By Setacci, F Sirignano, P; de Donato, G; Chisci, E; Palasciano, G; Setacci, C
Adventitlal cystic disease (ACD) is an uncommon condition with only around 300 cases reported in the literature. ACD consists of a collection of gelatinous material within a cyst, that is adjacent or surrounding a vessel. In the last five years three cases of ACD have been observed: the first case was a 48-year-old man, ex nicotine abuser, with a four-month history of progressive claudication; the second case was a 55-year-old man, ex nicotine abuser, with ischemic heart failure and a one-year history of progressive claudication; the third case was a 70-year-old man, with diabetes, dislipidemia and current nicotine abuser with a few-day history of acute pain in the right leg. In two cases the cysts were incised and the contents evacuated. The adventitia was repaired and the wound closed. The first patient is currently asymptomatic after four years from surgery. The second one, at 21 months, follow-up, presented newly severe claudication. Duplex ultrasound scan and computed tomography angiography demonstrated a cranial progression of the lesions. This lesion was treated with bare stent, with complete regression of the symptoms. The third patient was treated with interposition of an autogenous saphenous vein. The patient is asymptomatic at the three- month follow-up. Even if ACD is quite rare, it should be taken in consideration in young patients with severe claudication and no- or poor comorbidities. The best treatment is the incision of the cysts and the advential reconstruction. Short lesions can be treated with endovascular therapy. KEY WORDS: Cysts – Popliteal artery – Intermittent claudication – Reconstructive surgical procedures.
Adventitial cystic disease (ACD) is an uncommon condition with only around 300 cases reported in the literature 1,2 since 1974, when the first case in the external iliac artery was described by Atkins and Key.3 ACD consists of a collection of gelatinous material within a cyst, that is adjacent to or surrounding a vessel. cases of ACD have been reported involving the external iliac, popliteal, femoral, radial, and ulnar arteries 4-8 and the external iliac, femoral and superficial veins.4, 9 The incidence of ACD is 1 out of 1 000 femoral angiograms and 1 out of 1 200 cases of claudication.9. 10 The ratio of males to females is 15:1.11 The onset age varies between the fifth (men) and sixth (women) decade of the life,9 although cases have been reported at other ages.10,12
In the last five years (2002-2007), in the Center of Vascular and Endovascular Surgery at the University of Siena (Siena, Italy) three cases of ACD have been observed.
Clinical series
Case 1.-The first case was that of a 48-year-old man, a former nicotine abuser, without comorhidities, with a four-month history of progressive claudication of the left leg. The Winsor Index was 0.7 in the left leg and >1 in the right. A duplex ultrasound scan (DUS) was performed and revealed a cystic formation compressing the right popliteal artery (Figure 1). Conventional angiography showed the typical curvilinear scimitar sign due to extrinsic compression of the lumen, without further signs of atherosclerosis. The magnetic resonance imaging (MRI) exposed a cystic structure closely related to and surrounding the popliteal artery, indicative of adventitial cystic disease (Figure 2). Surgical treatment involved a posterior approach to the popliteal fossa (Figure 3). The intraoperative findings confirmed the clinical and radiologic diagnosis of cystic adventitial disease of the popliteal artery. Three cysts were incised and the clear, mucinous contents evacuated (Figures 4, 5). The adventitia was repaired and the wound closed. The postoperative Winsor Index was >1 in the treated leg. The patient is currently asymptomatic, with a Winsor Index >1 at four-year follow-up.
Figure 1.-DUS of the involved popliteal artery.
Figure 3.-Surgical incision.
Figure 2.-MRA of the same lesion showed in figure 1.
Figure 4.-Cysts incision and evacuation of the clear, mucinous contents.
Case 2.-The second case was that of a 55-year-old man, a former nicotine abuser, with ischemic heart failure and one-year history of progressive claudication of the right leg. The Winsor Index was 0.9 in the left leg and 0.6 in the right one. The DUS showed a cystic formation compressing the popliteal artery. The MRI exposed a cystic structure surrounding the artery. A preoperative angiography was not performed. In this case, too, the surgical technique involved a posterior approach to the popliteal fossa. The cysts were incised and the contents evacuated, then the adventitia was repaired and the wound closed. The postoperative Winsor Index was =1 in the treated leg. At the 21-month follow-up, the patient presented new severe claudication. DUS and MRI demonstrated a cranial progression of the lesions (length=1 cm), the Winsor Index was 0.3- A periprocedural angiography was then performed. The exam confirmed progression of the disease with a tight stenosis of the AK popliteal artery. The lesion was treated with a bare stent. At three-year follow-up, the patient was completely asymptomatic, with a Winsor Index =1.
Figure 5.-Reconstruction of the adventitia.
Figure 6.-Autogenous saphenous vein graft, after the excision of the lesion.
Case 3.-The third case was a 70-year-old man, with diabetes and dislipidemia, a current nicotine abuser, without cardiac comorbidity and with a few-day history of acute pain on the right leg. The Winsor Index was 0.3 in the right leg and 0.8 on the left. DUS and MRI were performed, which revealed the presence of multi-loculated cysts compressing the popliteal artery and the total occlusion of the arterial segment. It was decided to substitute the diseased arterial segment with a reversed autogenous saphenous vein (Figure 6). The patient was asymptomatic at three-month follow-up.
Discussion
The etiology of ACD is currently unclear. Four theories have been proposed: 1) repetitive local trauma; 2) abnormalities in embryological development; 3) ACD as part of a systemic disease; 4) the synovial hypothesis.6, 11, 13, 14 The synovial (or ganglion) and the abnormal embryological development are currently the most accepted theories.15 Leu etal.4 believe that ACD is caused by a ganglion or bursalike ectopic tissue originating from scleroblastoma. Scleroblastoma is responsible for the formation of joint capsules and bursae. Both ganglia and Baker’s cysts might communicate with joints or with tendon sheaths. Cases of ACD in which the cyst was communicating with the adjacent knee joint and a Baker’s cyst have been described.14-16
The traumatic theory is unlikely because ACD can occur in children and is not frequent in athletes; also a past history of repetitive trauma is uncommon.4, 14 ACD has not shown any consistent association with a systemic disease.14
ACD of the popliteal artery should be clinically suspected in an adult male patient, between the fourth and fifth decades of life, who presents with a sudden onset of claudication in the legs without significant evidence of atherosclerotic disease.6, 11, 13, 15 There is, therefore, no age group that is immune from ACD. Early recognition of the condition is important, because it is often rapidly progressive 11 and because treatment of the condition before it progresses to popliteal occlusion is associated with lower morbidity. However, reaching a diagnosis of ACD is fraught with problems. The symptoms of calf claudication in patients with ACD tend to wax and wane. Severe claudication may suddenly improve spontaneously and completely disappear, only to recur a few months later.17, 18 Direct communication between the cyst and the knee joint is often demonstrated 4 and this could explain the reported waxing and waning of symptoms as well as the reported cases of spontaneous resolution of ACD. 19, 20 Such a history coupled with perfectly normal peripheral pulses and ankle pressure is enough to make a vascular cause for the presenting symptoms unlikely to all except the most attentive of clinicians. Normal pulses, ankle pressure and even ankle pressure after exercise have been reported in association with ACD.11, 21, 22 In patients with a clear history of intermittent claudication and normal peripheral pulses it is worth checking for foot pulses during knee flexion. Ishikawa reported the disappearance of foot pulses on knee flexion or after exercise in patients with ACD, now known as Ishikawa’s sign.23 The symptoms typically worsen with flexion of the knee.15 If direct communication of the adventitial cyst with the joint space or a Baker’s cyst exists, exercise or repetitive trauma can cause rapid growth of the cyst with an accelerated progression of symptoms.4, 13, 16 Spontaneous rupture of the cyst is a rare event and might result in relief of symptoms by decompression of the cyst.19 The recurrence after spontaneous rupture might depend on the extension of the perforation in the wall of the cyst.19 ACD is classically a unilateral process. It is generally not possible to feel the cyst on physical examination, but sometimes ACD can present as soft-tissue- like masses involving the extremities and causing it to be confused with a slowly growing soft tissue tumor.14, 23
When ACD involves the popliteal artery, stenosis is more common than occlusion. Arterial occlusion has been observed in 30% of patients.24 Clinical history and physical examination, plus a DUS examination is usually enough to establish a diagnosis of ACD. US is readily available and inexpensive. A gray-scale sonogram demonstrates hypoechoic cystic lesions with or without multiple septated hypoechoic spaces. DUS clearly shows stenosis with turbulent flow or arterial occlusion with absent color flow. An intra-arterial sonogram shows a large cystic mass arising from the adventitia of the vessel.25, 26 Computed tomography angiography (CTA) and magnetic resonance angiography (MRA) are excellent diagnostic tools that can accurately depict the anatomy of the popliteal fossa, showing the cystic lesions and their relationships to the vessels.9, 10, 13, 27 Additionally, MRA and CTA can noninvasively demonstrate arterial stenosis or occlusion and also determine the inflow and the distal runoff of the arterial segments.11, 27, 28
Digital angiography (DA) frequently shows a characteristic smooth tapering stenosis, referred to as the scimitar sign, without post- stenotic dilation and with no evidence of atherosclerotic disease.6 However, in 30% of cases ACD produces a non-specific complete occlusion that can easily be mistaken for an endoluminal lesion while in other cases angiography may be completely normal. Other disadvantages of arteriography include the high cost, invasiveness, and radiation exposure. While DUS can be helpful in identifying the cystic lesion of ACD around the vessel, lowechoic lesions can sometimes be missed and in other cases ultrasound may suggest a popliteal aneurysm.11 CTA, MRA and intravascular ultrasound have all been recommended in the diagnosis of ACD, and of these MRA imaging is probably being the best modality.29 In view of the difficulties raised by the presentation and investigation of the condition, it is not surprising that many cases are misdiagnosed. Several treatment options have been proposed for ACD. Aspiration of the cystic collection has been performed successfully, although recurrence is frequent. This is not surprising considering the fact that many of these cysts communicate with the adjacent joint and aspiration will clearly not obliterate the communication. Surgical incision and partial or complete excision without opening the artery has been performed with good results when the vessel is patent, excision of the lesion is mandatory when the popliteal segment involved is too long, in order to avoid restenosis. Identifying and ligating any channels with the adjacent joint is claimed to reduce the risk of recurrence following cyst excision. Most cases of popliteal occlusion secondary to ACD have been treated with resection of the affected segment and autogenous vein graft reconstruction.1 The complications associated with this procedure are more common and serious than with simple cyst excision. To avoid resection, thrombolytic therapy followed by non-resectional cystostomy has been used to treat ACD that has progressed to vessel occlusion.30
Conclusions
In summary, the clinician should be alert to the possibility of ACD in patients whose symptoms of claudication wax and wane and in whom claudication seems to develop rapidly. The presence of normal peripheral pulses and normal ankle pressure does not exclude ACD. MRA and DUS may be helpful in the diagnosis of this condition. DA may only demonstrate occlusion of the popliteal artery on knee flexion against resistance. Although the disease is rare, it should be take into consideration in young patients with severe claudication and non or limited co-morbidities. In our experience the best treatment is the incision of the cysts and the advential reconstruction. Short lesions can also be treated with endovascular therapy.
Received on September 18, 2007.
Accepted for publication on 10 March, 2008.
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F. SETACCI, P. SIRIGNANO, G. de DONATO., E. CHISCI, G. PALASCIANO, C. SETACCI
Vascular and Endovascular Unit
Department of Surgery
University of Siena, Italy
Address reprint requests to: C. Setacci, Department of Surgery, Vascular and Endovascular Surgery Unit, University of Siena, viale Bracci, 53100 Siena, Italy. E-mail: [email protected]
Copyright Edizioni Minerva Medica Apr 2008
(c) 2008 Journal of Cardiovascular Surgery. Provided by ProQuest Information and Learning. All rights Reserved.
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