Recurrent Cystic Adventitial Disease of the Iliofemoral Artery
Article Outline
We present the case of a 39-year-old man with recurrence of intermittent claudication 4 years after successful Dacron patch repair of the right common femoral artery for cystic adventitial disease. Magnetic resonance angiography results were inconclusive, while conventional angiography demonstrated 90% stenosis of the right common femoral artery. The patient underwent excision and replacement of the affected artery with PTFE and was asymptomatic at 6-month follow-up. In recurrent cystic adventitial disease, excision and replacement of the affected artery with a prosthetic interposition graft provides a successful outcome with minimal chance of recurrence.
Case Report
A 39-year-old man presented with progressive right leg claudication that prevented him from walking 4 km to work. He had cystic adventitial disease of the right common femoral artery and, apart from a history of moderate smoking, he had no other risk factors for atherosclerotic disease. Four years earlier, he underwent successful Dacron patch repair of the right common femoral artery. The cystic degeneration appeared to affect the anterior two thirds of the artery, which was removed and replaced with a large Dacron patch. The macroscopic disease was removed. He remained asymptomatic for 4 years before presenting with a recurrence of his symptoms.
Duplex ultrasound showed that the luminal diameter of the right common femoral artery had narrowed, although there were reasonable velocities passing through it and treadmill Doppler test was positive. Magnetic resonance angiography (Fig. 1) showed signal loss in the distal right common femoral artery and proximal superficial femoral artery, indicating a degree of turbulence, but it was multiplanar angiography (Fig. 2) that identified extensive cystic adventitial disease of the posterior wall causing 90% stenosis of the right common femoral artery and origin of the right superficial femoral artery. The patient's worsening symptoms were considered to be due to either aggressive myointimal hyperplasia or recurrence of the cystic adventitial disease affecting the remaining part of the common femoral artery that was previously unaffected.
Fig. 1
Magnetic resonance angiogram that is inconclusive for stenosis of the right common and superficial femoral arteries.
Fig. 2
Multiplanar computed tomography angiogram demonstrating severe stenosis of the right common femoral artery and origin of the right superficial femoral artery.
On the basis of the patient's symptoms and the results of the investigations, it was decided that it would be appropriate to excise and replace the affected length of common femoral artery. Intraoperatively, a large cystic sac within the adventitia was compressing the right common femoral artery and the origin of the right superficial femoral artery. The termination of the external iliac artery was transected, the profunda femoris was transected just beyond its origin, and the superficial femoral artery was transected 4 cm beyond its origin. An 8-mm PTFE externally supported graft was then anastomosed to the external iliac artery end-to-end and the distal anastomosis was performed end-to-end to the profunda femoris artery. Following this, flow was restored to the profunda femoris. An anastomosis was then performed to the superficial femoral artery end-to-end and the proximal anastomosis was end-to-side onto the PTFE graft. Excellent flows were achieved as a result. Histology revealed that the resection margins were free of cystic adventitial disease. At 6-month follow-up, the patient's symptoms had completely resolved, he had a full complement of peripheral pulses in the right leg, and treadmill Doppler results were normal.
Discussion
Cystic adventitial disease is a rare cause of intermittent claudication with an incidence of 1:1200 and predominantly affecting males (male-to-female ratio 15:1).1 The condition was first described by Atkins and Key2 in 1947 involving the external iliac artery in a 40-year-old man. Since then, approximately 320 cases have been reported.1 The majority of these cases have involved the popliteal artery; only 36 cases report iliofemoral artery involvement.1, 3
The etiology of this condition remains unclear. Several theories have been suggested and were most recently reviewed by Levien and Benn in 1998.4 These include the suggestions that cystic adventitial disease is part of a myxomatous systemic degenerative condition, caused by repeated microtrauma; the ganglion theory that adventitial cysts originate as capsular synovial structures that involve the adjacent blood vessel; and the developmental theory that mucin-secreting cells from an adjacent joint become included in the adventitia during development. Levien and Benn suggested their own unifying hypothesis that cystic adventitial disease is a developmental condition caused by nonsynovial less-differentiated joint-related mesenchymal cell rests in nonaxial blood vessels. Cystic adventitial disease should be suspected in patients with intermittent claudication in the absence of peripheral arterial disease or atherosclerotic risk factors. It may present acutely but does not usually manifest as rest pain. Clinically, there are diminished or absent pulses, and a bruit may be audible over the stenosed artery. The ankle-brachial pressure index may also be reduced.
Investigations include duplex ultrasound, which shows arterial stenosis and a cyst, with no flow, appearing as anechoic or hypoechoic masses in the vessel wall. Angiography demonstrates distinctive stenosis of the arterial lumen, which has an “hourglass” appearance or demonstrates the “scimitar sign” depending on the configuration of the cyst. Magnetic resonance imaging (MRI) is said to be as effective at diagnosing cystic adventitial disease as conventional angiography. Cysts are hyperintense on T2-weighted images and have variable signal intensity on T1-weighted images due to the variable amount of mucoid material within the cysts.5 However, in our patient, MRI (Fig. 1) was inconclusive, whereas multiplanar angiography (Fig. 2) defined the severe stenosis.
Several treatment methods have been used in the management of cystic adventitial disease. In a small number of cases, conservative management has resulted in spontaneous resolution of symptoms.6, 7, 8, 9 However, the majority of patients require intervention. Ultrasound- or computed tomography (CT)-guided aspiration has been used with varying success. Do et al.10 describe successful ultrasound-guided aspiration in seven patients without recurrence at a mean follow-up of 14.8 months, while Sieunarine et al.11 and Colombier et al.12 both report recurrences following CT-guided aspiration requiring subsequent surgical intervention. Our experience suggests that recurrence is likely unless the segment of artery is removed. Balloon angioplasty does not appear to be successful as it does not affect the cystic compression of the artery.13
Surgical intervention appears to provide the most successful long-term outcome, which is of particular relevance as cystic adventitial disease affects relatively young patients. Cyst excision can be performed without arterial reconstruction but is associated with a 10% recurrence rate.14 We have reported recurrence in a patient who originally had cyst excision performed with patch repair. Ohta et al.15 reported a case of cystic adventitial disease recurring within an interposed autogenous saphenous vein graft.
In patients with recurrent cystic adventitial disease, excision and replacement of the affected artery with a prosthetic interposition graft provides a successful outcome with minimum chance of recurrence. However, it is still important to consider the complications of prosthetic graft interposition. In addition, it is vital that all patients receive follow-up.
References
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PII: S0890-5096(09)00313-6
doi:10.1016/j.avsg.2009.05.020
© 2010 Annals of Vascular Surgery Inc. Published by Elsevier Inc All rights reserved.
