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VEITH 2023 | Irwin V Mohan: Management of popliteal artery epithelial cyst disease

时间: 2024-03-20

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Adventitial Cystic Disease (ACD) is a rare disease.In 1947, Atkins et al. first described a case of adventitial cyst involving the right external iliac artery. To date, there have been about 600 reports covering about 800 patients, with over 80% of lesions involving the popliteal artery. At the recent 50th International Vascular and Endoluminal Vascular Congress (VEITH 2023), Prof. Irwin V Mohan from the University of Sydney, Australia, shared the management of popliteal artery epiphyseal cyst disease.

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ACD pathogenesis

Patients with arterial ACD are predominantly male, with a male-to-female ratio of about 4:1, while the male-to-female ratio for venous ACD is 1.6:1. The average age of onset of the disease is about 40 years old. At present, the etiology of ACD is still controversial, and there are four theories on its pathogenesis as follows.

Trauma theory: chronic degenerative lesions caused by repetitive trauma.

Ganglion theory: synovial cyst enlarges and spreads along vascular branches, eventually entering the periphery of neighboring great vessels.

Systemic disease theory: degeneration of the tunica albuginea and cyst formation are part of systemic connective tissue diseases.

Developmental theory: During the embryonic period, mucin-secreting mesenchymal stromal cells from nearby joints are located in the vascular epithelium, and the secretion of mucin leads to the formation of cysts.

Connections between adjacent joint capsules and cysts were found on imaging and intraoperatively in up to 17% of cases, supporting some form of developmental abnormality. Another modified developmental theory suggests that a less differentiated population of joint-associated mesenchymal cells is responsible for cystic exostoses. The simultaneous development of nonaxial arteries and joints supports the embryological hypothesis that mesenchymal tissue is encased in nearby developing nonaxial vessels. Formation of arterial cystic lesions occurs later in life, when these encapsulated mesenchymal cells begin to secrete mucus material (Figure 1).

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Fig. 1. Theory of cystic epiphyseal formation of joints in the popliteal artery

Clinical manifestations of ACD

The typical symptom in cases of ACD involving the popliteal artery is claudication, which usually occurs briefly or suddenly or after a single episode of exertion. In contrast to claudication due to ischemia of the lower extremities caused by atherosclerosis, this symptom may disappear completely for a period of time, but it will soon reappear or deteriorate rapidly; and it takes longer to recover from the pain of this symptom. It may also manifest as below-knee pain due to pressure. Venous ACD lesions can lead to swelling of the limb (due to partial/complete occlusion). When veins are completely occluded, patients may develop deep vein thrombosis (Figure 2). Clinically, ACD is difficult to diagnose and needs to be differentiated from popliteal vascular entrapment syndrome, popliteal cysts, entrapment aneurysms, and popliteal artery embolism, taking into account the age of the patient, the location of the lesion, and the medical history. Venous ACD, on the other hand, is easily diagnosed as deep vein thrombosis because patients usually present with leg swelling.

Figure 2. Venous epithelial cysts

ACD imaging

1. Ultrasound

CAD is seen in the wall of the diseased vessel as a pike-shaped or elongated cystic echogenic area with homogeneous and consistent signal, traveling along the wall of the vessel and presenting as a simple fluid-like pattern, which is often misdiagnosed as an aneurysm.

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2. IVUS examination

IVUS exams use a higher frequency and are able to differentiate between intima and extima in the circumferential direction when the intima is normal and plaque is low.IVUS can also evaluate and differentiate between intraluminal or vessel wall abnormalities (e.g., thrombosis, PAD, entrapment) as well as extravascular abnormalities (e.g., popliteal ganglion, Baker's cyst, popliteal artery compression syndrome).

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3. CTA examination

CTA检查可3D重建，直观描述显示病变的囊性性质和血管阻塞。

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4. MRI/MRA examination

Non-invasive examination with high soft tissue resolution, ACD shows low signal in T1-weighted image and high signal in T2-weighted image, with clear boundary and close relationship with popliteal artery.MRI can clearly show the lesion in multiple planes, and the relationship between lesion and popliteal artery can be clearly shown in coronal plane, and the "Machete's sign" can be shown very well at the same time. MRA can show the popliteal artery compression, stenosis and occlusion, as well as the formation of collateral circulation around the popliteal artery. Steady-state MRA imaging is more useful in cases of venous CAD associated with limb edema, whereas conventional venography may be unreliable.

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5、Angiography

ACD is characterized on arteriography by the "scimitar sign", a curvilinear, smooth narrowing of the vessel. In circumferential lesions, an "hourglass" shape can also be observed.

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Management of ACD

Surgical treatment of ACD includes percutaneous aspiration, endoluminal intervention, and surgical resection + reconstructive surgery. Percutaneous aspiration may fail due to incomplete aspiration and has a high recurrence rate. In contrast, endoluminal intervention is performed by stenting to overcome cyst compression and maintain arterial lumen. However, this procedure lacks long-term data on transcatheter stenting in young patients. In addition, studies have shown that percutaneous intervention (aspiration or angioplasty) is a risk factor for cyst recurrence (P < 0.001). Currently, open cystectomy + arterial reconstruction is the optimal surgical management of ACD.

Reprinted from Outpatient Magazine

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