ABSTRACT: We report a case of adventitial cystic disease of the right popliteal artery in a 43-year-old female patient admitted with complaints of her right foot “falling asleep,” claudication, and cool toes for 6 days. Noninvasive studies indicated some degree of arterial insufficiency of the right lower extremity. The lesion was identified by angiography and treated with percutaneous transluminal angioplasty resulting in segmental thrombosis of the popliteal artery. The patient subsequently underwent resection of the abnormal segment of the blood vessel and interposition vein grafting. Endovascular treatment of this disease process with currently available technology is not the treatment of choice. In the acute setting, the best treatment option is resection of the affected segment of blood vessel and interposition grafting.
VASCULAR DISEASE MANAGEMENT 2012:9(9):E155-E158
Fewer than 400 reports of adventitial cystic disease (ACD) have been published since the disease was first described in 1947.1 While the cyst in the initial case presentation occurred in the external iliac artery, cases of adventitial cystic disease have since been observed to occur in the axillary, brachial, radial, ulnar, femoral, and popliteal arteries as well as in veins, including the saphenous vein. Of these sites, the popliteal artery is overwhelmingly the most common location with 311 of 375 reported cases occurring at that site as of 2005.2 The disease predominately affects men with a 15:1 ratio of cases in men to women.2 Classically the disease presents within the fourth or fifth decade of life in males (median onset at age 42),3 and in females in the sixth decade of life, in otherwise relatively healthy individuals with few atherosclerotic risk factors.2,4 Patients typically present with sudden onset and rapid progression of severe intermittent claudication.5 While the prevalence of adventitial cystic disease is 1 in 1200 cases of claudication, the disease is becoming increasingly recognized.1,5
Several treatment modalities have been utilized to treat ACD, but despite the evolution of endovascular technology, percutaneous transluminal angioplasty has been reported as a suboptimal treatment option. Here we present a case that illustrates both this observation as well as the currently accepted gold standard for management of this disease process in the acute setting.
A 43-year-old African American female patient presented with complaints of her right foot falling asleep, intermittent claudication in the right calf upon walking approximately 20 feet, and cool toes for 6 days. Past medical history was unremarkable for atherosclerotic risk factors except for the presence of obesity and a 20-pack/year history of tobacco use.
Exam revealed the right foot to be cool distally with decreased sensation over the great toe but good capillary refill and normal strength. On the right foot a dorsalis pedis signal was present as was a weak posterior tibial signal.
Noninvasive studies showed a normal ankle brachial index (ABI) on the right at 1.03 with the left ABI 1.37. However, diminished pulse volume recordings distally indicated some degree of arterial insufficiency of the right lower extremity. A right lower extremity angiogram revealed a smooth severe stenosis at the level of the popliteal artery (Figure 1A). The lesion was percutaneously angioplastied with a 5 mm balloon at 8 atm for 30 seconds, resulting in segmental thrombosis of the blood vessel (Figure 1B).
Vascular surgery was consulted, the patient was heparinized, and an MR study of the popliteal fossa obtained. This scan demonstrated that the popliteal artery was enlarged in overall diameter with an abnormal lentiform region of hyperintensity on T2 imaging. This was felt to be consistent with a complex 2 cm ganglion cyst arising from the posterior root of the medial meniscus with extension of the ganglion into the adventitia of the popliteal artery. A convex region exerting a mass effect upon the popliteal artery was noted, approximately 6 cm in length and 2.5 cm in diameter.
Subsequently the patient underwent exploration of the popliteal artery via posterior approach. We noted an obvious dissection of the popliteal artery, presumably at the level of the prior angioplasty. The abnormal, dilated segment of the popliteal artery measuring approximately 3 to 4 cm long, was excised. Upon transaction of the popliteal artery at the proximal margin of the normal-appearing vessel, a significant amount of gelatinous material was encountered, confirming adventitial cystic disease. The vessel was reconstructed with a non-reversed great saphenous vein (segment harvested first with patient in the supine position) interposition graft, resulting in restoration of normal blood flow to the foot.
Pathologic evaluation of the cyst remnant and artery showed benign fibrous tissue along with vascular tissue with periadventitial mild chronic inflammation.
The patient’s clinical course was complicated by the occurrence of bilateral lobar pulmonary emboli 9 days following surgery. Hypercoagulable work-up was negative. The patient was heparinized, and subsequently begun on a coumadin regimen. Lower extremity venous duplex revealed a tibial vein deep venous thrombosis of the contralateral lower extremity. At 2 months following surgery, ABI on the right lower extremity was 1.43, with triphasic waveforms throughout the lower extremity. Duplex imaging showed a widely patent graft as well as surrounding artery and vein.
At 9-month follow-up, both feet were well perfused with palpable pedal pulses. The graft remained widely patent with normal flow and no evidence of graft stenosis (Figure 2). The ABI on the right lower extremity was 1.24, and on the left was 1.33.
Several treatment modalities have been considered for treating adventitial cystic disease of the popliteal artery. As demonstrated in this case, an endovascular approach with percutaneous transluminal balloon angioplasty (PTA) is not an effective treatment. While it may appear attractive as endovascular technology continues to advance and expand treatment options, the method is plagued by a high recurrence rate and the literature repports suboptimal results.5 In perhaps the most dramatic demonstration that PTA does not provide lasting benefits in ACD, Khoury describes a case in which the popliteal artery stenosis recurred within 24 H of PTA therapy.3 Part of the failure of PTA as a treatment can be recognized in the pathology of the disease with the lack of involvement of the intimal layer. In ACD the mucinous cyst lies between the media and adventitia of the affected artery, providing external compression of the lumen.2,5 Luminal compression in PTA, even if able to elicit the release of cystic material, does nothing to treat the underlying disease process, making recurrence likely. It has also been noted that the affected vessels in cases of ACD are more compliant than atherosclerotic vessels, making them more likely than atherosclerotic vessels to re-stenose following dilation.5 However, even in cases in which PTA was coupled with intraluminal stenting, stenosis and symptoms of claudication have been noted to recur.6 Self-expanding stents may be another option, however limited information exists in the literature regarding use of these stents in this specific pathology. Perhaps the most compelling reason for avoiding the use of PTA, however, is the likely disruption of the healthy intima and subsequent thrombosis of the blood vessel, as was the case in our patient.
While the affected vessel remains patent, aspiration or simple excision of the cyst may be considered. Cyst aspiration (generally performed under ultrasound or CT guidance) is not always possible due to the high viscosity of the mucinous cyst contents.6 Other requirements for cyst aspiration include a safe path to the cyst and a unilocular occurence.6 Even when possible, cyst aspiration does not correct the underlying pathology, as the mucin-secreting mesenchymal cells remain at the site,1 and this method shows a recurrence rate of 10%.3 In our patient, this was not an option because of the acute nature of the problem.
Simple excision of the cyst is an alternative in a non-occluded vessel, but there is concern that a resultant weakening of the arterial wall will occur, which would require patching.7,8 Among the early patients treated with cyst evacuation and patch angioplasty using the autogenous vein, 2 of 9 developed patch aneurysms.4
Although invasive, the optimal therapy is a complete excision of the cyst and artery with reconstruction by arterial bypass of the affected artery, preferably with the saphenous vein.7 While this treatment is unequivocally the preferred method of treatment when the vessel is occluded, there is some argument within the literature regarding the necessity of this vessel and cyst excision when the vessel remains patent, with some authors stating this course of treatment is only appropriate in the case of arterial occlusion.6 Certainly in the acute setting such as ours, we concur with the position that resection of the cyst and involved artery with repair using a saphenous vein graft would be appropriate treatment of ACD and would also provide the most reliable method of treatment.1,8
ACD of the popliteal artery is a rare condition typically seen in middle-aged adults with few atherosclerotic risk factors presenting with sudden onset of rapidly progressive claudication. In cases of adventitial cystic disease with acute occlusion of arterial flow, resection of the artery with interposition grafting appears to be the best treatment modality. In non-occlusive cases, cyst aspiration or simple excision of the cyst may also be considered, but these methods are plagued by significant recurrence rates. As we have shown in this case, as has been noted elsewhere in the literature, endovascular treatment by percutaneous transluminal angioplasty is neither an effective nor appropriate method of treatment of ACD.
- Tsilimparis N, Hanack U, Yousefi S, Alevizakos P, Rückert RI. Cystic adventitial disease of the popliteal artery: an argument for the developmental theory. J Vasc Surg. 2007;45(6):1249-1252.
- Rutherford RB. Vascular Surgery. 6th ed. Philadelphia: Elsevier/Saunders; 2005.
- Khoury M. Failed angioplasty of a popliteal artery stenosis secondary to cystic adventitial disease--a case report. Vasc Endovascular Surg. 2004;38(3):277-280.
- Flanigan DP, Burnham SJ, Goodreau JJ, Bergan JJ. Summary of cases of adventitial cystic disease of the popliteal artery. Ann Surg. 1979;189(2):165-175.
- Fox RL, Kahn M, Adler J, et al. Adventitial cystic disease of the popliteal artery: failure of percutaneous transluminal angioplasty as a therapeutic modality. J Vasc Surg. 1985;2(3):464-467.
- Deridder P, Van der Tempel G, Vanoverbeke H, Decoster J, Verheul G. Cystic adventitial disease of the popliteal artery: a case report. EJVES Extra. 2004;8:123-126.
- Vasudevan A, Halak M, Lee S, Ong S, Nadkarni S. Cystic adventitial disease: a case report and literature review. ANZ J Surg. 2005;75(12):1120-1122.
- di Marzo L, Peetz DJ Jr, Bewtra C, Schultz RD, Feldhaus RJ, Anthone G. Cystic adventitial degeneration of the femoral artery: is evacuation and cyst excision worthwhile as a definitive therapy? Surgery. 1987;101(5):587-593.
- Rai S, Davies RS, Vohra RK. Failure of endovascular stenting for popliteal cystic disease. Ann Vasc Surg. 2009;23(3):410.e1-410.e5.
From the 1Division of Vascular Surgery, Northshore University HealthSystem, Skokie, Illinois and the 2Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire.
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. Dr. Gupta reports a pilot grant from Medtronic for his institution. Dr. Spangler reports no conflicts of interest regarding the content herein.
Manuscript received December 26, 2011, provisional acceptance given January 23, 2012, final version accepted January 27, 2012.
Address for correspondence: NavYash Gupta, MD, FACS, Northshore University HealthSystem, Division of Vascular Surgery, Suite 355, 9977 Woods Drive, Skokie, IL 60077, USA. Email: email@example.com