Adventitial Cystic Disease of the Femoral Vein
Article Outline
Adventitial cystic disease of the blood vessel wall is a rare disease. It occurs most commonly in arteries and very rarely in veins (Levien and Benn, J. Vasc. Surg. 1998;28:193–205). We report the case of a 28-year-old man with severe adventitial cystic disease of the femoral vein who underwent a resection of the cystic wall.
Case Report
A 28-year-old man presented with a 1-week history of spontaneous swelling of the left lower extremity. He had no preceding history of trauma or comorbidity. On the basis of only physical examination, it seemed that he suffered from deep vein thrombosis. So we commenced treatment with antibiotics and anticoagulant therapy to prevent cellulitis and deep vein thrombosis of the left extremity.
An ultrasonogram showed a hypoechoic mass in the left groin (Fig. 1). The mass caused a narrowing of the femoral vein. For further evaluation, we performed computed tomography (CT) with the patient in a supine position (Fig. 2). The contrast-enhanced CT showed a cystic mass, with a diameter of 2 cm in the left inguinal area. No enlarged lymph nodes or abnormal muscular structures were found around the vein.
Fig. 1
Ultrasonogram showing a hypoechoic mass (
) in the left groin. The mass causes a narrowing of the femoral vein lumen. FA, femoral artery; FV, femoral vein.
Fig. 2
Contrast-enhanced computed tomography shows a cystic mass (arrow) measuring 2 cm in diameter and located in the left inguinal area. The mass has caused a narrowing of the femoral vein lumen. A Axial image, B Coronal image.
The patient was diagnosed with extrinsic compression of the left femoral vein because of a cystic mass. He underwent surgery to remove the cyst and decompress the venous system. The common femoral artery was exposed and retracted laterally to facilitate the dissection of the common femoral vein (CFV). The CFV, profunda femoral vein, and superficial veins were dissected. A 2.0 × 1.8 cm cystic mass extending from the lateral to the posterior surface of the left CFV and severely narrowing its lumen was observed (Fig. 3). The division of the vein from the cyst was very difficult. A venotomy was made in the anterior wall (2.0 × 1.0 cm wide) and a gelatinous mucoid substance was removed from the cystic mass. The cystic wall was then resected for pathological examination and opened. The lumen of the vein appeared to be of a normal size. No connections with neighboring structures or joints were seen.
Fig. 3
Intraoperative view of the cyst (arrow). An intravenous cast containing mucinous material was found in the left femoral vein. FA, femoral artery; FV, femoral vein.
Pathological examination of the cystic wall revealed a fibrous layer and the absence of an endothelial lining beneath the adventitia. Also no mucoid degenerated cells were observed.
The postoperative course of the patient was uneventful. When last seen 6 months after his operation, he was well and asymptomatic, and an ultrasonogram showed a patent left femoral vein without adventitial cystic disease (ACD).
Discussion
ACD of the vein is a rare condition in which a mucin-containing cyst forms in the wall of the vessels. To our knowledge, less than 30 cases of ACD in veins have been reported in worldwide medical literature.1, 2, 3, 4, 5
ACD of the veins has been documented in 25 patients with a mean age of 64 years (range, 23-73 years) and has occurred with an equal frequency in both genders: 15 men and 10 women. Most cysts appeared in the lower limbs, predominantly in the CFV. The most characteristic clinical presentation was edema (n = 19) in the lower limb or a groin mass (n = 5). ACD of the femoral vein is very difficult to diagnose because of the similarity of its symptoms with deep vein thrombosis.
The exact etiology of ACD remains unclear, but several theories have been proposed: (1) repeated traumatic theory (the adventitia undergoes cystic degeneration as a result of stretching and distortion near joints), (2) ectopic aganglionosis theory (synovial cells implant into adventitia near joints), (3) systemic disorder theory (degeneration of the adventitia as a result of connective tissue diseases), and (4) developmental theory (mesenchymal cells from nearby joints implant into the adventitia of the vessel during embryological development).6, 7 The cause of ACD in the femoral vein in the present patient is difficult to explain because he did not have a history of recurrent trauma, and no pedicle connection with a neighboring joint was observed. Also, a histological analysis showed the absence of mucoid cells in the lining of the adventitial cyst.
Treatment is based on two approaches: percutaneous aspiration and surgical resection. The use of needle aspiration of the fluid, guided by ultrasound or CT, has been tried successfully in some cases, although the fluid has a tendency to reaccumulate because the mucin-secreting mesenchymal cells are left in situ. Surgical resections of the femoral vein include transluminal fenestration, transadventitial evacuation, and segmental resection. In our patient, the femoral vein was patent, so the ACD wall was segmentally resected. Further follow-up of the patient will be necessary to ensure a successful outcome.
References
- . Adventitial cystic disease: a unifying hypothesis. J Vasc Surg. 1998;28:193–205
- Adventitial cystic disease of the femoral vein: report of a case. Surg Today. 2004;34:286–288
- Cystic adventitial disease of the femoral vein presenting as deep vein thrombosis: a case report and review of the literature. J Vasc Surg. 2006;44:871–874
- . Adventitial cystic disease of the femoral vein: a case report with the CT venography. Korean J Radiol. 2009;10:89–92
- Cystic adventitial degeneration of the femoral vein: report of a case. Jpn J Phlebol. 2007;18:179–183
- . Summary of cases of adventitial cystic disease of the popliteal artery. Ann Surg. 1979;189:165–175
- . Subadventitial hematoma of the popliteal artery. J Cardiovasc Surg. 1973;14:447–451
PII: S0890-5096(10)00151-2
doi:10.1016/j.avsg.2010.03.007
© 2010 Annals of Vascular Surgery Inc. Published by Elsevier Inc All rights reserved.
