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Adventitial cystic disease of the popliteal artery communicating with the knee joint
Adventitial cystic disease of the popliteal artery communicating with the knee joint A case report Cécile Galle, MD, Jean-Christophe Cavenaile, MD, Anh Dung Hoang, MD, Thuc Le Minh, MD, Marie-Odile Peny, MD, Philippe Braudé, MD, Philippe Dehon, MD, José Ferreira, MD, Serge Motte, MD, PhD, Jean-Claude Wautrecht, MD, and Jean-Pierre Dereume, MD, Brussels, Belgium We describe the case of a patient with adventitial cystic disease of the popliteal artery in which a direct anatomic communication between the cysts and the nearby knee joint was demonstrated by magnetic resonance imaging and confirmed by surgery. This unusual observation could shed some light on the much debated question of the cause, the pathogenesis, and the management of the affection. Moreover, it emphasizes the importance and the role of magnetic resonance imaging in the diagnosis of adventitial cystic disease of the popliteal artery. (J Vasc Surg 1998;28:738-41.)
Adventitial cystic disease of the popliteal artery is an uncommon but well recognized cause of intermittent claudication,1,2 particularly in young patients. The condition is characterized by the development of cystic spaces with mucoid content into the adventitia, which is responsible for localized stenosis or occlusion of the artery lumen. However, the precise cause remains a matter of controversy. Several hypotheses are evoked in the literature, including repeated microtrauma,3 an embryological origin,2,4,5 and a direct communication with the nearby joint.2,4,5 There are some reports that show an anatomic connection between the cyst and the knee joint, which could support the third hypothesis.4-12 We describe the case of a patient with adventitial cystic disease of the popliteal artery in which a communication with the capsule of the knee joint was demonstrated by magnetic resonance imaging (MRI) and confirmed at operation. The importance of this observation in the understanding of the
From the Departments of Vascular Diseases (Drs Galle, Cavenaile, Hoang, Minh, Dehon, Ferreira, Motte, Wautrecht, and Dereume), Pathology (Dr Peny), and Radiology (Dr Braudé), Hôpital Erasme, Université Libre de Bruxelles, Belgium. Reprint requests: C. Galle, MD, Department of Vascular Diseases, Hôpital Erasme, Route de Lennik 808, B-1070 Brussels, Belgium. Copyright © 1998 by The Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter. 0741-5214/98/$5.00 + 0 24/4/92627
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cause, the management of the condition, and the place of MRI in the diagnosis of the disorder is discussed. CASE REPORT A 25-year-old man presented with a 1-month history of abrupt onset of cramp-like pain behind the left knee, followed by progressively worsening intermittent calf claudication. His walking distance was about 20 m. There was no history of trauma. The patient was a smoker (20 cigarettes a day for 8 years) but had no other risk factors for vascular disease. He had no past medical or surgical history. Physical examination disclosed a weak left popliteal pulse (with no modification induced by knee flexion) and an abolition of left distal pulses. There was no evidence of acute ischemia or signs of distal embolization. The left ankle systolic pressure index was 0.45 at rest, falling to 0.26 after treadmill exercise (110 m). Doppler ultrasound study of the left popliteal fossa suggested a short (1-cm) popliteal occlusion associated with a hypoechogenic structure surrounding the lumen. Arteriography via right femoral access (Fig 1) showed a subocclusive stenosis of the left popliteal artery without poststenotic dilation. No signs of atherosclerosis were found. MRI (Fig 2) was performed and revealed a cystic structure closely related to and surrounding the popliteal artery. MRI also clearly showed an anatomic connection of the cyst with the knee joint. Surgical exploration of the popliteal space through a posterior approach revealed a 5-mm-diameter multi-
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loculated cyst and confirmed the communication with the knee joint. Resection of the affected segment was performed, and a venous bypass graft was inserted. Histologic examination of the specimen (Fig 3) showed fibrous-walled cystic spaces developed into the adventitia, presenting an extension to the capsule of the knee joint; both of them containing a mucoid gel. After surgery, the patient had completely recovered normal pulses and ankle pressures. He was completely free of symptoms and was discharged on the 5th day. At 1-year follow up, the patient was fully symptom free with normal peripheral pulses. DISCUSSION Described for the first time in 1954 by Ejrup and Hiertonn,1 adventitial cystic disease of the popliteal artery is a rare disorder with an estimated incidence of 1:1200 in patients with intermittent claudication,1,2 or 1:1000 angiographies.1,2 The condition is characterized by the presence of fluid-filled structures developed within the adventitia, responsible for compression and narrowing of the arterial lumen. Classic clinical presentation is intermittent claudication of sudden onset in young patients. The popliteal pulse is often absent, and the angiographic appearance is of occlusion and displacement or stenosis of the artery, showing a characteristic curvilinear defect called the “scimitar sign.” Diagnosis is established on the basis of clinical history and, classically, on angiographic findings. Nevertheless, more recently, noninvasive imaging techniques like MRI have also been described.8,11,12 The cause and the pathogenesis of the formation of these cystic spaces remain uncertain. Several theories are proposed in the literature. On the one hand, repeated minor trauma on the arterial wall leading to disruption and subsequent cystic degeneration of the adventitia has been mentioned.3 On the other hand, an embryological origin has been described2,4,5 as a developmental inclusion of mucin-secreting cells within the arterial wall. Lastly, a direct communication with the adjacent knee joint, similar to true ganglia, has been suggested.2,4,5 In fact, adventitial cysts have been reported to be biochemically and histologically similar to ganglia.2 Moreover, the description of an anatomic communication between the cyst and the nearby joint could support this pathogenetic hypothesis. Shute and Rothnie4 reported for the first time, in 1973, 2 cases of adventitial cystic disease of the popliteal artery presenting a communication with the knee joint. Later, 8 authors also demonstrated the same rare findings.5-12 Among these, it is
Fig 1. Popliteal angiography shows a smooth narrowing of the popliteal artery at the level of the knee joint (arrow A) and otherwise normal vessel (arrows B and C).
interesting to note that only a few communications were identified before the operation by arthrography5,7 or computed tomography7 and, more recently, by MRI.8 Furthermore, no conclusive evidence has been published on the treatment of this affection. Some case reports described nonresectional procedures, including either surgical incision and aspiration of the cyst with ligation of its expansion and without disruption of arterial continuity4-6,9-12 or computed tomography-guided percutaneous aspiration of the cyst.7 Other reports describe surgical resection of the affected arterial segment and its extension to the joint, in association with venous grafting.4,6,8,9 Both therapeutic options provided satisfactory midterm (6 to 12 months)4-8,11,12 and even long-term (8 to 17 years)6,9,10 results. This suggests that treatment of adventitial cystic disease of the popliteal artery presenting a communication with the nearby knee joint should include a complete excision and evacuation of the cyst with ligation or resection of the joint connection in order to prevent recurrence, which might occur after partial excision.2,4,6,11,12 Graft replacement of the affected artery seems to be required in cases of arterial occlusion or thrombosis or when cyst removal is unlikely to produce favorable results.2,4,6,9,10 Our case report of a direct communication of the
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Fig 2. A, Axial T2 MRI scans of the popliteal fossa show a low “water density” lesion surrounding the artery (arrow A) and expanding to the joint capsule (arrow B). B, Sagittal T2 MRI scans through the same area also show the adventitial cyst (arrow A) and its expansion to the nearby joint (arrow B).
FPO A
B Fig 3. Histologic section. The cystic lesion is developed into the adventitia coat (arrow A) of the arterial wall lined by a fibrous tissue (arrow B) and containing a mucoid fluid (arrow C). The communication with the knee joint is clearly shown (arrow D).
joint space with the area of cysts corroborates the previously reported observations and led us to support the hypothesis of a cause related to the nearby knee joint. It also emphasizes the importance of MRI not only to provide morphological details on
the arterial wall, the intra-arterial content, and the surrounding musculoskeletal anatomy8,11,12 but also to reveal a communication of the cysts with the knee joint before surgery, which may contribute to an improvement in the management of this condition.
JOURNAL OF VASCULAR SURGERY Volume 28, Number 4
REFERENCES 1. Ejrup B, Hiertonn T. Intermittent claudication: three cases treated by free vein graft. Acta Chir Scand 1954;108: 217-30. 2. Lewis GJT, Douglas DM, Reid W, Kennedy WJ. Cystic adventitial disease of the popliteal artery. BMJ 1967;3:411-5. 3. Ishikawa K, Mishima Y, Kobayashi S. Cystic adventitial disease of the popliteal artery. Angiology 1961;12:357-66. 4. Shute K, Rothnie NG. The aetiology of cystic arterial disease. Br J Surg 1973;60:397-400. 5. Hunt BP, Harrington MG, Goode JJ, Galloway JMB. Cystic adventitial disease of the popliteal artery. Br J Surg 1980; 67:811-2. 6. Devereux D, Forrest H, Mc Leod T, Ahweng A. The nonarterial origin of cystic adventitial disease of the popliteal artery in two patients. Surgery 1980;88:723-7. 7. Deutsch AL, Hyde J, Miller SM, Diamond CG, Schanche AF. Cystic adventitial degeneration of the popliteal artery: CT demonstration and direct percutaneous therapy. AJR 1985;145:117-8.
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8. Chiche L, Barranger B, Cordoliani YS, Darrieus H, Guyon P, Vicq P. Two cases of cystic adventitial disease of the popliteal artery: current diagnostic approach. Journal des Maladies Vasculaires 1994;19:57-61. 9. Lassonde J, Laurendeau F. Cystic adventitial disease of the popliteal artery: clinical aspects and etiology. Am Surg 1982; 48:341-3. 10. Mellière D, Ecollan P, Kassab M, Becqemin JP. Adventitial cystic disease of the popliteal artery: treatment by cyst removal. J Vasc Surg 1988;8:638-42. 11. Crolla RMPH, Steyling JF, Hennipman A, Slootweg PJ, Taams A. A case of cystic adventitial disease of the popliteal artery demonstrated by magnetic resonance imaging. J Vasc Surg 1993;18:1052-5. 12. Miller A, Salenius JP, Sacks BA, Gupta SK, Shoukimas GM. Noninvasive vascular imaging in the diagnosis and treatment of adventitial cystic disease of the popliteal artery. J Vasc Surg 1997;26:715-20. Submitted Mar 17, 1998; accepted May 26, 1998.
Adventitial cystic disease of the popliteal artery is an uncommon but well recognized cause of intermittent claudication,1,2 particularly in young patients. The condition is characterized by the development of cystic spaces with mucoid content into the adventitia, which is responsible for localized stenosis or occlusion of the artery lumen. However, the precise cause remains a matter of controversy. Several hypotheses are evoked in the literature, including repeated microtrauma,3 an embryological origin,2,4,5 and a direct communication with the nearby joint.2,4,5 There are some reports that show an anatomic connection between the cyst and the knee joint, which could support the third hypothesis.4-12 We describe the case of a patient with adventitial cystic disease of the popliteal artery in which a communication with the capsule of the knee joint was demonstrated by magnetic resonance imaging (MRI) and confirmed at operation. The importance of this observation in the understanding of the
From the Departments of Vascular Diseases (Drs Galle, Cavenaile, Hoang, Minh, Dehon, Ferreira, Motte, Wautrecht, and Dereume), Pathology (Dr Peny), and Radiology (Dr Braudé), Hôpital Erasme, Université Libre de Bruxelles, Belgium. Reprint requests: C. Galle, MD, Department of Vascular Diseases, Hôpital Erasme, Route de Lennik 808, B-1070 Brussels, Belgium. Copyright © 1998 by The Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter. 0741-5214/98/$5.00 + 0 24/4/92627
738
cause, the management of the condition, and the place of MRI in the diagnosis of the disorder is discussed. CASE REPORT A 25-year-old man presented with a 1-month history of abrupt onset of cramp-like pain behind the left knee, followed by progressively worsening intermittent calf claudication. His walking distance was about 20 m. There was no history of trauma. The patient was a smoker (20 cigarettes a day for 8 years) but had no other risk factors for vascular disease. He had no past medical or surgical history. Physical examination disclosed a weak left popliteal pulse (with no modification induced by knee flexion) and an abolition of left distal pulses. There was no evidence of acute ischemia or signs of distal embolization. The left ankle systolic pressure index was 0.45 at rest, falling to 0.26 after treadmill exercise (110 m). Doppler ultrasound study of the left popliteal fossa suggested a short (1-cm) popliteal occlusion associated with a hypoechogenic structure surrounding the lumen. Arteriography via right femoral access (Fig 1) showed a subocclusive stenosis of the left popliteal artery without poststenotic dilation. No signs of atherosclerosis were found. MRI (Fig 2) was performed and revealed a cystic structure closely related to and surrounding the popliteal artery. MRI also clearly showed an anatomic connection of the cyst with the knee joint. Surgical exploration of the popliteal space through a posterior approach revealed a 5-mm-diameter multi-
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loculated cyst and confirmed the communication with the knee joint. Resection of the affected segment was performed, and a venous bypass graft was inserted. Histologic examination of the specimen (Fig 3) showed fibrous-walled cystic spaces developed into the adventitia, presenting an extension to the capsule of the knee joint; both of them containing a mucoid gel. After surgery, the patient had completely recovered normal pulses and ankle pressures. He was completely free of symptoms and was discharged on the 5th day. At 1-year follow up, the patient was fully symptom free with normal peripheral pulses. DISCUSSION Described for the first time in 1954 by Ejrup and Hiertonn,1 adventitial cystic disease of the popliteal artery is a rare disorder with an estimated incidence of 1:1200 in patients with intermittent claudication,1,2 or 1:1000 angiographies.1,2 The condition is characterized by the presence of fluid-filled structures developed within the adventitia, responsible for compression and narrowing of the arterial lumen. Classic clinical presentation is intermittent claudication of sudden onset in young patients. The popliteal pulse is often absent, and the angiographic appearance is of occlusion and displacement or stenosis of the artery, showing a characteristic curvilinear defect called the “scimitar sign.” Diagnosis is established on the basis of clinical history and, classically, on angiographic findings. Nevertheless, more recently, noninvasive imaging techniques like MRI have also been described.8,11,12 The cause and the pathogenesis of the formation of these cystic spaces remain uncertain. Several theories are proposed in the literature. On the one hand, repeated minor trauma on the arterial wall leading to disruption and subsequent cystic degeneration of the adventitia has been mentioned.3 On the other hand, an embryological origin has been described2,4,5 as a developmental inclusion of mucin-secreting cells within the arterial wall. Lastly, a direct communication with the adjacent knee joint, similar to true ganglia, has been suggested.2,4,5 In fact, adventitial cysts have been reported to be biochemically and histologically similar to ganglia.2 Moreover, the description of an anatomic communication between the cyst and the nearby joint could support this pathogenetic hypothesis. Shute and Rothnie4 reported for the first time, in 1973, 2 cases of adventitial cystic disease of the popliteal artery presenting a communication with the knee joint. Later, 8 authors also demonstrated the same rare findings.5-12 Among these, it is
Fig 1. Popliteal angiography shows a smooth narrowing of the popliteal artery at the level of the knee joint (arrow A) and otherwise normal vessel (arrows B and C).
interesting to note that only a few communications were identified before the operation by arthrography5,7 or computed tomography7 and, more recently, by MRI.8 Furthermore, no conclusive evidence has been published on the treatment of this affection. Some case reports described nonresectional procedures, including either surgical incision and aspiration of the cyst with ligation of its expansion and without disruption of arterial continuity4-6,9-12 or computed tomography-guided percutaneous aspiration of the cyst.7 Other reports describe surgical resection of the affected arterial segment and its extension to the joint, in association with venous grafting.4,6,8,9 Both therapeutic options provided satisfactory midterm (6 to 12 months)4-8,11,12 and even long-term (8 to 17 years)6,9,10 results. This suggests that treatment of adventitial cystic disease of the popliteal artery presenting a communication with the nearby knee joint should include a complete excision and evacuation of the cyst with ligation or resection of the joint connection in order to prevent recurrence, which might occur after partial excision.2,4,6,11,12 Graft replacement of the affected artery seems to be required in cases of arterial occlusion or thrombosis or when cyst removal is unlikely to produce favorable results.2,4,6,9,10 Our case report of a direct communication of the
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Fig 2. A, Axial T2 MRI scans of the popliteal fossa show a low “water density” lesion surrounding the artery (arrow A) and expanding to the joint capsule (arrow B). B, Sagittal T2 MRI scans through the same area also show the adventitial cyst (arrow A) and its expansion to the nearby joint (arrow B).
FPO A
B Fig 3. Histologic section. The cystic lesion is developed into the adventitia coat (arrow A) of the arterial wall lined by a fibrous tissue (arrow B) and containing a mucoid fluid (arrow C). The communication with the knee joint is clearly shown (arrow D).
joint space with the area of cysts corroborates the previously reported observations and led us to support the hypothesis of a cause related to the nearby knee joint. It also emphasizes the importance of MRI not only to provide morphological details on
the arterial wall, the intra-arterial content, and the surrounding musculoskeletal anatomy8,11,12 but also to reveal a communication of the cysts with the knee joint before surgery, which may contribute to an improvement in the management of this condition.
JOURNAL OF VASCULAR SURGERY Volume 28, Number 4
REFERENCES 1. Ejrup B, Hiertonn T. Intermittent claudication: three cases treated by free vein graft. Acta Chir Scand 1954;108: 217-30. 2. Lewis GJT, Douglas DM, Reid W, Kennedy WJ. Cystic adventitial disease of the popliteal artery. BMJ 1967;3:411-5. 3. Ishikawa K, Mishima Y, Kobayashi S. Cystic adventitial disease of the popliteal artery. Angiology 1961;12:357-66. 4. Shute K, Rothnie NG. The aetiology of cystic arterial disease. Br J Surg 1973;60:397-400. 5. Hunt BP, Harrington MG, Goode JJ, Galloway JMB. Cystic adventitial disease of the popliteal artery. Br J Surg 1980; 67:811-2. 6. Devereux D, Forrest H, Mc Leod T, Ahweng A. The nonarterial origin of cystic adventitial disease of the popliteal artery in two patients. Surgery 1980;88:723-7. 7. Deutsch AL, Hyde J, Miller SM, Diamond CG, Schanche AF. Cystic adventitial degeneration of the popliteal artery: CT demonstration and direct percutaneous therapy. AJR 1985;145:117-8.
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8. Chiche L, Barranger B, Cordoliani YS, Darrieus H, Guyon P, Vicq P. Two cases of cystic adventitial disease of the popliteal artery: current diagnostic approach. Journal des Maladies Vasculaires 1994;19:57-61. 9. Lassonde J, Laurendeau F. Cystic adventitial disease of the popliteal artery: clinical aspects and etiology. Am Surg 1982; 48:341-3. 10. Mellière D, Ecollan P, Kassab M, Becqemin JP. Adventitial cystic disease of the popliteal artery: treatment by cyst removal. J Vasc Surg 1988;8:638-42. 11. Crolla RMPH, Steyling JF, Hennipman A, Slootweg PJ, Taams A. A case of cystic adventitial disease of the popliteal artery demonstrated by magnetic resonance imaging. J Vasc Surg 1993;18:1052-5. 12. Miller A, Salenius JP, Sacks BA, Gupta SK, Shoukimas GM. Noninvasive vascular imaging in the diagnosis and treatment of adventitial cystic disease of the popliteal artery. J Vasc Surg 1997;26:715-20. Submitted Mar 17, 1998; accepted May 26, 1998.