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   Table of Contents      
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 2  |  Page : 135-138

Cystic Adventitial Disease of the Popliteal Artery : A Rare Case with Review of Literature


Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

Date of Submission27-Feb-2020
Date of Decision15-Apr-2020
Date of Acceptance07-May-2020
Date of Web Publication29-Aug-2020

Correspondence Address:
MD Ayush Jain
Radiodiagnosis, Room 114, RMO Hostel, KEM Hospital Campus, Parel-400012, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mamcjms.mamcjms_15_20

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  Abstract 


Cystic adventitial disease (CAD) is a rare non-atheromatous affection of the popliteal artery, often misdiagnosed, leading to increasing morbidity and eventual disability. More commonly unilateral, this condition tends to affect males in their fourth and fifth decades. Diagnosis is usually suggested with ultrasonography (USG) and is further confirmed with digital subtraction angiography (DSA). Magnetic resonance imaging (MRI) angiography is another non-invasive method for diagnosis of CAD. Where DSA gives information about the luminal contour and severity of narrowing, MRI helps in evaluation of the extramural component and surrounding soft tissue. Management is in the form of resection of the involved popliteal artery segment.

Keywords: claudication, cystic adventitial disease, popliteal artery


How to cite this article:
Jain A, Sankhe SS, Sontakke DM. Cystic Adventitial Disease of the Popliteal Artery : A Rare Case with Review of Literature. MAMC J Med Sci 2020;6:135-8

How to cite this URL:
Jain A, Sankhe SS, Sontakke DM. Cystic Adventitial Disease of the Popliteal Artery : A Rare Case with Review of Literature. MAMC J Med Sci [serial online] 2020 [cited 2020 Oct 27];6:135-8. Available from: https://www.mamcjms.in/text.asp?2020/6/2/135/293881



Key Messages:

CAD of the popliteal artery is an uncommon but important non-atheromatous cause of claudication in middle-aged men.

Etiology of this condition is unclear, however imaging features reveal uni or multiloculated cysts causing compression and luminal compromise of the vessel.

Ultrasound is the primary imaging modality, while MRI and DSA are confirmatory.

Early recognition is important to allow complete resolution post-surgery.


  Introduction Top


Despite its short length, the popliteal artery (PopA) is affected by a wide range of pathologies. Among the commoner causes such as atherosclerosis and aneurysms, cystic adventitial disease (CAD) and PopA entrapment syndrome are rarer pathologies.[1] CAD is a rare non-atheromatous occlusive-stenotic disease of the PopAs characterized by cyst formation within the adventitial layer of the arterial wall. Clinical presentation is in the form of limb claudication, usually in males of fourth and fifth decades without any evidence of atherosclerotic vascular disease. Commonly misdiagnosed as peripheral arterial disease, this rare condition is important to recognize for effective management and follow-up of these patients. Imaging modalities such as ultrasonography (USG), computerized tomography (CT) angiography, magnetic resonance imaging (MRI) angiography and digital subtraction angiography (DSA) are useful for accurate diagnosis and determination of the extent of involvement.

We hereby present a case of CAD involving the PopA, who was being treated as peripheral arterial disease until he underwent DSA and subsequent MRI angiography.


  Case Report Top


A 40 year old male patient was presented to the surgical outpatient department (OPD) with the complaints of pain in the left leg and calf on walking since 4 months. He could walk for a distance of about 200 metres before having to stop for rest. There were no similar symptoms on the right side. No shooting sensation or any neural complaints were reported. Patient was not a diabetic. There was no history of smoking.

Clinical examination of the left leg revealed a normal texture of the overlying skin. No signs of ischemia or gangrene were present. Peripheral pulses were palpable, however were feeble on the left side. USG Doppler of the left lower limb had been performed multiple times elsewhere which had repeatedly shown subacute thrombosis of the PopA with mild atherosclerotic changes in the form of intimo-medial thickening. Even at our institution, a USG examination performed just prior to scheduling the patient for DSA was reported as popA thrombosis. Patient was then scheduled for DSA of the left lower limb with a plan for stenting. The study revealed a slit-like configuration of the popA, with complete obliteration of its lumen in the mid-segment. The vessel was seen to be reformed by the arterial arcade around the knee joint [Figure 1]c and d. These findings suggested the presence of an extrinsic compression of the popA with distal reformation. Stenting was not performed and MRI was suggested to evaluate for the cause of extrinsic compression. MRI [Figure 2]a and 2b showed a uniloculated cystic hyperintense lesion causing obliteration of the lumen of left mid popA. Contrast MRI angiogram of the left popA showed severe extrinsic compression of the mid vessel. No abnormal dilatation of the vessel was seen. The cystic lesion was hypointense on T1 weighted images, excluding a possibility of a thrombosed aneurysm. No obvious communication with the joint cavity was seen. The lesion was not in the expected location of a bakers cyst. A repeat USG was performed which showed [Figure 1]A and 1B a well-defined anechoic oblong cystic lesion causing compression and luminal displacement of the mid left PopA. The compressed vessel showed high-velocity flow with a high resistance waveform. The distal anterior tibial and posterior tibial arteries showed low resistance flow with normal velocity. Based on these imaging findings, a diagnosis of CAD of the popliteal artery was made.
Figure 1 (a): USG and Doppler images in the left popliteal fossa showing a well defined anechoic cyst causing anterior displacement and obliterating the lumen of left PopA. No internal vascularity is seen. (b–c) showing the cyst (C) in relation to the artery (A) and vein (V)

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Figure 2 (a–b) DSA of the left lower limb showing severe eccentric extrinsic narrowing of the mid left popliteal artery (PopA) (Black arrow) with distal reformation. Few surrounding collateral vessels are seen. Also note that no signs of atherosclerosis are seen on the given images. (c–e) magnetic resonance imaging (MRI) and MRI Angiogram of the left PopA. (C) Axial T2 and (d) PDFS images at the level of the distal femur showing a well defined cyst (White arrow) obliterating the lumen of the popliteal artery. MR Angiogram (e) confirming DSA findings. PDFS, proton density fat suppressed

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As the cystic lesion could be accessed by USG, a trial of USG guided aspiration of the cyst contents was given. The procedure was performed with aspiration of 2 ml of straw-coloured fluid with a thick consistency. Procedure was uneventful and there was significant reduction in the cyst size, with relief in the patient symptoms on follow-up. Currently the patient is on follow-up with USG examinations performed every 6 months to look for increase in size of the cyst and symptoms, which would mandate surgery.


  Discussion Top


The PopA is a direct continuation of the superficial femoral artery as it exits through the adductor hiatus. Normal location of the PopA is between medial and lateral heads of gastrocnemius muscle and posterior to the popliteus. The reason why few pathologies preferentially affect the PopA lies in its embryology. Lower limb arteries develop from two basins i.e. sciatic (axial) basin and basin of the external iliac artery. The sciatic artery is formed on day 30 of embryonic life and continues along with the sciatic nerve into the leg via the posterior flexors of the thigh. At around the same time(day 32), a second arterial segment is formed − the external iliac artery passing through the anterior flexors, giving rise to the femoral artery. In the subsequent week of development, a communicating branch from the femoral artery is formed which communicates with the proximal axial artery via the adductor hiatus.[2] Thus, the popliteal artery is formed in three segments, which makes it prone to developmental anomalies.

CAD of the popliteal artery is one such disorder, which results in mucinous cyst formation within the adventitial layer of the popliteal artery walls.[3] CAD was first described by Atkins and Key[4] in 1946, where they described cystic degeneration of the external iliac artery. Since then, approximately 600 such cases have been reported in the literature. Men are more commonly affected than women with a ratio of 4:1. The mean age of presentation is 46 years and the most commonly involved site is the popliteal segment of external iliac artery.[5] The condition is usually unilateral, although bilateral cases have been reported.

Multiple theories for etio-pathogenesis of CAD have been described including chronic trauma, systemic collagen disorders and that of true ganglion formation. However, the most accepted theory is that of connection of the cyst with the neighboring joint. Cyst formation begins as a small rent within the joint cavity with fluid tracking along the branch of a neighboring artery.[5] Hence, it may be worthwhile to perform MRI for the same and demonstrate a communication which may change the management. Commonest clinical presentation is in the form of intermittent claudication (89% of cases), although few cases of acute and chronic limb ischemia have been described.[6]

Imaging modalities for evaluation include USG, CT angiogram (CTA), MR angiogram (MRA) and DSA. USG demonstrates anechoic/hypoechoic well-defined round lesions with posterior acoustic enhancement originating from the arterial walls. Sometimes, they may show low-level internal echoes due to proteinaceous content.

CT findings show fluid density masses surrounding and causing compression of the affected artery. MRI shows uni or multilocular cystic lesions, hyperintense on fluid sensitive sequences. Owing to their mucinous content, cysts may also show hyperintensity on T1 weighted images.[6] On contrast administration, there typically is peripheral rim enhancement of the cysts with non-enhancement of the mucinous component. MR angiography and DSA may show extrinsic compression of the vessel with smooth margins. The stenosis may appear scimitar shaped when the compression is eccentric or hourglass shaped when it is concentric.[7] DSA has been considered the gold standard for diagnosis of CAD. However, angiography may be non-specific, and even normal and in a few conditions, may mimic other causes of extrinsic compression.[8] Also, it comes with the cost of ionizing radiation and invasive nature.

Clinical differential diagnosis of CAD includes atherosclerosis, PopA entrapment syndrome (PAES) and PopA aneurysm. Evaluation of relation of the PopA with adjacent tendons is important to exclude PAES.

Imaging differentials for CAD include a thrombosed PopA aneurysm, ganglion cyst and a parameniscal cyst. A unilocular CAD may resemble a thrombosed aneurysm on ultrasound,[8] as was the case in our patient. However, DSA revealed a scimitar deformity suggestive of extrinsic compression. CAD at times may be extremely difficult to differentiate from parameniscal or bakers cysts, because many of them show communication with the joint cavity. Bakers cyst can be differentiated on the basis of its relation with the medial head of gastrocnemius and semimembranosus muscles. Parameniscal cysts are usually in proximity to menisci and are associated with meniscal tears.

Management of CAD, may be surgical or non-surgical. Surgical approach is recommended for symptomatic patients and includes complete resection of the affected arterial segment followed by re-anastomosis using a patch or graft. Other approaches such as percutaneous cyst aspiration, transluminal angioplasty with cyst evacuation have also been used.[6]

Financial support and sponsorship

None.

Conflicts on interest

There are no conflicts of interest.



 
  References Top

1.
Jarraya M, Simmons S, Farber A, Teytelboym O, Naggara N, Guermazi A. Uncommon diseases of the popliteal artery: a pictorial review. Insights imaging 2016;7:679-88.  Back to cited text no. 1
    
2.
Kuznetsov MR, Reshetov IV, Magnitskiy LA, Vasiliev V, Marchenko IP, Matveev AD et al. Embryology, anatomy and rare pathologies of the popliteal artery: peculiarities of surgical treatment. Angiol Sosud Khir 2018;24:146-57.  Back to cited text no. 2
    
3.
Motaganahalli RL, Smeds MR, Harlander-Locke MP, Lawrence PF, Fujimura N, DeMartino RR et al. A multi-institutional experience in adventitial cystic disease. J Vasc Surg 2017;65:157-61.  Back to cited text no. 3
    
4.
Atkins HJ, Key JA. A case of myxomatous tumour arising in the adventitia of the left external iliac artery. Br J Surg 1947;34:426–7.  Back to cited text no. 4
    
5.
Desy NM, Spinner RJ. The etiology and management of cystic adventitial disease. J Vasc Surg 2014;60:235-45.  Back to cited text no. 5
    
6.
Lejay A, Ohana M, Delay C, Georg Y, Girsowicz E, Thaveau F et al. Cystic adventitial pathology as an entity in peripheral arterial disease. J Cardiovasc Surg (Torino) 2015;56:1-2.  Back to cited text no. 6
    
7.
Flanigan DP, Burnham SJ, Goodreau JJ, Bergan JJ. Summary of cases of adventitial cystic disease of the popliteal artery. Ann Surg 1979;189:165.  Back to cited text no. 7
    
8.
Brodmann M, Stark G, Pabst E, Seinost G, Schweiger W, Szolar D et al. Cystic adventitial degeneration of the popliteal artery—the diagnostic value of duplex sonography. Eur J Radiol 2001;38:209-12.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]



 

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