Cystic adventitial disease—case series and review of literature

Abstract

Cystic adventitial disease (CAD) is a rare vascular disorder that involves the arteries and rarely the veins, most commonly found in the popliteal artery of male patients. Etiology of CAD is uncertain and currently without a consensus agreement. Clinically, the most common presenting symptom is claudication.Diagnosis requires a strong clinical suspicion in patients with intermittent claudication, but without other risk factors for atherosclerotic disease. Angiography, ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) can all be used for diagnosis. Treatment of CAD can be done via surgical resection or percutaneous intervention such as aspiration. CAD can rarely recur after treatment. Case presentation Case 1A 54-year-old male who presented after an abnormal ultrasound (US) with Doppler exam at an outside institution with a 2 months history of new, progressive right leg claudication. His medical history is significant for hypertension and a maternal history of fatal abdominal aortic aneurysm rupture. The patient states a history of trauma to the right knee of uncertain nature. He denies illicit drug use but confirms a long history of cigarette smoking. Review of systems is otherwise negative. On physical examination, no swelling, varicose veins or stiffness in the right leg were identified. The right pedal pulses were palpable but greatly reduced. The left leg is unremarkable. The initial abnormal Doppler US performed at an outside institution (image not available) reported a 1.5 cm right popliteal artery aneurysm with a possible dissection within this aneurysm and luminal narrowing. An urgent computed tomography angiography (CTA) abdomen and pelvis with runoff was performed which demonstrated a cystic structure intimately associated with the right above-theknee popliteal artery, causing near complete occlusion (Figures 1,2) without evidence of dissection. A presumptive diagnosis of cystic adventitial disease (CAD) is made at this time, and the patient was taken to the operating room for definitive diagnosis and management. A posterior surgical approach was taken and a cyst in the wall of the right popliteal artery was identified. The cyst was entered with the evacuation of viscous, jelly-like amber material. The entire cyst wall was resected. There was strong distal popliteal artery pulse status post cystectomy. The patient had an unremarkable recovery. Follow up with Doppler US performed at 2 and 4 months post-cystectomy showed a normal right popliteal artery, without evidence of cyst recurrence. Case 2A 58-year-old male developed an acute onset of left lower extremity claudication and numbness. There were no resting pain, skin ulcerations or prior history of claudication symptoms. On physical examination, the patient's right lower extremity pulses were within normal limits. The left lower extremity pedal pulses were palpable but reduced, and became nonpalpable with left knee flexion (positive Ishikawa sign). A Doppler US demonstrated an anechoic lesion intimately associat...

Cystic adventitial disease of the popliteal artery presenting with features of entrapment syndrome

“…First described by Atkins and Key in 1947, CAD is a rare disorder, comprising 0.1% of vascular diseases. [2][3][4][5] It is characterized by the presence of an adventitial cyst resulting in lower extremity arterial stenosis and subsequent ischemic symptoms. The cysts are composed of an accumulation of mucinous fluid formed by proteoglycans, mucoproteins, mucopolysaccharides, hyaluronic acid, and hydroxyproline.…”

“…2,5,6,8 Ninety percent of cases of CAD are unilateral, with involvement of the popliteal artery. [2][3][4][5][6] Less commonly, the external iliac, femoral, radial, ulnar, brachial, and axillary arteries are implicated. 2,3,5 Rare reports exist of CAD affecting veins.…”

A case of cystic adventitial disease of the poplitealartery: selection of incision of the cyst wall overvessel replacement

“…Angiography, US, CT, and MRI can all be performed for diagnosis 1,4) . Studies emphasize the importance of MRI and show evidence of the synovial theory through careful and repeated MRI 1,3) .…”

“…Surgical treatment is recommended and performed in most cases. Replacement with a vein graft is the most recommended and preferred method 4,7) . Removal of the cyst and artery should decrease the risk of recurrence in remnant mesenchymal cells.…”

Noninvasive Angiography of Peripheral Arteries

“…20.12), although MRI is the technique of choice in case of suspected CAD due to its ability to definitively identify the mucoid cystic source of the arterial narrowing. Although infrequently encountered, treatment consists of simple percutaneous aspiration of the mucoid material which results in immediate relief of symptoms [27].…”