Imaging Features and Outcomes in 10 Cases of Idiopathic Azygos Vein Aneurysm

doi:10.1016/j.athoracsur.2013.10.029

The Annals of Thoracic Surgery

Volume 97, Issue 3, March 2014, Pages 873-878

https://doi.org/10.1016/j.athoracsur.2013.10.029 Get rights and content

Background

Idiopathic azygos vein aneurysm (AVA) is rare. This retrospective study evaluated the imaging features and outcomes in 10 cases of idiopathic AVA.

Methods

We retrospectively evaluated 10 patients with surgically proven or typical imaging features of idiopathic AVA encountered in our institution between 1990 and 2012. Chest roentgenography and computed tomography (CT) were performed in all 10 patients, and magnetic resonance imaging (MRI) was performed in 4 of these patients. The clinical features, AVA morphologic characteristics, and outcomes were analyzed.

Results

Chest roentgenograms showed a right paratracheal nodule or mediastinal mass in 7 cases. CT and MRI disclosed 4 thrombosed saccular AVAs (short axis, 3–6 cm; mean, 4.7 cm) and 6 fusiform AVAs (short axis, 2.2–3 cm; mean 2.7 cm). Two large saccular AVAs that presented with chest tightness were resected shortly after diagnosis. One saccular AVA manifested as a pulmonary embolism, whereas the remaining AVA was asymptomatic; they showed 25% to 40% short-axis growth in a 3- to 5-year interval before subsequent AVA resection. Conversely, all 6 fusiform AVAs were asymptomatic and found incidentally, remaining rather stable with less than 8% short-axis growth during 3 to 8 years of follow-up. Compared with fusiform AVAs, saccular AVAs were larger and had a greater frequency of AVA-related symptoms, intralesional thromboses, and greater than 20% short-axis growth during the follow-up period.

Conclusions

Saccular AVAs are larger than fusiform aneurysms, presenting with greater frequency of chest symptoms, intralesional thrombosis, considerable lesion growth, and need for surgical intervention. In contrast, fusiform AVAs are asymptomatic and rather stable in long-term follow-up.

Section snippets

Patients and Methods

Between January 1990 and December 2012, a total of 10 patients with typical imaging features or surgically proven AVA were identified. Their imaging data and medical histories were collected from 3 medical facilities of our institution (1 in southern Taiwan and the other 2 in the north, with a total of 6,000 beds and 15,000 outpatients per day). Our institutional review board approved this study and waived the need for written informed consent from the patients because of the retrospective and

Results

A summary of the 10 cases of AVA is shown in Table 1.

Comment

AVAs are rare and most have been documented as case reports 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14. To our knowledge, the present study is the largest case series of AVAs with long-term follow-up information. A venous aneurysm of the extremity is defined as a persistent isolated focal venous dilatation with a diameter 2-fold that of the normal vein [15]. All our patients harbored focal venous dilatation of the azygos arch with a short-axis length 2.5 times larger than that of the normal

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Citation Excerpt :
An MRI study usually confirms the diagnosis of an AVA (Fig 3) and may also identify blood clots or organized hematoma of various ages within a thrombosed AVA.2 Moreover, it is particularly helpful for its differentiation from tumors and lymphadenopathies.11 However, both imaging tests may provide an image similar to a solid mass, especially when blood flow in the AVA is sluggish15 or the AVA is completely thrombosed.27
Azygos vein aneurysms (AVAs) are uncommon and infrequently diagnosed. When confronted with a patient presenting with an AVA, physicians can rely on only a few case reports after an extensive literature search. To date, no guideline, no rule, and no review on the optimal treatment strategy for these patients exist.
A PubMed and MEDLINE database search for papers and case reports describing AVA was performed. Cases from our own institutions were also reviewed.
The literature search identified 57 published case reports that were reviewed for inclusion. Of those published cases, etiologic factors can be classified into idiopathic, acquired, and traumatic causes. Most AVAs are limited to the azygos arch, a congenital anatomic weak point. Clinical symptoms generally remain nonspecific. Computed or magnetic resonance tomography scans are effective diagnostic tools, although the optimal therapeutic plan remains unclear. Complications include rupture, thromboembolism, mediastinal mass effects, and pulmonary artery hypertension.
Conservative treatment along with oral anticoagulation may be reasonable for some AVAs, but to date, there is no clear guideline or evidence-based threshold for surgical or interventional therapy. In review of the existing data and from our clinical and scientific knowledge, interventional or surgical treatment should strongly be considered in cases with clinical symptoms, pulmonary embolism or pulmonary arterial hypertension, thrombus formation within the AVA in patients with oral anticoagulation or for patients with a contraindication to oral anticoagulants, considerable increase in diameter or compression of adjacent structures, saccular AVA, or an underlying connective tissue disease. The most common procedure is surgical ligation of the AVA, although endovascular occlusion of the aneurysms is becoming more frequent.
Azygos vein aneurysm: An unusual and rare diagnostic
2016, Revue de Pneumologie Clinique
Saccular aneurysm of the azygos vein in a patient with azygos accessory fissure
2015, Radiologia
El aneurisma de la vena ácigos es extremadamente raro, y cuando se localiza en el trayecto de una cisura pulmonar accesoria plantea un reto diagnóstico ya que puede confundirse con tumores originados en la pleura accesoria, como el tumor fibroso pleural solitario o el mesotelioma. El diagnóstico se debe hacer idealmente mediante métodos incruentos como la TC o RM con técnica angiográfica en varias fases, evitando así una intervención quirúrgica innecesaria. El caso presentado es excepcional dado que se trata del primero publicado en el que coinciden ambas situaciones clínicas, una cisura accesoria de la ácigos con un aneurisma venoso en ella.
The saccular aneurysm of the azygos vein is an extremely rare condition, which when located in the path of an accesory pulmonary fissure, becomes a diagnostic challenge because it can be mistaken for tumors arising from the accesory pleura, like solitary fibrous tumor of the pleura or mesothelioma. The diagnosis should ideally be done by non-invasive methods such as CT or MR angiographic technique in multiple phases, thus avoiding unnecessary surgical intervention. This case is exceptional because, to our knowledge, it is the first to report both concomitant clinical situations, an aneurysm of the azygos vein in the pathway of its accessory fissure.
Single-port thoracoscopic removal of an azygos vein aneurysm: a case report and literature review
2023, Journal of Cardiothoracic Surgery

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Original articleAdult cardiacImaging Features and Outcomes in 10 Cases of Idiopathic Azygos Vein Aneurysm

Background

Methods

Results

Conclusions

Section snippets

Patients and Methods

Results

Comment

Ann Thorac Surg

Am J Med

Heart Lung Circ

Clin Radiol

Ann Thorac Surg

Ann Thorac Surg

Arch Bronconeumol

Ann Vasc Surg

Thrombosis of an idiopathic saccular azygos aneurysm

Eur J Cardiothorac Surg

Computed tomography appearance of idiopathic aneurysm of the azygos vein

Can Assoc Radiol J

Original article
Adult cardiac
Imaging Features and Outcomes in 10 Cases of Idiopathic Azygos Vein Aneurysm