CASE 12604 Published on 11.05.2015

Double-fissured aneurysm of the aorta

Section

Cardiovascular

Case Type

Clinical Cases

Authors

Pacciardi F, Pancrazi F, Lorenzi S, Bettini G, Ruschi F, Tonerini M, D'aniello D, Orsitto E.

Pisa, Italy; E
mail:pancrazi_francesca@libero.it

Patient

73 years, female

Categories

Area of Interest Arteries / Aorta, Abdominal wall ; Imaging Technique Catheter arteriography, CT

Procedure Diagnostic procedure ; Special Focus Aneurysms, Acute ;

Show more Show less

Download as PDF Print Show related cases Notify admin

Clinical History

A 73-year-old female patient came to our attention after a few episodes of haematemesis and acute anterior and posterior chest pain. The patient was known to be affected by systemic lupus erythematosus. On first examination, the patient was conscious and in good haemodynamic conditions: she presented only a mild tachycardia, whereas blood pressure was normal.

Imaging Findings

Spiral CT of the thorax and abdomen with contrast demonstrated two aortic aneurysms: one involving the aortic arch and one abdominal aneurysm. No fistulas were demonstrated at CT images.
The aortic arch aneurysm measured 77x78 mm (APxLL diameters) and had an eccentric thrombotic apposition on the left side; fissuring thrombus signs were also detected. The patent lumen measured 53x46x75 mm (APxCCxLL diameters) and was immediately distal to the emergency of the left subclavian artery. The thoraco-abdominal aortic aneurysm began just after the diaphragmatic hiatus to end just proximal to the renal arteries, it measured 62x54x60 mm (APxCCxLL diameters), with a patent lumen of 38x32 mm (APxLL diameters); this aneurysm presented an eccentric thrombotic apposition on the right side and fissuring thrombus signs.
The aortic arch aneurysm was treated endovascularly with percutaneous transfemoral placement of a stent graft.
CT examination one month after the procedure revealed the normal positioning of the graft.

Discussion

Systemic lupus erythematosus (SLE) is an autoimmune disease with multi-organ involvement [1].
SLE is often associated with cardiovascular diseases, such as pancarditis and microvascular damage (small vessel vasculitis and coronary arteritis). Complications in main vessels are rare in these patients and aortic aneurism is an unusual occurrence [2].
The pathogenesis of aortic aneurysms has been attributed mainly to vasculitis and medial degeneration. Histopathological alterations associated with aneurysm formation in SLE patients are separation of elastic fibres, patchy mucoid degeneration, and perivascular lymphoplasmacytic infiltrates with obliterative endarteritis of the vasa vasorum, resulting in microinfarcts and medial necrosis. In these patients TGF-β formation is reduced because of the presence of impaired NK-cells; this entails a lack of extracellular matrix and medial cystic necrosis [1, 3].
In addition, atherosclerosis plays a role in aneurysms development. Antiphospholipid antibody syndrome (APS) may be associated with SLE and induces thrombosis and premature atherosclerosis. Moreover, a prolonged steroid therapy, routinely employed in SLE patients, may accelerate the aneurysm development process by suppressing the production of granulation tissue and chondroitin sulfate, thus leading to connective tissue laxity [2, 4].
Literature considers two different mechanisms of aneurysm development: the non-atherosclerotic thoracic aneurysm formation, associated with cystic medial degeneration, probably due to vasculitis, and the atherosclerotic abdominal aneurism formation, associated with long-term steroid therapy [5].
Different techniques might show vessel dilatations, but CT represents the gold standard to study an aneurysm because of its availability, velocity, high sensibility and specificity. CT is usually performed first without contrast medium, to look for intramural haematoma and calcifications; then contrast medium is employed for the key scan or scans. This technique allows to obtain high resolution images to measure, with extremely high exactitude, anatomy, morphology, size and location of the lesion and also its relationship with the surrounding structures, as well as possible complications, such as dissection and rupture. All this is fundamental to guide the surgeon and help to identify the best treatment, surgical approach (endovascular or transthoracic) and the best type of prosthesis for each patient [6, 7, 8].

Differential Diagnosis List

Fissured thoracic and abdominal aortic aneurysms

Not-fissurated aortic aneurysm

Not-SLE-associated aortic aneurysm

Final Diagnosis

Fissured thoracic and abdominal aortic aneurysms

References

[1] Khan AS, Spiera HJ. (1998) Association of aortic aneurysm in patients with systemic lupus erythematosus: a series of case reports and a review of the literature. J Rheumatol 25(10):2019- 21. (PMID: 9779861)

[2] Takagi H, Mori Y, Iwata H, Kimura M, Itokazu M, Shimokawa K, et al. (2002) Nondissecting aneurysm of the thoracic aorta with arteritis in systemic lupus erythematosus. J Vasc Surg 35(4):801-4. (PMID: 11932683)

[3] de Conti DO, Dias RR, Fiorelli AI, Stolf NA (2011) Ruptured thoracic aortic aneurysm in patient with systemic lupus erythematosus. Rev Bras Cir Cardiovasc 26.1: 128-130 (PMID: 21881722)

[4] Tomoko Miyashita, Yukio Abe, Yasuyuki Kato, Eiichiro Nakagawa, Ryushi Komatsu, Koji Hattori, Toshihiko Shibata, Katsunobu Yoshioka, Takahiko Naruko and Akira Itoh (2010) Aneurysm with Severe Aortic Regurgitation in a Patient with Systemic Lupus Erythematosus. Inter Med 49: 2263-2266 (PMID: 20962447)

[5] Kurata A, Kawakami T, Sato J, Sakamoto A, Muramatsu T, Nakabayashi K (2011) Aortic aneurysms in systemic lupus erythematosus: a meta-analysis of 35 cases in the literature and two different pathogeneses. Cardiovascular Pathology 20 (2011) e1–e7 (PMID: 20133169)

[6] Agarwal P.P., Chughtai A., Matzinger F.R., Kazerooni E.A. (2009) Multidetector CT of Thoracic Aortic Aneurysms. Radiographics 29(2):537-52 (PMID: 19325064)

[7] Rivas L.A.,Fishman J.E., Múnera F., Bajayo D.E (2003) Multislice CT in thoracic trauma. Radiol Clin N Am 41(3):599-616 (PMID: 12797608)

[8] Demetriades D., Velmahos G.C., Scalea T.M., Jurkovich G.J.Karmy-Jones R., Teixeira P.G. et al. (2008) Diagnosis and treatment of blunt thoracic aortic injuries: changing perspectives. J Trauma 64(6):1415-9 (PMID: 18545103)

Case information

URL:	https://eurorad.org/case/12604
DOI:	10.1594/EURORAD/CASE.12604
ISSN:	1563-4086

Figure 1

Axial CT images of the aortic arch aneurysm

The aortic arch aneurysm measures 77x78 mm (APxLL diameters) and has an eccentric thrombotic apposition on the left side. The thrombus is fissured in the antero-lateral zone.

Figure 2

3D Volume rendering of the aorta

3D Volume rendering of the aorta, showing the two aneurysms. The proximal aneurysm involves the aortic arch distally to the origin of the left subclavian artery. The distal aneurysm is located under the diaphragm hiatus.

3D Volume rendering of the aorta left-oblique lateral view, showing the two aneurysms.

3D Volume rendering of the aorta right-oblique lateral view, showing the two aneurysms.

Figure 3

Interventional imaging of the aorta

Interventional imaging of the aorta: the endovascular treatment was performed by percutaneous transfemoral placement of a stent graft.

Figure 4

Post-interventional 3D volume rendering

Post-interventional 3D volume rendering of the aorta showing the graft and its extension over the whole length of the thoracic aorta.

Figure 5

Axial CT imaging of the abdominal aorta

The thoraco-abdominal aortic aneurysm begins just after the diaphragmatic hiatus and ends proximal to the renal arteries. The thrombus, on the right side, is fissured in its lateral part.

Figure 6

Coronal MPR of the aorta

Coronal MPR of the aorta demonstrating thorax and abdomen aneurysms: one involving the aortic arch and one abdominal aneurysm. Both presenting fissuration.

Figure 7

MPR of the aorta - fissuration