Aberrant right subclavian artery (arteria lusoria): a case of asymptomatic rupture.
Aberrant right subclavian artery (ARSA) is also called as a lusorian artery that is extremely rare.  However, it is amongst the most common aortic arch anatomic anomalies.  The incidence of ARSA in the general population is in the range between 0.1-2.3%. [1, 2] It emerges as fourth branch of the proximal descending aorta.  Clinically, many patients with ARSA are asymptomatic. However, dysphagia, shortness of breath, and chest pain may be the first symptoms because of compression. ARSA are tend to rupture. In acute ruptured ARSA cases, the mortality rate is reported as high as 50%.  Therefore, it should be electively repaired as soon as it was discovered. It can be diagnosed by a barium esophagram or upper endoscopy, showing a pulsating compression of the posterior wall [5, 6], or by imaging of the aortic arch with computed tomography (CT) angiography or magnetic resonance (MR) angiography.  We present a patient admitting at emergency room with shortness of breath caused by ruptured ARSA.
A 65-year-old male patient was referred to our hospital for sudden onset of shortness of breath and chest pain. In the initial examination, the physical findings were normal. He was in severe respiratory distress. His medical history had an abdominal aortic aneurysm about six years ago. He had been a smoker of 1 pack of cigarettes per day.
In addition, the patient reported no discomfort on swallowing and did not had any history of previous important chest trauma or injury. His blood pressure was 135/75 mmHg both in the right and left arms. Routine chest X-ray was nonspecific. His heart rate was 99 beats/min and oxygen saturation was 96%. Laboratory findings showed (white blood cell count: 12 000/[mm.sup.3], the haemoglobin was 14.5 gm/dl, and C-reactive protein value was 15 mg/dl). About four hours after the admittance to the hospital, ecchymosis occurred on his anterior neck region. The ruptured ARSA were demonstrated by CT-angiography and MR-angiography (figure 1A & 1B). MR revealed hematoma around the ARSA (Figure 2). We offered an operation for the patient, but the patient rejected because of his operation fear.
In 80% of the cases ARSA crosses between esophagus and vertebral column, in 15%, between esophagus and trachea, and in 5% of the cases, anterior to trachea and esophagus.  In our case, ARSA was passing between the esophagus and vertebral column. The most common symptom in such patients is dysphagia because of the compression of the ARSA or the hematoma. However, the patient didn't have any complaints about dysphagia.
There are several treatment options for an ARSA. One of them is conventional surgery. The mortality rate of conventional surgical repair in ARSA patients has a range
between 18% and 25%.  The method proposed by Kieffer et al.  is posterolateral thoracotomy for ligation and repair of ARSA origin involving reconstruction of the right subclavian artery. They reported that the right subclavian artery can be achieved by transposing the distal ARSA onto the ipsilateral common carotid through a cervical incision. Moreover, they reported that 10 patient died in a study that included 33 patients with ARSA. Tochii et al. reconstructed the ARSA in the normal position to avoid the compression of the esophagus and trachea.  Furthermore, median sternotomy can be preferred for easier exposure of the neck vessels, for simultaneous surgical treatment of the coronary or valve lesion, and for the repair of distal arch aneurysm without Kommerell's diverticulum. 
Endovascular treatment and hybrid approch are amongst other options as alternates to surgery. Lacroix et al.  claimed that hybrid treatment for ARSA patients was feasible, safe, and effective. Hybrid approach in that the ARSA is occluded distal to the aneurysm combined with a covered stent at the origin of the aorta is advised in recent literature, even in acute settings. [13, 14] After that, revascularization of the right arm by a bypass or reimplantation may be obtained by using an caroticosubclavian bypass via a right supraclavicular incision. When our patient didn't accept an open surgery, we offered an endovascular hybrid treatment option, but he also rejected it and he was discharged through his own decisions.
A study by Austin et al.  showed that rupture of the aneurysm occurred in six of 31 patients with aneurysms of the ARSA, and it was reported that all of whom subsequently died. A research performing by Kopp et al.  demonstrated that a pre- and intra-operative rupture rate of 22.6% associated with a mortality rate of 100%, both of which were independent from the aneurysm diameter.  Some authors have also reported hemorrhagic shock caused by rupture of ARSA.  In our case, the patient with ruptured ARSA was quite well in his general condition, and the patient was still healthy at the time he was leaving.
In the literature, it was reported that the coincidence of ARSA aneurysm and an abdominal aortic aneurysm (AAA) were between 10% and 20%.  Kopp et al.  reported that three of four patients with an ARSA aneurysm underwent additional operative treatment for their AAA. When our patient were operated about six years ago, ARSA had overlooked. Therefore, we think that the patients with AAA should have continuous screening for ARSA or other arcus aorta anomalies.
In conclusion, treatment of a non-aneurysmal or ruptured ARSA depends on patient's status, anatomical relationship to neighbouring organs, characteristics of the lesion within the ARSA, and complaints of the patient. An endovascular approach may be preferred in cases of high-risk and in cases of rejecting the open conventional surgery. However, open surgery using a bypass or re-implantation is inevitable in young healthy patients that accept open conventional surgery. To the best of our knowledge, this is the first case report of a patient presenting with ecchymosis on anterior neck region with acute onset due to the ruptured ARSA.
Received Date: 07.08.2013
Accepted Date: 16.01.2014
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Source of Support: Nil
Conflict of interest: None declared
Kemalettin Erdem (1), Ahmet Ozden (2), Mansur Kursat Erkuran (3), Tank Ocak (3), Bahadir Daglar (1)
(1) Department of Cardiovascular Surgery, Faculty of Medicine, Abant Izzet Baysal University, Bolu, Turkey
(2) Department of Radiology, Faculty of Medicine, Abant Izzet Baysal University, Bolu, Turkey
(3) Department of Emergency Medicine, Faculty of Medicine, Abant Izzet Baysal University, Bolu, Turkey
Correspondence to: Kemalettin Erdem (email@example.com)
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|Title Annotation:||CASE REPORT|
|Author:||Erdem, Kemalettin; Ozden, Ahmet; Erkuran, Mansur Kursat; Ocak, Tarik; Daglar, Bahadir|
|Publication:||International Journal of Medical Science and Public Health|
|Article Type:||Clinical report|
|Date:||Jan 1, 2014|
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