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Venous Cystic Adventitial Disease Presenting as an Enlarging Groin Mass
Venous Cystic Adventitial Disease Presenting as an Enlarging Groin Mass Mark F. Scott,1 Timothy Gavin,2 and Steven Levin,2 Minneapolis, Minnesota
Venous cystic adventitial disease is an exceedingly rare vascular disorder, with 12 cases reported in the past decade. A 60-year-old woman presented with a painful, palpable groin mass without leg swelling. She was initially thought to have a nonreducible inguinal hernia. A computed tomography scan was obtained that revealed a cystic mass involving the right common femoral vein. Previous imaging revealed that the mass had enlarged over time. In the operating room, the cyst wall was excised without compromising vein integrity. The patient had an uneventful recovery and her pain resolved. We review the presentation, diagnosis, and treatment of this condition. We believe that the rapid evolution of this lesion suggests that an unknown inciting factor triggers its onset and growth.
CASE REPORT A 60-year-old woman with a history of hypertension, tobacco use, and alcoholic pancreatitis presented with a 4- to 5-day history of right groin swelling and discomfort. The physical examination revealed a small, mildly tender mass that was enlarged with valsalva. A computed tomography (CT) scan was obtained that revealed a cystic mass measuring 26.2 20.3 mm. The mass involved a 5-cm segment of the right common femoral vein (CFV) with significant compression of the lumen (Fig. 1A). We later reviewed a CT scan that had been obtained 17 months earlier for evaluation of her pancreatitis. Although it was not noted at the time the scan was performed, we found that the lesion had increased in size from 16.8 mm 14.4 mm (Fig. 1B). On further questioning, the patient reported intermittent right foot swelling that preceded the groin pain and her awareness of the mass. While no edema was present on examination, the patient was referred for femoral duplex ultrasonography to exclude a deep vein thrombosis (DVT). The ultrasound revealed multiple cystic lesions surrounding the right CFV without evidence of 1
Hennepin County Medical Center, Minneapolis, MN.
2
Division of Vascular Surgery, University of Minnesota, Minneapolis, MN. Correspondence to: Steven Levin, MD, Division of Vascular Surgery, University of Minnesota, 420 Delaware Street SE, MMC 195, Minneapolis, MN 55455, USA; E-mail: [email protected] Ann Vasc Surg 2014; 28: 489.e15–489.e18 http://dx.doi.org/10.1016/j.avsg.2013.04.019 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: December 22, 2012; manuscript accepted: April 8, 2013; published online: September 27, 2013.
thrombus (Fig. 2). The size on ultrasound corresponded closely with CT (26.0 mm 21.9 mm 49.0 mm). The waveforms within the right CFV and superficial femoral veins were dampened, consistent with partial obstruction, although no thrombus was present. The patient was diagnosed with venous cystic adventitial disease (CAD) based on the radiographic findings. Because of her symptoms and the degree of venous compression, we recommended surgical excision of the cysts, with interposition grafting if needed. Intraoperatively, the common femoral artery was exposed and retracted laterally; the CFV was noted to be covered with multiple cystic masses with a dense surrounding inflammatory reaction. These extended from just above the inguinal ligament to the bifurcation of the femoral and greater saphenous veins. After obtaining proximal and distal control with vessel loops, the wall of the largest cyst was incised at the level of the bifurcation, releasing a gelatinous clear fluid. The multiple cysts were found to interconnect and were completely removed without compromising the integrity of the vein (Fig. 3). The patient had an uneventful recovery with resolution of her pain, and she was discharged home. Follow-up duplex ultrasonography at 1 month revealed normal flow within the vein. Surgical pathology revealed a benign cyst lined by dense fibrous connective tissue without a definitive lining, consistent with previous reports of CAD (Fig. 4). The patient’s recovery was complicated by a postoperative seroma in her incision. This was percutaneously aspirated to confirm that it indeed contained simple fluid and did not represent a recurrence of her cysts. Two months postaspiration, a small residual fluid collection remained. Duplex ultrasonograpy showed it to lie superficially to the vessels with no evidence of recurrence of her CAD.
489.e15
489.e16 Case reports
Fig. 1. (A) A computed tomography scan revealing a septated cystic structure (arrow) in the right femoral region measuring 26.2 mm 20.3 mm. The femoral vein lumen is reduced to a sliver (arrowhead ). (B) A computed
Annals of Vascular Surgery
tomography scan revealing a septated cystic structure (arrow) of the right femoral vein measuring 16.8 mm 14.4 mm.
Fig. 2. Longitudinal view of the cystic lesion seen surrounding the common femoral vein (dotted line) measuring 26.0 mm 21.9 mm 49.0 mm.
DISCUSSION The physical examination findings and symptoms of CAD mimic other, more common, vascular disorders. In both arterial and venous CAD, the symptoms result from obstruction of blood flow in the affected vessel. Arterial CAD generally presents with claudication symptoms and can be mistaken for atherosclerotic disease.1 In contrast, venous CAD typically presents with unilateral extremity swelling and is frequently misdiagnosed as a DVT.1e6 The correct diagnosis is often made after a lack of improvement with anticoagulants and/or thrombolytic medications. Since 2000, there has been 1 other reported case of venous CAD in which a palpable mass was present; in that case, the patient presented with limb swelling. The patient reported
Fig. 3. The cyst wall has been partially excised, revealing a trabeculated cyst lumen. The intact intima of the femoral vein can be seen at the base of the cysts; the lumen has not been breached.
the presence of an inguinal mass for several months before the onset of her limb swelling.1 In our case, we document a similar mass, and document the progressive enlargement of the lesion on serial imaging. Presumably, if left untreated, the mass would eventually cause sufficient compression, resulting in edema or thrombosis of the vessel.
Vol. 28, No. 2, February 2014
Fig. 4. Histologic examination of the excised vein tissue revealed a fibrous cyst wall (arrow) with spaces containing myxoid material in a collagen matrix (asterisk).
In the setting of unilateral extremity swelling, ultrasound is frequently the initial diagnostic test. On ultrasound, the characteristic finding consists of either 1 or multiple thin-walled, simple fluidfilled cysts of the vessel wall with narrowing of the vessel lumen. Reviewing the published cases since 2000, it is notable that these characteristic findings were not seen in 3 of the 10 patients who underwent ultrasound examinations, all of whom presented with DVTs.3e5 In one report, the authors describe a patient who underwent a series of 3 ultrasound examinations before referral to a vascular surgeon. These studies identified the DVT yet failed to visualize the compressive cystic lesions causing it, which were seen on a fourth ultrasound study in their vascular laboratory.3 We suspect that these missed diagnoses were related to operator experience rather than reflecting an intrinsic weakness of ultrasound for the diagnosis of CAD in the setting of a DVT. CT and magnetic resonance imaging (MRI) scans can help localize the lesions and define the extent of the vascular involvement. On CT scans, CAD can be seen as hypodense fluid-filled cysts arising from the vessel walls; on MRI scans, the cyst fluid appears T2-hyperintense. Recent published reports on venous CAD describe 3 modes of treatment. One option is complete excision of the affected vein and replacement with a vascular graft.2,4,7,8 There have been no reports of recurrence after this treatment, although this introduces the potential for future problems related to graft patience, particularly in cases in which synthetic conduit is used. A second option is excision of the cyst wall, while preserving the underlying endothelium.1,3,5,9e12 This technique is possible because the cystic process does
Case reports 489.e17
not violate the lumen of the vessel, which allows the cysts to be incised and drained, followed by excision of the cyst wall. Since 2000, there has been a single report of recurrence after this treatment,1 with reported follow-up intervals of 6 months to 4 years.1,3,5,10e12 A third option is percutaneous aspiration and sclerosis.6 Percutaneous drainage has not met with success.6,9 In our case, complete excision of the involved vein would have required a retroperitoneal exposure to gain proximal control. Because of her other comorbidities, complete cyst excision through a femoral exposure was chosen to avoid the added risks of a larger operation and the potential complications related to a graft. Venous CAD is exceedingly rare, with only 36 cases reported in the literature, including ours.3,8,12 While arterial CAD is also rare, it is well described in the literature and thought to account for 1:1,200 cases of claudication.9,13,14 Although both diseases predominantly affect patients in their fourth to sixth decades of life,3,9 important differences have been noted between arterial and venous CAD. Arterial CAD affects the popliteal artery in 85% of cases.9 In contrast, venous CAD affects the CFV in 20 of 36 (56%) cases, the external iliac in 8 of 36 (22%) cases, the popliteal vein in 3 of 36 (8%) cases, and other veins in the remainder.3,8,12 Venous CAD has been reported to have a less pronounced male predominance than arterial CAD (1.8:1 in venous CAD [22 men and 12 women in the 34 cases where sex was reported] and 5:1 in arterial CAD).9 The etiology of CAD remains unknown, with 4 principal hypotheses: the trauma theory, the ganglion theory, the systemic disorder theory and the developmental theory. These theories have been extensively reviewed elsewhere, with recent literature lending credence to the developmental theory for arterial CAD.13,14 Historically, venous CAD has been assumed to be the same disease entity as arterial CAD; however, this assumption has recently been called into question on the basis of the above described differences in disease frequency, affected vessels, and sex predominance. In fact, some authors have speculated that venous CAD is a distinct disease process from the arterial form.3,6 While nothing was seen histologically to specifically support any one of the proposed theories, the rapid evolution of this lesion raises suggests that some unknown inciting factor triggers their onset and growth. In conclusion, venous CAD is rare and may mimic other more common pathologies. It appears to follow a progressive course of gradual enlargement over months with eventual venous obstruction and thrombosis. Venous CAD should be
489.e18 Case reports
considered in patients with unprovoked DVT. Duplex ultrasonography is often the initial imaging study but may be less sensitive for CAD in the setting of a DVT. CT and MRI scans can secure the diagnosis and facilitate surgical planning. Complete cyst wall excision appears to be highly effective at treating venous CAD and should be considered before vein excision and interposition grafting.
We thank Rachel Nygaard, PhD, for her assistance with the editing of the manuscript. REFERENCES 1. Maldonado-Fernandez N, Lopez-Espada C, MorenoEscobar J, et al. Recurring adventitial cyst in the left external iliac vein. EJVES Extra 2004;8:10e4. 2. Sugimoto T, Yamamoto K, Tanaka S, et al. Adventitial cystic disease of the femoral vein: report of a case. Surg Today 2004;34:286e8. 3. Jones DW, Rezayat C, Winchester P, et al. Adventitial cystic disease of the femoral vein in a 5-year-old boy mimicking deep venous thrombosis. J Vasc Surg 2012;55:522e4. 4. Gasparis AP. Adventitial cystic disease of the external iliac vein presenting with deep venous thrombosis: a case report. Vasc Endovascular Surg 2004;38:273e6.
Annals of Vascular Surgery
5. Dix FP, McDonald M, Obomighie J, et al. Cystic adventitial disease of the femoral vein presenting as deep vein thrombosis: a case report and review of the literature. J Vasc Surg 2006;44:871e4. 6. Johnson JM, Kiankhooy A, Bertges DJ, et al. Percutaneous image-guided aspiration and sclerosis of adventitial cystic disease of the femoral vein. Cardiovasc Intervent Radiol 2009;32:812e6. 7. Seo JY, Chung DJ, Kim JH. Adventitial cystic disease of the femoral vein: a case report with the CT venography. Korean J Radiol 2009;10:89e92. 8. Jayaraj A, Shalhub S, Deubner H, et al. Cystic adventitial disease of the common femoral vein. Ann Vasc Surg 2011;25:558.e9e11. 9. Paravastu SCV, Regi JM, Turner DR, et al. A contemporary review of cystic adventitial disease. Vasc Endovascular Surg 2012;46:5e14. 10. Sakamoto A, Tanaka K, Matsuda S, et al. Adventitial cystic disease of the popliteal vein: report of a case. Surg Today 2006;36:1098e100. 11. Morizumi S, Suematsu Y, Gon S, et al. Adventitial cystic disease of the femoral vein. Ann Vasc Surg 2010;24: 1135.e5e7. 12. Kwun WH, Suh BY. Adventitial cystic disease of common femoral vein. J Korean Surg Soc 2011;80(suppl 1):S75e9. 13. Levien LJ, Benn CA. Adventitial cystic disease: a unifying hypothesis. J Vasc Surg 1998;28:193e205. 14. 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.
Venous cystic adventitial disease is an exceedingly rare vascular disorder, with 12 cases reported in the past decade. A 60-year-old woman presented with a painful, palpable groin mass without leg swelling. She was initially thought to have a nonreducible inguinal hernia. A computed tomography scan was obtained that revealed a cystic mass involving the right common femoral vein. Previous imaging revealed that the mass had enlarged over time. In the operating room, the cyst wall was excised without compromising vein integrity. The patient had an uneventful recovery and her pain resolved. We review the presentation, diagnosis, and treatment of this condition. We believe that the rapid evolution of this lesion suggests that an unknown inciting factor triggers its onset and growth.
CASE REPORT A 60-year-old woman with a history of hypertension, tobacco use, and alcoholic pancreatitis presented with a 4- to 5-day history of right groin swelling and discomfort. The physical examination revealed a small, mildly tender mass that was enlarged with valsalva. A computed tomography (CT) scan was obtained that revealed a cystic mass measuring 26.2 20.3 mm. The mass involved a 5-cm segment of the right common femoral vein (CFV) with significant compression of the lumen (Fig. 1A). We later reviewed a CT scan that had been obtained 17 months earlier for evaluation of her pancreatitis. Although it was not noted at the time the scan was performed, we found that the lesion had increased in size from 16.8 mm 14.4 mm (Fig. 1B). On further questioning, the patient reported intermittent right foot swelling that preceded the groin pain and her awareness of the mass. While no edema was present on examination, the patient was referred for femoral duplex ultrasonography to exclude a deep vein thrombosis (DVT). The ultrasound revealed multiple cystic lesions surrounding the right CFV without evidence of 1
Hennepin County Medical Center, Minneapolis, MN.
2
Division of Vascular Surgery, University of Minnesota, Minneapolis, MN. Correspondence to: Steven Levin, MD, Division of Vascular Surgery, University of Minnesota, 420 Delaware Street SE, MMC 195, Minneapolis, MN 55455, USA; E-mail: [email protected] Ann Vasc Surg 2014; 28: 489.e15–489.e18 http://dx.doi.org/10.1016/j.avsg.2013.04.019 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: December 22, 2012; manuscript accepted: April 8, 2013; published online: September 27, 2013.
thrombus (Fig. 2). The size on ultrasound corresponded closely with CT (26.0 mm 21.9 mm 49.0 mm). The waveforms within the right CFV and superficial femoral veins were dampened, consistent with partial obstruction, although no thrombus was present. The patient was diagnosed with venous cystic adventitial disease (CAD) based on the radiographic findings. Because of her symptoms and the degree of venous compression, we recommended surgical excision of the cysts, with interposition grafting if needed. Intraoperatively, the common femoral artery was exposed and retracted laterally; the CFV was noted to be covered with multiple cystic masses with a dense surrounding inflammatory reaction. These extended from just above the inguinal ligament to the bifurcation of the femoral and greater saphenous veins. After obtaining proximal and distal control with vessel loops, the wall of the largest cyst was incised at the level of the bifurcation, releasing a gelatinous clear fluid. The multiple cysts were found to interconnect and were completely removed without compromising the integrity of the vein (Fig. 3). The patient had an uneventful recovery with resolution of her pain, and she was discharged home. Follow-up duplex ultrasonography at 1 month revealed normal flow within the vein. Surgical pathology revealed a benign cyst lined by dense fibrous connective tissue without a definitive lining, consistent with previous reports of CAD (Fig. 4). The patient’s recovery was complicated by a postoperative seroma in her incision. This was percutaneously aspirated to confirm that it indeed contained simple fluid and did not represent a recurrence of her cysts. Two months postaspiration, a small residual fluid collection remained. Duplex ultrasonograpy showed it to lie superficially to the vessels with no evidence of recurrence of her CAD.
489.e15
489.e16 Case reports
Fig. 1. (A) A computed tomography scan revealing a septated cystic structure (arrow) in the right femoral region measuring 26.2 mm 20.3 mm. The femoral vein lumen is reduced to a sliver (arrowhead ). (B) A computed
Annals of Vascular Surgery
tomography scan revealing a septated cystic structure (arrow) of the right femoral vein measuring 16.8 mm 14.4 mm.
Fig. 2. Longitudinal view of the cystic lesion seen surrounding the common femoral vein (dotted line) measuring 26.0 mm 21.9 mm 49.0 mm.
DISCUSSION The physical examination findings and symptoms of CAD mimic other, more common, vascular disorders. In both arterial and venous CAD, the symptoms result from obstruction of blood flow in the affected vessel. Arterial CAD generally presents with claudication symptoms and can be mistaken for atherosclerotic disease.1 In contrast, venous CAD typically presents with unilateral extremity swelling and is frequently misdiagnosed as a DVT.1e6 The correct diagnosis is often made after a lack of improvement with anticoagulants and/or thrombolytic medications. Since 2000, there has been 1 other reported case of venous CAD in which a palpable mass was present; in that case, the patient presented with limb swelling. The patient reported
Fig. 3. The cyst wall has been partially excised, revealing a trabeculated cyst lumen. The intact intima of the femoral vein can be seen at the base of the cysts; the lumen has not been breached.
the presence of an inguinal mass for several months before the onset of her limb swelling.1 In our case, we document a similar mass, and document the progressive enlargement of the lesion on serial imaging. Presumably, if left untreated, the mass would eventually cause sufficient compression, resulting in edema or thrombosis of the vessel.
Vol. 28, No. 2, February 2014
Fig. 4. Histologic examination of the excised vein tissue revealed a fibrous cyst wall (arrow) with spaces containing myxoid material in a collagen matrix (asterisk).
In the setting of unilateral extremity swelling, ultrasound is frequently the initial diagnostic test. On ultrasound, the characteristic finding consists of either 1 or multiple thin-walled, simple fluidfilled cysts of the vessel wall with narrowing of the vessel lumen. Reviewing the published cases since 2000, it is notable that these characteristic findings were not seen in 3 of the 10 patients who underwent ultrasound examinations, all of whom presented with DVTs.3e5 In one report, the authors describe a patient who underwent a series of 3 ultrasound examinations before referral to a vascular surgeon. These studies identified the DVT yet failed to visualize the compressive cystic lesions causing it, which were seen on a fourth ultrasound study in their vascular laboratory.3 We suspect that these missed diagnoses were related to operator experience rather than reflecting an intrinsic weakness of ultrasound for the diagnosis of CAD in the setting of a DVT. CT and magnetic resonance imaging (MRI) scans can help localize the lesions and define the extent of the vascular involvement. On CT scans, CAD can be seen as hypodense fluid-filled cysts arising from the vessel walls; on MRI scans, the cyst fluid appears T2-hyperintense. Recent published reports on venous CAD describe 3 modes of treatment. One option is complete excision of the affected vein and replacement with a vascular graft.2,4,7,8 There have been no reports of recurrence after this treatment, although this introduces the potential for future problems related to graft patience, particularly in cases in which synthetic conduit is used. A second option is excision of the cyst wall, while preserving the underlying endothelium.1,3,5,9e12 This technique is possible because the cystic process does
Case reports 489.e17
not violate the lumen of the vessel, which allows the cysts to be incised and drained, followed by excision of the cyst wall. Since 2000, there has been a single report of recurrence after this treatment,1 with reported follow-up intervals of 6 months to 4 years.1,3,5,10e12 A third option is percutaneous aspiration and sclerosis.6 Percutaneous drainage has not met with success.6,9 In our case, complete excision of the involved vein would have required a retroperitoneal exposure to gain proximal control. Because of her other comorbidities, complete cyst excision through a femoral exposure was chosen to avoid the added risks of a larger operation and the potential complications related to a graft. Venous CAD is exceedingly rare, with only 36 cases reported in the literature, including ours.3,8,12 While arterial CAD is also rare, it is well described in the literature and thought to account for 1:1,200 cases of claudication.9,13,14 Although both diseases predominantly affect patients in their fourth to sixth decades of life,3,9 important differences have been noted between arterial and venous CAD. Arterial CAD affects the popliteal artery in 85% of cases.9 In contrast, venous CAD affects the CFV in 20 of 36 (56%) cases, the external iliac in 8 of 36 (22%) cases, the popliteal vein in 3 of 36 (8%) cases, and other veins in the remainder.3,8,12 Venous CAD has been reported to have a less pronounced male predominance than arterial CAD (1.8:1 in venous CAD [22 men and 12 women in the 34 cases where sex was reported] and 5:1 in arterial CAD).9 The etiology of CAD remains unknown, with 4 principal hypotheses: the trauma theory, the ganglion theory, the systemic disorder theory and the developmental theory. These theories have been extensively reviewed elsewhere, with recent literature lending credence to the developmental theory for arterial CAD.13,14 Historically, venous CAD has been assumed to be the same disease entity as arterial CAD; however, this assumption has recently been called into question on the basis of the above described differences in disease frequency, affected vessels, and sex predominance. In fact, some authors have speculated that venous CAD is a distinct disease process from the arterial form.3,6 While nothing was seen histologically to specifically support any one of the proposed theories, the rapid evolution of this lesion raises suggests that some unknown inciting factor triggers their onset and growth. In conclusion, venous CAD is rare and may mimic other more common pathologies. It appears to follow a progressive course of gradual enlargement over months with eventual venous obstruction and thrombosis. Venous CAD should be
489.e18 Case reports
considered in patients with unprovoked DVT. Duplex ultrasonography is often the initial imaging study but may be less sensitive for CAD in the setting of a DVT. CT and MRI scans can secure the diagnosis and facilitate surgical planning. Complete cyst wall excision appears to be highly effective at treating venous CAD and should be considered before vein excision and interposition grafting.
We thank Rachel Nygaard, PhD, for her assistance with the editing of the manuscript. REFERENCES 1. Maldonado-Fernandez N, Lopez-Espada C, MorenoEscobar J, et al. Recurring adventitial cyst in the left external iliac vein. EJVES Extra 2004;8:10e4. 2. Sugimoto T, Yamamoto K, Tanaka S, et al. Adventitial cystic disease of the femoral vein: report of a case. Surg Today 2004;34:286e8. 3. Jones DW, Rezayat C, Winchester P, et al. Adventitial cystic disease of the femoral vein in a 5-year-old boy mimicking deep venous thrombosis. J Vasc Surg 2012;55:522e4. 4. Gasparis AP. Adventitial cystic disease of the external iliac vein presenting with deep venous thrombosis: a case report. Vasc Endovascular Surg 2004;38:273e6.
Annals of Vascular Surgery
5. Dix FP, McDonald M, Obomighie J, et al. Cystic adventitial disease of the femoral vein presenting as deep vein thrombosis: a case report and review of the literature. J Vasc Surg 2006;44:871e4. 6. Johnson JM, Kiankhooy A, Bertges DJ, et al. Percutaneous image-guided aspiration and sclerosis of adventitial cystic disease of the femoral vein. Cardiovasc Intervent Radiol 2009;32:812e6. 7. Seo JY, Chung DJ, Kim JH. Adventitial cystic disease of the femoral vein: a case report with the CT venography. Korean J Radiol 2009;10:89e92. 8. Jayaraj A, Shalhub S, Deubner H, et al. Cystic adventitial disease of the common femoral vein. Ann Vasc Surg 2011;25:558.e9e11. 9. Paravastu SCV, Regi JM, Turner DR, et al. A contemporary review of cystic adventitial disease. Vasc Endovascular Surg 2012;46:5e14. 10. Sakamoto A, Tanaka K, Matsuda S, et al. Adventitial cystic disease of the popliteal vein: report of a case. Surg Today 2006;36:1098e100. 11. Morizumi S, Suematsu Y, Gon S, et al. Adventitial cystic disease of the femoral vein. Ann Vasc Surg 2010;24: 1135.e5e7. 12. Kwun WH, Suh BY. Adventitial cystic disease of common femoral vein. J Korean Surg Soc 2011;80(suppl 1):S75e9. 13. Levien LJ, Benn CA. Adventitial cystic disease: a unifying hypothesis. J Vasc Surg 1998;28:193e205. 14. 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.