Journal of Vascular Surgery
Volume 51, Issue 2 , Pages 471-474, February 2010

High spatial resolution magnetic resonance imaging of cystic adventitial disease of the popliteal artery

  • Ismaeel M. Maged, MD

      Affiliations

    • Department of Radiology, University of Virginia Health System, Charlottesville, Va
    • Department of Radiology, Suez Canal University, Ismailia, Egypt
    • ,
  • Ulku C. Turba, MD

      Affiliations

    • Department of Radiology, University of Virginia Health System, Charlottesville, Va
    • ,
  • Ahmed M. Housseini, MD

      Affiliations

    • Department of Radiology, University of Virginia Health System, Charlottesville, Va
    • Department of Radiology, Suez Canal University, Ismailia, Egypt
    • ,
  • John A. Kern, MD

      Affiliations

    • Department of Surgery, University of Virginia Health System, Charlottesville, Va
    • ,
  • Irving L. Kron, MD

      Affiliations

    • Department of Surgery, University of Virginia Health System, Charlottesville, Va
    • ,
  • Klaus D. Hagspiel, MD

      Affiliations

    • Department of Radiology, University of Virginia Health System, Charlottesville, Va
    • Corresponding Author InformationReprint requests: Klaus D. Hagspiel, MD, Division of Noninvasive Cardiovascular Imaging, Department of Radiology, University of Virginia Health System, Box 800170, Lee St, Charlottesville, VA 22908

Received 18 June 2009; accepted 25 August 2009.

Article Outline

High spatial resolution magnetic resonance imaging (MRI) of patients with cystic adventitial disease can demonstrate connections between cysts in the adventitia and the adjacent joint, which is important for successful treatment. The inability to identify these during surgery can lead to a recurrence; thus, high spatial resolution MRI has the potential to affect therapy. This article presents the high spatial resolution MRI findings of cystic adventitial disease in a series of three consecutive patients and discusses the relevance of these findings to the etiology and therapy.

 

Cystic adventitial disease, a rare vascular disorder of the peripheral arteries characterized by the collection of a mucinous substance inside the adventitia of the vessel,1 was first described by Atkins and Key in 1947.2 The main symptom is progressive calf claudication, without evidence of atherosclerotic disease.3 The therapeutic options vary, depending on the patient's symptoms and the anatomic site affected.4 The etiology of cystic adventitial disease is not definitely known and several theories exist.5

We describe our experience with three consecutive patients with popliteal cystic adventitial disease, in all of whom connections between adventitial cysts and the adjacent joint were demonstrated on high spatial resolution (HSR) magnetic resonance imaging (MRI), a finding that is thought to support the developmental theory by previous authors6 and that has been described in two previously published cases.7 All scans were performed on 1.5 T MR systems (Siemens Sonata or Avanto, Siemens Medical Systems, Malvern, Pa) using dedicated peripheral phased-array coils.

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Case reports 

Patient 1 

A 48-year-old woman presented with progressive left lower extremity claudication which began 8 months earlier. MR angiography (MRA) showed a 2.5-cm-long occlusion of the left popliteal artery, with no other abnormalities. HSR MRI demonstrated a lobulated, septated cystic mass within the artery extending anteriorly towards the knee joint, with intra-articular cysts showing muscle isointensity on T1w and hyperintensity on T2w and short tau inversion recovery (STIR) images without enhancement on the contrast-enhanced T1w images. The diagnosis of cystic adventitial disease was made.

A catheter digital subtraction arteriogram (DSA) confirmed the MRA findings. Surgical resection of the affected popliteal segment and ligation of the connections with the intra-articular cysts was done with placement of a venous interposition graft. The patient remains asymptomatic, with normal peripheral pulses, 3 years after surgery.

Patient 2 

An 18-year-old man presented with pain behind his left knee for 3 years. HSR MRI and MRA demonstrated thickening of the wall of the popliteal artery caused by multiple well-circumscribed cystic lesions showing high-signal intensity on T2 weighted images (Fig 1, A). These cysts were connected to intra-articular cysts within the adjacent knee joint (Fig 1, B) by a stalk with identical signal characteristics. The diagnosis of cystic adventitial disease was made. Open surgical enucleation of the adventitial cysts was performed through a posterior approach, with expression of mucinous gel-like material characteristic of this disease (Fig 1, C). The stalk was not ligated.

  • View full-size image.
  • Fig 1. 

    A, An axial T2-weighted magnetic resonance image (MRI) of adventitial cystic disease in patient 2 shows thickening of the wall of the popliteal artery caused by multiple well-circumscribed high-signal intensity lesions (arrowheads) representing adventitial cysts. A stalk is seen connecting these cysts with the adjacent knee joint (arrow). B, Axial T2-weighted MRI slightly above the image in Panel A shows a high-signal intensity intra-articular cyst at the intercondylar notch of the femur (arrow) as well as a smaller periarterial lesion (arrowhead). C, Intraoperative photograph shows the mucinous material (arrow) after incision of the popliteal artery wall characteristic of adventitial cystic disease. D, Axial T2 weighted MRI of the same knee 6 months after cyst enucleation shows recurrent high-signal intensity foci representing adventitial cysts (arrowheads) within the popliteal arterial wall and beyond, resulting in an approximately 50% narrowing of the arterial lumen (asterisk). The connecting stalk between these cysts and cysts within the adjacent knee joint, which was not identified intraoperatively, is again noted (arrow). V, Popliteal vein. E, Sagittal T2-weighted MRI of the knee 6 months after cyst enucleation shows multiple high-signal intensity adventitial cysts arising from the wall of the popliteal artery (arrowheads) connected to intra-articular cysts behind the posterior cruciate ligament (asterisk) by the clearly identifiable stalk (arrows).

He was asymptomatic at discharge but returned 6 months later with recurrent symptoms. Repeat HSR MRI and MRA showed recurrence of popliteal wall cysts, presumably through the stalk and the intra-articular components, resulting in approximately 50% stenosis of the popliteal artery (Fig 1, D and E). Intravascular ultrasound imaging demonstrated multiple echo-free adventitial cysts. A left lower extremity arteriogram confirmed the stenosis. Percutaneous transluminal angioplasty (PTA) of the lesion was performed with good result. The patient has remained asymptomatic for 2 years.

Patient 3 

A 43-year-old man presented with a 6-month history of left calf claudication and an ankle-brachial index of 0.8. DSA demonstrated a 6-cm-long occlusion of the left distal superficial femoral artery and the popliteal artery, with no other abnormalities. HSR MRI revealed an oval, well-circumscribed cyst completely obliterating the lumen of the artery and displaying high signal intensity on STIR images and relative hypointensity compared with muscle on T1w, without enhancement on postgadolinium T1w images. The cyst was connected by a thin stalk to multiple cysts within the adjacent knee joint (Fig 2, A-D). All cysts and the stalk showed identical signal behavior, and cystic adventitial disease was diagnosed.

  • View full-size image.
  • Fig 2. 

    A, Axial T1-weighted magnetic resonance image (MRI) of patient 3, who had adventitial cystic disease of the left popliteal artery and lower extremity claudication, shows a homogeneous structure of slightly lower signal intensity than skeletal muscle, representing an adventitial cyst completely obliterating the lumen of the popliteal artery (asterisk) as well as cysts in the joint (white arrows), representing intra-articular cysts. The popliteal vein (black arrow) and two small contributing veins (black arrowheads) are muscle isointense on this sequence and immediately posterior and to the left of the occluded artery. B, Axial 3D postgadolinium volumetric interpolated breath-hold examination (VIBE) MRI at the same level as Panel A shows rim enhancement but no central enhancement of the adventitial cyst occluding the popliteal artery (asterisk) as well as the cysts inside the joint (short arrows). Note the patent popliteal vein (long arrow) and two small veins draining into it (arrowheads). C, Axial short tau inversion recovery (STIR) MRI at the same level as Panels A and B shows the hyperintense cyst completely obliterating the lumen of the popliteal artery (asterisk). This sequence also shows the popliteal vein (long arrow), two small contributing veins (arrowheads), and multiple well-circumscribed cysts inside the adjacent knee joint (short arrows). D, Sagittal STIR MR image shows the adventitial cyst (arrowhead) within the popliteal artery and the small stalk connecting it with the knee joint (arrow). The asterisk designates the popliteal vein. E, Intraoperative photograph shows the popliteal artery, which is distended by the cyst (between the red vessel loops) and the discharge of mucinous material (arrow) after incision of the arterial wall.

During the surgical repair, the adventitia of the affected artery was opened, resulting in discharge of mucinous material (Fig 2, E). The distal superficial femoral artery was dissected distally to the proximal popliteal artery above the knee. The entire length of the cyst was opened, resulting in the discharge of mucinous material (Fig 2, E). The cyst wall was removed with resection and ligation of the connection stalk. The popliteal artery was chronically scarred and was completely resected. Endoscopically harvested saphenous vein was used to create an interposition of a femoral-to-popliteal artery graft. The patient remains asymptomatic 2 years after surgery, with an ankle-brachial index of 1.16.

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Discussion 

Cystic adventitial disease is a rare vascular condition that most often affects young or middle-aged men, with claudication symptoms being the most common presentation.8 Other symptoms include lower extremity pain and a slowly growing soft tissue mass.3 Several theories have been postulated for its pathogenesis:

•The trauma theory suggests minor repetitive trauma causes chronic degeneration of the adventitia;

•the systemic disorder theory suggests that adventitial cyst formation is part of a generalized connective tissue disorder;

•the ganglion theory suggests that synovial cysts track along small vascular branches and ultimately implant in the adventitia of larger adjacent vessels; and

•the developmental theory suggests the presence of ectopic mucin-secreting mesenchymal cells inside the adventitia derived from and connected with the nearby joints.5

Most authors consider MRI with MRA the preeminent diagnostic modality, although some prefer color-coded Doppler ultrasound imaging.9 MRI shows the cyst originating in the vessel wall as well as connections with the adjacent joint, especially on T2-weighted or fluid-sensitive STIR sequences, where the lesions appear bright with no or only rim enhancement on gadolinium-enhanced sequences. MRA demonstrates the stenosis or occlusion of the affected artery.3, 6 Owing to the nonspecific presentation and the rarity of cystic adventitial disease, the diagnosis is frequently not made before operation.10 In all our patients, however, the diagnosis was made preoperatively, which could be attributed to the use of HSR MRI.

A number of therapeutic strategies exist. The two main surgical approaches are resection of the diseased segment, with interposition of an autologous vein graft, and cyst enucleation. The former is considered the treatment of choice by many vascular surgeons, even in patients without complete occlusion.6

The different therapeutic approaches result in variable recurrence rates. Resection of the affected arterial segment with a bypass vein graft is indicated in the setting of complete arterial occlusion with weakened arterial wall or in case of secondary intimal ulceratrion.11 Resection with a vein graft has a recurrence rate of about 5%, and cyst evacuation with removal of the wall results in recurrence in about 6% of patients. Percutaneous cyst aspiration alone has a 34% recurrence rate. Other methods that are rarely used because of high recurrence rates are endovascular treatment with PTA or endovascular stent placement, both of which have reported recurrence rates as high as 100%.12, 13, 14

Resection of the adventitia, the diseased arterial segment, and all cystic structures during surgical exploration as well as ligation of any connection with the adjacent joint are known to lower the recurrence rate.6 However, even if all these steps are undertaken, the disease can recur either from cysts within the knee joint or even from cyst remnants deposited during the surgical procedure. This leads some vascular surgeons to resect and ligate fibrous tissues around the removed artery and cysts.4

Connections between adventitial cysts and the adjacent joint have been previously identified intraoperatively and were thought to support the developmental theory.5, 7 Further support for the developmental theory comes from immunohistochemical examinations of resected cysts.15 To the best of our knowledge, however, these connections have previously only been described in two patients.7 Identification and resection or ligation of these connections might improve the outcome of cyst evacuation. Although our series comprises only three patients, it represents one of the largest single-institution series.

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Conclusions 

HSR MRI allowed for identification of connections between adventitial cysts and the adjacent joint in three consecutive patients with cystic adventitial disease seen at our institution. This finding has important therapeutic implications, because failure to identify and ligate these connections may lead to recurrence in some cases.

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References 

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 Competition of interest: none.

 The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a competition of interest.

PII: S0741-5214(09)01809-6

doi:10.1016/j.jvs.2009.08.079

Journal of Vascular Surgery
Volume 51, Issue 2 , Pages 471-474, February 2010

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