Popliteal Cystic Adventitial Disease Causing Intermittent Claudication in a Young Athlete: A Case Report

Paritosh Sharma, MRCS; Nat Padhiar, MSc, PhD, FCPodS; Constantinos Kyriakides, MD, FRCS

Disclosures

South Med J. 2008;101(11):1154-1156. 

In This Article

Discussion

CAD is a rare condition, characterized by the presence of unilocular or multilocular mucinous or gelatinous cysts in the adventitial layer of the vessel wall. It has an incidence of 1:1200 among patients with intermittent claudication (1:1000 peripheral angiograms) and typically affects males between the ages of 20 and 50 years. The first case of CAD was reported in the iliac artery by Atkins and Key[1] in 1947, and approximately 350 cases have been reported to date. In 85% of cases, the popliteal artery is affected. Other affected sites include the femoral, external iliac, radial and ulnar arteries and the femoral, external iliac and superficial veins.[2]

The exact etiology of CAD remains unclear, but several theories have been put forward to explain its occurrence.[3] These theories include the developmental theory, which postulates that mesenchymal cells become incorporated into the adventitial layer, giving rise to cysts. The synovial theory suggests persistent communication between the synovial space of an adjacent joint and the adventitial layer of the vessel wall. It has also been suggested that repeated trauma may bring about cystic changes in the vessel wall, which may compress the lumen. The systemic disorder theory explains CAD as part of a systemic disease, but this theory does not have many proponents.

Normal popliteal and foot pulses will usually be palpable in patients with popliteal CAD, although they may diminish on exercise or upon knee flexion (Ishikawa sign). On arteriography, a smooth curvilinear (scimitar) sign, an hourglass stenosis, or complete occlusion of the artery is seen with an otherwise normal arterial circulation. The diagnosis in most cases is confirmed by a duplex ultrasound, where grayscale images reveal cystic compression, and a magnetic resonance angiography that delineates vessel wall anatomy.

Classically, CAD has been managed surgically, either by replacing the affected segment by an interposition graft or by evacuating the cyst contents while preserving the medial and intimal layers. Interposition grafting is usually reserved for cases with complete occlusion of the popliteal artery, where the native vessel cannot be repaired. An initial 94% success rate has been associated with both cyst excision and interposition grafting.[4] Some studies have suggested 10% medium- to long-term recurrence rates with cyst excision.[5,6] Angioplasty has not been recommended in these patients due to the risk of distal embolization. Ultrasound or computed tomography-guided percutaneous aspiration of cyst contents has also been proposed, but arguably the success of these techniques depends on the consistency of the contents of the cyst and its communication with an adjacent joint. A few cases of spontaneous resolution of the cyst have also been reported,[5] though this is an unpredictable outcome.

Popliteal artery entrapment syndrome (PAES) has been thought to be responsible for up to 60% cases of calf claudication in young athletes.[7] PAES results from an aberrant relationship between the popliteal artery and the gastrocnemius muscle. Four types have been described, depending on the anatomic positions of the medial head of the gastrocnemius and the popliteal artery. A functional variant has also been described with normal anatomy, but with all the signs and symptoms of popliteal artery entrapment.

PAES and popliteal cystic adventitial disease are remarkably similar in terms of presentation and affected population. They are rare conditions and should be considered in young patients with intermittent claudication, particularly when other cardiovascular risk factors are absent.

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