Cystic adventitial disease of the common femoral vein: A case report

Introduction

Cystic adventitial disease (CAD) can affect both arteries and veins but is more common in arteries. Since the first description of venous CAD in tributaries of the saphenous vein in 1972, there are only 43 reported cases of venous CAD worldwide,^1–36 make it an extremely rare disease. Patients with venous CAD present with limb swelling or very rarely deep vein thrombosis (DVT). ³⁷ We found a total ingrown venous CAD in the left common femoral vein (CFV) by ultrasound and Computed Tomography Venography (CTV) in a patient presented with swelling on his left leg. The surgical resection has been completed successfully by us. A brief report of this case was summarized as follows.

Case report

A 54-year-old male presented with 1-year history of left leg swelling and advancing swelling pain in recent 5 days. The patient was no history of trauma and no risk factors of DVT such as smoking, diabetes, hyperlipidaemia, and hypercoagulability. At clinical examination, the circumference of the left leg was 4.8 cm longer than that of the right side, but pigmentation, deposition dermatitis, and skin ulcers were not found. The popliteal and pedal pulses of the left leg were normal. Angiological examination of the right leg was normal. A primary diagnosis of DVT was made after admitting. Doppler ultrasound examination detected a cystic mass about 1.9 cm × 1.4 cm with clear boundaries originated from the left CFV (Figure 1). To obtain more detailed information, a computed tomography (CT) scan was further performed to exclude other possible reasons for vessel obstruction such as hemangioma or exogenous pressure. CT images detected an isolated and endogenously grown cystic mass at the initial segment of left CFV, and that the circulation of left CFV being partially obstructed (Figure 2). Also, D-dimer test of the patient was normal. Therefore, the clinical diagnosis of CAD was proposed. After 5-days treatment of anticoagulation, a surgical excision was performed.

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Figure 1.

Doppler ultrasound photograph showed a roughly 1.9 × 1.4 cm cystic dark area (seen the red arrow) arising on the posterior wall of the common femoral vein.

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Figure 2.

An axial computed tomography (CT) image at the level of the common femoral vein showing the endogenous l compression “Coma signs.”

Under total intravenous anesthesia, patient’s left CFV was exposed adequately and was explored. Then the whole left CFV was dissected and controlled. A bolus dose of 2500 U of heparin was administered before clamping the vessel, and a longitudinal venotomy was made in the CFV. The CFV was found no thrombus. The lumen was almost entirely compressed by intramural cystic structure in the posterior wall of the vein (Figure 3). A venotomy was made in the posterior wall to reveal thick gelatinous mucoid material lying within a two endogenous cystic cavities (1.4 cm × 1.2 cm and 0.6 cm × 0.4 cm) formed by the vein wall. The cyst was excised, and the posterior layers of the vein were closed primarily using a continuous 6–0 polypropylene suture. Flow in the CFV was resumed after closure of the anterior venotomy using the same suture material. Postoperatively, the patient received anticoagulation with warfarin and the limb swelling disappeared 3-week late. Histological examinations on the dissected cyst clearly showed a cyst wall lined by dense fibrous connective tissues, consistent with a diagnosis of benign cyst (Figure 4). At the 1-year follow-up, the swelling in the leg had resolved, and the CFV was patent on color duplex imaging, with no mass effect (Figure 5).

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Figure 3.

Intraoperative photograph showing the cyst lying in the posterior wall of the common femoral vein and attaching to the intimal (seen the arrow and circle), and the wall was thin with light yellow gelatinous mucoid fluid in it.

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Figure 4.

Postoperatively, the light microscopy photograph revealed the wall of the fibrous tissue indicating that the pathological diagnosis was consistent with cystic disease.

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Figure 5.

Postoperatively, a reexamination by Doppler ultrasound showed the wall of the femoral vein was smooth, and there was no obvious clumps echo display inside.

Discussion

As an extremely rare venous disease, venous CAD is caused by outgrown cysts in venous adventitia, and most reported cases have involved the iliofemoral veins. ³⁸ Clinical manifestations of venous CAD are severe limb swelling due to the blood flow obstruction and pain due to the stimulation of vessel adventitial nerves.^32,34 Venous CAD is frequently misdiagnosed because the patients primarily present with DVT.^14,20,29,33 This is an unusual case because no DVT occurred so that we can identify the cyst solely by imaging methods. The operative exploration also confirmed that this was a case of a completely ingrown venous CAD.

The etiology of CAD remains unclear, although various models including the trauma theory, the ganglion theory, the systemic disorder theory, and the developmental theory have been postulated.^23,26 The gelatinous mutinous substance in the cyst removed from our surgery and the postoperative pathological examination results are both consistent with manifestations of CAD as previously reported. Therefore, it is possible that the gelatinous mutinous fluid secreted from vessel adventitial cells formed adventitial cyst.^39,40

For a timely and accurate diagnosis, duplex ultrasound may delineate the cyst in the vessel wall, which appears as a hypodense fluid-filled cyst rather than a hyperdense thrombus with blood flow obstruction as those in DVT.^9,23,38 CT and magnetic resonance imaging (MRI) scans can help to localize the lesions precisely and identify the intravascular contents. On CT scans, CAD displayed as hypodense fluid-filled cysts arising from the vessel walls; while on MRI images, the cyst fluid appears as T2-hyperintense signal. ⁴¹ Also, D-dimer test can help to exclude diagnosis of acute DVT.

No optimal treatment plan can be applied at the current stage. The surgical removal of the intracavity cyst followed by venous angioplasty, theoretically, should relieve the blood vessel from obstruction. An alternative surgical choice is the implantation of intravenous stent, which can eliminate the blood flow obstruction but leaving the cyst intact. We believe that the surgical resection of the cyst coupled with angioplasty may be a better choice.^{17,22,23,27,29,30} This receives partial support from our reported case in which the patient had a satisfactory prognosis. Collectively, the optimal surgical plan and treatment have not been fully illustrated against CAD, whose etiology and mechanism need to be further investigated.

Conclusion

As a rare disease with unknown etiology, venous CAD appears to follow a progressive course of gradual enlargement over months and eventual venous obstruction and thrombosis. So far, no treatment plan has been recognized, mainly due to the limited number of cases. We recommend the complete resection of the cyst as an effective method, which should be applied as the primary choice under feasible circumstances.