Eur J Vasc Endovasc Surg 15, 456458 (1998)

C A S E R E P O R T

Cystic Adventitial Disease

P. Madhavan 1, 1". Boyle 1, J. Coyle 1, M. Cox 1, N. McEniff 2, M. Molloy 2 and T. M. Feeley 1

1Department of Surgery, Meath/Adelaide Hospitals, Dublin and 2Department of Radiology, St. James' Hospital, Dublin, Ireland

Introduction

Cystic adventitial disease (CAD) of the popliteal artery is a recognised cause of arterial insufficiency in a young individual.

Case Report

A 23-year-old female presented with a five-week his- tory of left calf claudication at 100 yards. She had palpable pedal pulses. Her resting ABI was 1.23 on the right and 0.88 on the left, post-exercise were 1.32 (R) and 0.58 (L). An angiogram showed a stenosis in her left popliteal artery with an otherwise normal arterial system (Fig. 1). A magnetic resonance an- giogram was carried out and revealed an intramural swelling in the left popliteal artery extending for 3-4 cm (Figs 2, 3) which was compressing the lumen.

The MR suggested that the content of the swelling was fluid. There was no evidence of popliteal artery entrapment. The artery was explored through an S popliteal incision. Incision in the adventitial layer caused clear viscid material typical of ganglion content to exude from the vessel wall. A thin strip of cyst wall was excised but the intima was not incised. Distal arterial pressures re turned to normal. A few hours following surgery there was haemorrhage from the wound and the patient was re turned to theatre. She was found to have bleeding from the affected area with a number of perforations at the site of previous cyst wall excision. The artery was opened and half the circumference of the affected segment was excised. Repair was with a vein patch using short saphenous vein.

Postoperatively there were no problems and she made an uneventful recovery. On follow-up she was asymptomatic and post-exercise ABI's were normal.

Fig. 1. Selective left leg digital subtraction angiogram shows a smooth intraluminal filling defect with near occlusion of the upper popliteal artery with collateralisation and normal distal vessels.

Fig. 2. Axial gradient echo magnetic resonance images in the region of interest shows minimal flow in the popliteal artery with normal flow in the adjacent popliteal vein.

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Cystic Adventitial Disease 457

Fig. 3. Coronal T2 weighted images show high signal in the left popliteal artery, suggesting the presence of fluid.

Light microscopy of the excised strip of vessel wall was unhelpful and showed essentially normal ad- ventitia with occasional vas vasorum.

Discussion

CAD is a rare cause of intermittent claudication with an incidence estimated to be i in 1200 patients presenting with claudication and 1 in 1000 of those undergoing angiography with the sex incidence of eight males to one female--this is similar to the incidence of occlusive atherosclerosis. ~ Atkins and Key in 1947 reported a case of a myxomatous tumour arising in the external iliac artery. 2 CAD has been reported in other sites. Ishikawa in 1987 reviewed the world literature and compiled 234 cases of CAD from over 200 publications. He divided them into two groups--CAD arising in the popliteal artery (195 cases) and CAD arising in other sites (39 cases). 3

The clinical features of CAD are identical to those of arterial stenosis/occlusion from any cause. Suspicion is raised by presentation in a young person. Symptoms may be similar to those caused by entrapment of the popliteal artery. Angiography is still the best method of diagnosis. The "scimitar" sign as described by Harris and Jepson in 19654 or an hour glass deformity is said to be highly suggestive of CAD. Suy et al. 5 described the following characteristic angiographic findings: scimitar, hourglass, flute-embouchure, M-shaped and triangular filling defect. Ultrasound, 6 computed tomo- graphy 7 and more recently MRP have been used in diagnosis.

There has been much speculation as to the exact aetiology of this rare condition. These have been extensively reviewed by Ishikawa. 3 Suggestions

included repetitive microtrauma causing cystic de- generation of the adventitia, developmental anomaly where mucin secreting cells were sequestered in the adventitia, and a degenerative condition of the ad- ventitia. A recent electron microscopy s tudy by Di Marzo Luca 9 showed that a vascular origin of the cyst is unlikely and that the lectin binding pattern of CAD is similar to that of normal synovium. This strongly suggests that the cyst content is derived from synovial fluid producing cells but the origin of these cells in the adventitial wall is unclear. There is rarely any communication between the artery and tendons or joint space. 1°'11

Almost all patients need intervention for relief of symptoms, al though there have been reports of spon- taneous resolution. .2 As the lesion is easily crossed with a guide-wire, angioplasty ~3 is an attractive option, though recurrence is likely and this cannot be con- sidered a definitive treatment. There is also a risk of distal embolisation of the cyst contents. Ultrasound or CT guided aspiration can be used, though aspirating the thick gelatinous cystic contents is likely to be difficult and incomplete with recurrence highly likely. 7

There have been a number of case reports of suc- cessful incision and part excision of the cyst wall which prompted our initial procedure. ~'.4 In our patient, fol- lowing excision there was no immediate dilatation of the artery; however, the wall ruptured a few hours after surgery. It is evident that the cyst wall does contain the adventitial layer of the artery which gives an artery its strength and removal of part or all of this inevitably results in a weaknes which is prone to rupture. We would therefore suggest that incision with or without part excision of the cyst wall is not the ideal treatment, and surgical replacement of the dis- eased vessel is probably best. Many feel that complete replacement of the diseased artery wi th a vein graft is the ideal treatment. However, we feel that retention of half the circumference of the native artery in its normal alignment wi th its normal intima, particularly crossing a joint line, may be advantageous in these very young patients.

References

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Accepted 25 August 1997

Eur J Vasc Endovasc Surg Vol 15, May 1998