64 (R)/1.30 (L), respectively. Arterial duplex ultrasound detected >90% stenosis within the right popliteal artery. CT angiography revealed a high grade narrowing of the right popliteal artery near the femoral condyle, with possible hypodense material and a thin enhancing rim with a nonenhancing center confined within the adventitia (Fig. 1A, B). Cyst dimensions within the adventitia measured as 1.2 by 2.4 cm. A diagnosis of CAD of the right popliteal artery was suspected based on the CT findings. Given the suspicion of CAD a vascular surgery consultation was obtained. Subsequently, the cyst was resected via a posterior approach of the right popliteal fossa. The right saphenous vein was used as an anastomosis between the popliteal and tibial arteries. Histological examination revealed a segment of dilated vessel measuring 2.2 × 1.3 × 1.2 cm with a circumferential cyst filled with clear viscid fluid compressing the lumen of the vessel (Fig. 2). Microscopically, the tissue showed myxoid, cystic degeneration within the adventitia which was further consistent with CAD.
At 1 month postsurgery follow-up the patient had improved exercise tolerance with no postoperative complications. Follow-up arterial duplex ultrasound demonstrated no aneurysmal degeneration of the graft with normal patency; however, there was an area of focal narrowing at the distal graft site which most likely represented residual hyperplasia distal to the stenotic segment. Six-month follow-up with vascular surgery revealed continued improvement, with the residual symptom of minimal pain in the popliteal fossa only with running on concrete. He has no subsequent symptoms or limitations with soccer or other activities. Repeat arterial duplex ultrasound showed multiphasic waveforms throughout the right end-to-end popliteal artery bypass graft with no flow velocity elevations present, which was improved from prior studies. For surveillance, he will have an arterial duplex yearly for 3 years. If there continues to be no issues with the vein graft at that point, he will follow up as needed for recurrence of symptoms.
Cystic adventitial disease is a rare vascular disease which can involve the popliteal, external iliac, femoral, radial, and ulnar arteries (1,2). Cystic adventitial disease often affects individuals in the fourth and fifth decade of life; however, it has been reported in individuals ranging from 10 to 77 years of age. The incidence of CAD is 1 in 1200 cases of claudication and 1 in 1000 femoral angiograms with a male predominance of 15:1 (3). Cystic adventitial disease most often affects the popliteal artery and is characterized by intermittent claudication with sudden onset of pain located behind the knee which may quickly resolve after a short period unlike typical episodes of claudication due to atherosclerotic disease (4). Cystic adventitial disease has the potential for rapid progression due to excessive production of cystic content. Previous reports have shown various types of cystic content including fibrinogen, hydroxyproline, hyaluronic acid, or mucin (2,5).
The etiology of CAD remains controversial and four potential theories have been proposed, which include the following: 1) repetitive local trauma, 2) systemic disease, 3) synovial/ganglion, and 4) embryological.
The repetitive local trauma theory postulates that due to the location of affected vessels near joints, they are subject to repetitive microtrauma that can result in disruption of the adventitia from the media, resulting in intramural bleeding and development of cysts due to enzymatic activity within the vessel wall (6). There have been reports of trauma induced cysts; however, this theory is controversial as CAD can occur in children and is rare in athletes (2,5). In this case report, the subject did have a prior gastrocnemius tear as a potential risk factor for developing CAD. Linquette et al. proposed the systemic disease theory which suggests that CAD is related to a generalized disorder of myxomatous degeneration. This theory has failed to gain adequate support as patients did not show systemic lesions on follow-up (7,8). The synovial/ganglion theory and the embryological theory have recently gained more favor in the etiology of CAD. The synovial/ganglion theory proposes that adventitial cysts develop due to migration of synovial ganglions from the adjacent joint capsule or tendons, along vascular branches to the adventitia (9,10). The embryological theory hypothesizes that during development, mesenchymal cells are incorporated into the adventitia from adjacent joint tissue. These cells then secrete mucin over a number of years giving rise to adventitial cysts which have the potential to encroach on the arterial lumen (2,8). Because there is no single unifying theory able to account for the pathogenesis of all clinical cases, CAD may be better explained by a combination of multiple theories. Our patient, with an extensive history of soccer and athletic activities, was at an increased risk for repetitive microtrauma of the popliteal artery adventitia. CTA of his lower extremity revealed several intermediate oblong calcifications within the rectus femoris muscle which may be an indication of prior trauma in addition to his prior gastrocnemius tear.
The differential diagnosis for intermittent claudication in young patients includes: popliteal artery entrapment syndrome, chronic exertional compartment syndrome, popliteal artery CAD, popliteal artery aneurysm, popliteal artery atherosclerotic disease, and embolic disease (11,12). In addition to obtaining an extensive clinical history and physical examination, initial work-up includes obtaining ankle-brachial indexes and an arterial duplex ultrasound. Conventional arteriography was the gold standard for diagnosis; however, this has since been replaced with CT or magnetic resonance (MR) angiography as CT and MR are more likely to identify the hypodense or hypointense cyst contents within the adventitia (1,2,13).
Treatment options for CAD include: ultrasound-guided cyst aspiration, cyst resection with arterial preservation, or excision of involved arterial segment with interpositional bypass graft (1,2). While cyst aspiration is less invasive, there are reports of recurrence and the success rate is dependent upon the type of cystic contents (13,14). For young patients, the preferred treatment method is resection of the affected arterial segment with bypass grafting, because this has a much higher success rate (1,2,15). Long term follow-up including ABI measurement and duplex ultrasound is required due to the possibility of CAD recurrence and/or graft occlusion which could result in thrombosis (16).
Cystic adventitial disease is a rare vascular disease that is predominantly seen in males with minimal or no cardiovascular risk. The typical symptoms of popliteal artery CAD include relatively sudden onset of claudication and lower extremity pain with activity. Clinical history, physical examination, and CT or MR angiography are crucial in making a definitive diagnosis. Surgical intervention with cyst resection and venous bypass grafting is considered the treatment of choice in CAD.
The authors declare no conflict of interest and do not have any financial disclosures.
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