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Spontaneous Resolution of Cystic Adventitial Disease: A Word of Caution
Spontaneous Resolution of Cystic Adventitial Disease: A Word of Caution Li Zhang,1 Randy Guzman,1 Iain Kirkpatrick,2 and Julianne Klein,3 Winnipeg, Manitoba, Canada
Spontaneous resolution of cystic adventitial disease has been occasionally reported in the literature. It is unclear, however, whether this resolution is permanent. In this case report, we describe recurrence of a popliteal artery cystic adventitial disease after spontaneous resolution, which was successfully treated with surgery. The underlying mechanism is proposed. Without definitive treatment, the patients with spontaneous resolution of cystic adventitial disease may need long-term follow-up, given the risk of recurrence.
Cystic adventitial disease is a rare cause of popliteal artery occlusion, accounting for approximately 400 cases since 1947.1 The etiology and natural history are debatable. We report an unusual clinical course of a patient with popliteal artery cystic adventitial disease. This case report documents the risk of recurrence in patients after spontaneous resolution.
CASE REPORT A 38-year-old woman was referred to the vascular surgery service at our institution because of 1-year history of worsening claudication in the left leg. The patient had several risk factors for cardiovascular disease, including insulin-dependent type II diabetic mellitus, hypertension, hyperlipidemia, and smoking. She underwent resection of a Baker’s cyst at her left knee 10 years before this presentation (medical records during that period were not retrievable). On physical examination,
1
Department of Surgery, University of Manitoba, Winnipeg, Manitoba, Canada. 2 Department of Radiology, University of Manitoba, Winnipeg, Manitoba, Canada. 3 Department of Pathology, University of Manitoba, Winnipeg, Manitoba, Canada.
Correspondence to: Randy Guzman, MD, FRCSC, FACS, RVT, Department of Surgery, St Boniface General Hospital, 409 Tache Avenue, Winnipeg, Manitoba R2H2A6, Canada; E-mail: [email protected] Ann Vasc Surg 2012; 26: 422.e1e422.e4 DOI: 10.1016/j.avsg.2011.05.044 Ó Annals of Vascular Surgery Inc. Published online: January 27, 2012
she had normal pulses on the right lower limb. Her left femoral pulse was present, but the left popliteal and pedal pulses were absent. Her initial computed tomography (CT) scan showed extrinsic compression of the left popliteal artery by a 2.3 2 cm cystic mass, which caused severe stenosis, indicative of cystic adventitial disease (Fig. 1). While she was waiting for surgery, however, her symptoms subsided and she regained her left foot pulse. A magnetic resonance imaging (MRI) 2 months after her initial scanning failed to identify the cystic lesion (Fig. 2). The surrounding tissue edema and infiltration suggested spontaneous decompression. Surgery was cancelled and outpatient clinic follow-up was scheduled. Four months after cancellation of her surgery, the patient was seen in the clinic with recurrence of her symptoms. A second MRI demonstrated a popliteal artery lesion comparable with her CT findings (Fig. 3). Therefore, she was taken to the operating room approximately 8 months after her initial diagnosis. The diseased segment of left popliteal artery was approached through a posterior Sshaped incision. The cyst was 7 cm long and appeared to be communicating with the knee joint capsule through a duct-like structure. The involved segment of popliteal artery was resected and reconstructed by interposition grafting with a reversed saphenous vein. The duct was oversewn. Pathological examination of the specimen revealed an opened cyst with a smooth inner surface, containing thick cloudy mucus, and attached to the arterial wall. On microscopic examination, the cyst wall arose from the periadventitial tissue of the artery (Fig. 4A) and was comprised of bland fibroconnective tissue with occasional foci of myxoid degeneration (Fig. 4B). It was devoid of any epithelial lining. Hale’s colloidal iron and Alcian blue staining highlighted the presence of acidic mucin within
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Fig. 1. Computed tomographic angiogram of the lower extremities. (A) Coronal view: An interruption in the left popliteal artery (arrow). (B) Transverse view: A
fluid-attenuation cyst (arrow) completely effacing the lumen of the left popliteal artery, with a normal appearance on the right side.
the cyst (Fig. 4C). These findings were consistent with the clinical diagnosis of cystic adventitial disease. Her postoperative recovery was uneventful, and she continued to be asymptomatic at the time of her first annual follow-up. A repeat MRI showed no evidence of recurrence.
the fact that the cysts are very similar to the ganglions morphologically and contain a high concentration of hyaluronic acid. The development theory attributes cystic adventitial disease to implantation of mucin-secreting cells from nearby joints in the adventitia. A direct anatomic communication between the cyst and the knee joint is not a rare finding, which was considered as evidence supporting both the ganglion theory3 and the development theory.2 Of note, the histopathological findings in our case resemble those of a periarticular ganglion cyst, which has been demonstrated in other case reports.4 Spontaneous resolution of the disease has been previously reported by a few groups.5e8 Some authors claimed a ‘‘permanent’’ resolution without
DISCUSSION Cystic adventitial disease typically appears in healthy male patients in their fourth or fifth decade of life. Several hypotheses for its pathogenesis have been proposed.1,2 At present, no strong evidence suggests cystic adventitial disease is caused by trauma or systemic disorders. The ganglion theory postulates adventitial cysts are the result of capsular synovial structures growing and tracking in the adventitia along vascular branches, supported by
Vol. 26, No. 3, April 2012
Case reports 422.e3
completing a lifelong follow-up, which is inappropriate, given the clinical course of our patient. A similar manifestation was previously described as
‘‘intermittent claudication intermittence’’ by Buijsrogge et al.3 The predisposing factor speculated in their reportdrepetitive and excessive stress to the knee jointddid not seem to contribute to the recurrence in this patient. Our imaging studies did illustrate the change in clinical symptoms corresponding well with the change in cystic geometry. The underlying mechanism is likely related to the connection between the cyst and the synovium. Although adventitial cyst fluid is biochemically different from synovial fluid, it is possible that spontaneous cystic decompression occurs when a path forms under high-pressure gradients between the cyst and the synovium. Subsequent recurrence, in this case, could be explained by occlusion of this abnormal connection or by backflow from the synovium into the cyst. The variation of clinical course will certainly influence the management of these patients. Regular outpatient follow-up focusing on history and physical examination with or without imaging modalities is a reasonable approach to the patients with spontaneous resolution of clinical symptoms. As long as the connection between cysts and joint capsule exists, the term ‘‘permanent resolution’’ should not be used in the absence of a lifelong surveillance. Whether cystic adventitial disease is an indication of definitive treatment regardless of symptoms is debatable. Watchful observation is an option for patients with less or no significant symptoms. Cyst aspiration under ultrasound or CT guidance or endovascular repair does not offer reliable
Fig. 3. Preoperative gadolinium-enhanced magnetic resonance angiogram of the left lower extremity. Transverse (A) and sagittal (B) views show the left popliteal artery
(arrowheads) is displaced and stretched posteriorly by a recurrent well-formed cyst (arrows) at a level slightly inferior to its previous location, as demonstrated in Figure 1.
Fig. 2. Preoperative magnetic resonance imaging of the left lower extremity. A transverse T2-weighted image through the same level as shown in Figure 1B shows a normalappearing popliteal artery flow void (arrowhead ). The cystic structure seen on computed tomography is replaced with more diffuse high-signal soft-tissue edema posterior and medial to the popliteal vessels (arrow).
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results. Incision and evacuation of cyst contents is not a definitive therapy either. Cyst resection and reconstruction of the involved artery with autologous vein graft is generally recommended as treatment of choice for symptomatic patients1; however, this approach has its limitations. Longterm patency of vein grafts in a patient with multiple cardiovascular risk factors, as described in this report, is a concern. Nevertheless, at the time of surgery, any anatomic connection between the cyst and the synovium should be identified and closed to prevent recurrence.
CONCLUSION We describe a unique case of cystic adventitial disease. After spontaneous resolution of the disease, long-term follow-up is reasonable, given the risk of recurrence. The communication between adventitia and adjacent joint is likely the cause of recurrence, which should be located and closed at the time of surgery to ensure a durable result.
REFERENCES
Fig. 4. Histochemical examination of the specimen. Hematoxylin and eosin staining: (A) (80); a cystic structure adjacent to the adventitia (arrows) of the arterial wall, (B) (400); focal myxoid degeneration of the cyst wall (arrow). Hale’s colloidal iron staining: (C) (200); acid mucopolysaccharide (arrow) as content of the cyst.
1. Tsilimparis N, Hanack U, Yousefi S, et al. Cystic adventitial disease of the popliteal artery: an argument for the developmental theory. J Vasc Surg 2007;45: 1249e52. 2. Levien JL, Benn CA. Adventitial cystic disease: a unifying hypothesis. J Vasc Surg 1998;28:193e205. 3. Buijsrogge MP, van der Meij S, Korte JH, Fritschy WM. ‘‘Intermittent claudication intermittence’’ as a manifestation of adventitial cystic disease communicating with the knee joint. Ann Vasc Surg 2006;20:687e9. 4. Di Marzo L, Rocca CD, d’Amati G, et al. Cystic adventitial degeneration of the popliteal artery: lectin-histochemical study. Eur J Vasc Surg 1994;8:16e9. 5. Owen ER, Speechly-Dick EM, Kour NW, et al. Cystic adventitial disease of the popliteal arteryda case of spontaneous resolution. Eur J Vasc Surg 1990;4:319e21. 6. Furunaga A, Zempo N, Akiyama N, et al. Cystic disease of right popliteal artery with spontaneous resolution [in Japanese]. Nihon Geka Gakkai Zasshi 1992;93:1501e3. 7. Soury P, Riviere J, Watelet J, et al. Spontaneous regression of a sub-adventitial cyst of the popliteal artery. J Mal Vasc 1995;20:323e5. 8. Pursell R, Torrie EP, Gibson M, Galland RB. Spontaneous and permanent resolution of cystic adventitial disease of the popliteal artery. J R Soc Med 2004;97:77e8.
Spontaneous resolution of cystic adventitial disease has been occasionally reported in the literature. It is unclear, however, whether this resolution is permanent. In this case report, we describe recurrence of a popliteal artery cystic adventitial disease after spontaneous resolution, which was successfully treated with surgery. The underlying mechanism is proposed. Without definitive treatment, the patients with spontaneous resolution of cystic adventitial disease may need long-term follow-up, given the risk of recurrence.
Cystic adventitial disease is a rare cause of popliteal artery occlusion, accounting for approximately 400 cases since 1947.1 The etiology and natural history are debatable. We report an unusual clinical course of a patient with popliteal artery cystic adventitial disease. This case report documents the risk of recurrence in patients after spontaneous resolution.
CASE REPORT A 38-year-old woman was referred to the vascular surgery service at our institution because of 1-year history of worsening claudication in the left leg. The patient had several risk factors for cardiovascular disease, including insulin-dependent type II diabetic mellitus, hypertension, hyperlipidemia, and smoking. She underwent resection of a Baker’s cyst at her left knee 10 years before this presentation (medical records during that period were not retrievable). On physical examination,
1
Department of Surgery, University of Manitoba, Winnipeg, Manitoba, Canada. 2 Department of Radiology, University of Manitoba, Winnipeg, Manitoba, Canada. 3 Department of Pathology, University of Manitoba, Winnipeg, Manitoba, Canada.
Correspondence to: Randy Guzman, MD, FRCSC, FACS, RVT, Department of Surgery, St Boniface General Hospital, 409 Tache Avenue, Winnipeg, Manitoba R2H2A6, Canada; E-mail: [email protected] Ann Vasc Surg 2012; 26: 422.e1e422.e4 DOI: 10.1016/j.avsg.2011.05.044 Ó Annals of Vascular Surgery Inc. Published online: January 27, 2012
she had normal pulses on the right lower limb. Her left femoral pulse was present, but the left popliteal and pedal pulses were absent. Her initial computed tomography (CT) scan showed extrinsic compression of the left popliteal artery by a 2.3 2 cm cystic mass, which caused severe stenosis, indicative of cystic adventitial disease (Fig. 1). While she was waiting for surgery, however, her symptoms subsided and she regained her left foot pulse. A magnetic resonance imaging (MRI) 2 months after her initial scanning failed to identify the cystic lesion (Fig. 2). The surrounding tissue edema and infiltration suggested spontaneous decompression. Surgery was cancelled and outpatient clinic follow-up was scheduled. Four months after cancellation of her surgery, the patient was seen in the clinic with recurrence of her symptoms. A second MRI demonstrated a popliteal artery lesion comparable with her CT findings (Fig. 3). Therefore, she was taken to the operating room approximately 8 months after her initial diagnosis. The diseased segment of left popliteal artery was approached through a posterior Sshaped incision. The cyst was 7 cm long and appeared to be communicating with the knee joint capsule through a duct-like structure. The involved segment of popliteal artery was resected and reconstructed by interposition grafting with a reversed saphenous vein. The duct was oversewn. Pathological examination of the specimen revealed an opened cyst with a smooth inner surface, containing thick cloudy mucus, and attached to the arterial wall. On microscopic examination, the cyst wall arose from the periadventitial tissue of the artery (Fig. 4A) and was comprised of bland fibroconnective tissue with occasional foci of myxoid degeneration (Fig. 4B). It was devoid of any epithelial lining. Hale’s colloidal iron and Alcian blue staining highlighted the presence of acidic mucin within
422.e1
422.e2 Case reports
Annals of Vascular Surgery
Fig. 1. Computed tomographic angiogram of the lower extremities. (A) Coronal view: An interruption in the left popliteal artery (arrow). (B) Transverse view: A
fluid-attenuation cyst (arrow) completely effacing the lumen of the left popliteal artery, with a normal appearance on the right side.
the cyst (Fig. 4C). These findings were consistent with the clinical diagnosis of cystic adventitial disease. Her postoperative recovery was uneventful, and she continued to be asymptomatic at the time of her first annual follow-up. A repeat MRI showed no evidence of recurrence.
the fact that the cysts are very similar to the ganglions morphologically and contain a high concentration of hyaluronic acid. The development theory attributes cystic adventitial disease to implantation of mucin-secreting cells from nearby joints in the adventitia. A direct anatomic communication between the cyst and the knee joint is not a rare finding, which was considered as evidence supporting both the ganglion theory3 and the development theory.2 Of note, the histopathological findings in our case resemble those of a periarticular ganglion cyst, which has been demonstrated in other case reports.4 Spontaneous resolution of the disease has been previously reported by a few groups.5e8 Some authors claimed a ‘‘permanent’’ resolution without
DISCUSSION Cystic adventitial disease typically appears in healthy male patients in their fourth or fifth decade of life. Several hypotheses for its pathogenesis have been proposed.1,2 At present, no strong evidence suggests cystic adventitial disease is caused by trauma or systemic disorders. The ganglion theory postulates adventitial cysts are the result of capsular synovial structures growing and tracking in the adventitia along vascular branches, supported by
Vol. 26, No. 3, April 2012
Case reports 422.e3
completing a lifelong follow-up, which is inappropriate, given the clinical course of our patient. A similar manifestation was previously described as
‘‘intermittent claudication intermittence’’ by Buijsrogge et al.3 The predisposing factor speculated in their reportdrepetitive and excessive stress to the knee jointddid not seem to contribute to the recurrence in this patient. Our imaging studies did illustrate the change in clinical symptoms corresponding well with the change in cystic geometry. The underlying mechanism is likely related to the connection between the cyst and the synovium. Although adventitial cyst fluid is biochemically different from synovial fluid, it is possible that spontaneous cystic decompression occurs when a path forms under high-pressure gradients between the cyst and the synovium. Subsequent recurrence, in this case, could be explained by occlusion of this abnormal connection or by backflow from the synovium into the cyst. The variation of clinical course will certainly influence the management of these patients. Regular outpatient follow-up focusing on history and physical examination with or without imaging modalities is a reasonable approach to the patients with spontaneous resolution of clinical symptoms. As long as the connection between cysts and joint capsule exists, the term ‘‘permanent resolution’’ should not be used in the absence of a lifelong surveillance. Whether cystic adventitial disease is an indication of definitive treatment regardless of symptoms is debatable. Watchful observation is an option for patients with less or no significant symptoms. Cyst aspiration under ultrasound or CT guidance or endovascular repair does not offer reliable
Fig. 3. Preoperative gadolinium-enhanced magnetic resonance angiogram of the left lower extremity. Transverse (A) and sagittal (B) views show the left popliteal artery
(arrowheads) is displaced and stretched posteriorly by a recurrent well-formed cyst (arrows) at a level slightly inferior to its previous location, as demonstrated in Figure 1.
Fig. 2. Preoperative magnetic resonance imaging of the left lower extremity. A transverse T2-weighted image through the same level as shown in Figure 1B shows a normalappearing popliteal artery flow void (arrowhead ). The cystic structure seen on computed tomography is replaced with more diffuse high-signal soft-tissue edema posterior and medial to the popliteal vessels (arrow).
422.e4 Case reports
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results. Incision and evacuation of cyst contents is not a definitive therapy either. Cyst resection and reconstruction of the involved artery with autologous vein graft is generally recommended as treatment of choice for symptomatic patients1; however, this approach has its limitations. Longterm patency of vein grafts in a patient with multiple cardiovascular risk factors, as described in this report, is a concern. Nevertheless, at the time of surgery, any anatomic connection between the cyst and the synovium should be identified and closed to prevent recurrence.
CONCLUSION We describe a unique case of cystic adventitial disease. After spontaneous resolution of the disease, long-term follow-up is reasonable, given the risk of recurrence. The communication between adventitia and adjacent joint is likely the cause of recurrence, which should be located and closed at the time of surgery to ensure a durable result.
REFERENCES
Fig. 4. Histochemical examination of the specimen. Hematoxylin and eosin staining: (A) (80); a cystic structure adjacent to the adventitia (arrows) of the arterial wall, (B) (400); focal myxoid degeneration of the cyst wall (arrow). Hale’s colloidal iron staining: (C) (200); acid mucopolysaccharide (arrow) as content of the cyst.
1. Tsilimparis N, Hanack U, Yousefi S, et al. Cystic adventitial disease of the popliteal artery: an argument for the developmental theory. J Vasc Surg 2007;45: 1249e52. 2. Levien JL, Benn CA. Adventitial cystic disease: a unifying hypothesis. J Vasc Surg 1998;28:193e205. 3. Buijsrogge MP, van der Meij S, Korte JH, Fritschy WM. ‘‘Intermittent claudication intermittence’’ as a manifestation of adventitial cystic disease communicating with the knee joint. Ann Vasc Surg 2006;20:687e9. 4. Di Marzo L, Rocca CD, d’Amati G, et al. Cystic adventitial degeneration of the popliteal artery: lectin-histochemical study. Eur J Vasc Surg 1994;8:16e9. 5. Owen ER, Speechly-Dick EM, Kour NW, et al. Cystic adventitial disease of the popliteal arteryda case of spontaneous resolution. Eur J Vasc Surg 1990;4:319e21. 6. Furunaga A, Zempo N, Akiyama N, et al. Cystic disease of right popliteal artery with spontaneous resolution [in Japanese]. Nihon Geka Gakkai Zasshi 1992;93:1501e3. 7. Soury P, Riviere J, Watelet J, et al. Spontaneous regression of a sub-adventitial cyst of the popliteal artery. J Mal Vasc 1995;20:323e5. 8. Pursell R, Torrie EP, Gibson M, Galland RB. Spontaneous and permanent resolution of cystic adventitial disease of the popliteal artery. J R Soc Med 2004;97:77e8.