ISSN : 1301-5680
e-ISSN : 2149-8156
Turkish Journal of Thoracic and Cardiovascular Surgery     
A rare condition secondary to aberrant right subclavian artery syndrome: Aphagia lusoria
Fatih Ada1, Salih Güler1, Fatmagül Demir2, Ekber Şahin2
1Department of Cardiovascular Surgery, Sivas Cumhuriyet University School of Medicine, Sivas, Türkiye
2Department of Thoracic Surgery, Sivas Cumhuriyet University School of Medicine, Sivas, Türkiye
DOI : 10.5606/tgkdc.dergisi.2022.20723


Dysphagia lusoria is a well-described clinical entity caused by aberrant right subclavian artery. Herein, we present a 42-yearold male case with aberrant right subclavian artery-associated aphagia and progressed to aphagia lusoria presentation. To the best of our knowledge, this is the first associated case presented with aphagia.

Aberrant right subclavian artery syndrome (ARSA) is frequently seen among aortic arch anomalies and the incidence of the disease is 0.5 to 1.8%.[1,2] As 60 to 80% of the patients are asymptomatic, the diagnosis is usually established in incidental or postmortem examinations.[3] Symptomatic patients usually present with solid food intolerance, postprandial bloating, or chest pain. Symptoms often vary depending on the posture. Cough, thoracic pain, and Horner's syndrome are rare symptoms of ARSA. Dysphagia lusoria due to vascular compression of the esophagus is a wellknown clinical entity. Patients are usually admitted to internal medicine and gastroenterology departments initially and get diagnosed. Upper endoscopies of patients are usually normal. A typical external esophageal compression appearance is seen in the barium esophagram. However, the definitive diagnosis is made by computed tomography angiography (CTA) and magnetic resonance angiography (MRA).

Aphagia, the most advanced form of dysphagia lusoria, has not been described in the literature. As patients usually undergo surgery after diagnosis, dysphagia almost never progresses to aphagia. Herein, we present the management of a male ARSA patient who postponed the treatment due to novel coronavirus disease 2019 (COVID-19) pandemic and progressed to aphagia lusoria.

Case Presentation

A 42-year-old male patient was admitted to our clinic with the diagnosis of ARSA. His medical history revealed that he had a dysphagia which was present for a long time and worsened about one year before. A surgical operation was planned after his admission to the outpatient clinic at the beginning of March 2020. However, the patient did not attend to the hospital for operation, due to the concerns about COVID-19 pandemic. He was admitted to the hospital with impaired metabolic values and a weight loss approximately 35 kg at the end of May 2020. He could only drink water for the last three days and could not even swallow saliva on the day of admission to hospital. His routine laboratory tests showed that he was slightly anemic (hemoglobin: 13.2 g/dL), his total protein and albumin was decreased (total protein: 5.7 g/dL, albumin: 3.1 g/dL), and creatinine was slightly elevated (creatinine: 1.32 mg/dL). Coagulation parameters and thyroid function tests were found to be normal. Intravenous support was initiated after the patient was hospitalized. Following hemodynamic recovery, the patient underwent ligation and division of the aberrant right subclavian artery with right anterolateral thoracotomy (Figure 1). Also, the right carotid artery and the right subclavian artery bypass was done using a polytetrafluoroethylene (PTFE) graft with a supraclavicular approach simultaneously (Figure 2). The patient was drinking water easily on postoperative Day 1 and tolerated juicy foods on Day 3. His diet was adjusted predominantly as juicy foods and discharged uneventfully one week after operation. During follow-up, he could consume solid foods without any problem (Figure 3).

Figure 1. (a) Esophagus, (b) Clamped right aberrant subclavian artery.

Figure 2. (a) Preoperative appearance (3-dimensional computed tomography). (b) Postoperative appearance (3-dimensional computed tomography).

Figure 3. (a) Esophageal lumen is totally closed. (b) Esophageal lumen is open.


Aberrant right subclavian artery syndrome was first described by Hunauld[4] in 1735. Approximately five decades later, Bayford defined dysphagia lusoria and, thereafter, it is also called Bayford-Autenrieth dysphagia.[5,6]

Symptoms are usually dysphagia, dyspnea, retrosternal pain, cough, and weight loss in dysphagia lusoria. In a review consisting of 141 reports, the symptoms of female patients appeared earlier than men.[6] In our patient, the main symptoms were initiated with solid food dysphagia, cough, and weight loss. Due to delay in treatment, aphagia was developed and surgery was done under this condition. Barium esophagram is an important test for diagnosis of dysphagia lusoria. However, CTA, MRA and aortography are more valuable in definitive diagnosis. In our case, the diagnosis was made by CTA. Esophageal manometric measurements are used to predict the surgical outcome and follow-up after surgery.[7] In addition to direct complications of ARSA due to vascular ring, there are also late complications such as aneurysm and tracheoesophageal fistula.[8,9] Different congenital anomalies may accompany to ARSA. For instance, ARSA association with aortic coarctation has been described in the literature.[10]

Treatment depends on severity of the symptoms of disease. Diet adjustment may be sufficient in patients who have relatively slight compression, but most patients are candidates for surgical treatment.[11] In our case, surgical treatment was applied, as it was impossible to apply diet therapy.

Surgical mortality was reported between 16 and 25% in previous publications, but this rate has reached almost 0% with surgical and technological developments in recent years.[3,12] There is no standard approach for surgical treatment. It depends on the location of ARSA, complications, presence of other cardiovascular anomalies and experience of center.[10,13] Surgical approach may be right supraclavicular, right anterolateral thoracotomy, and median sternotomy.[14] The main goal of surgical treatment is ligation alone or ligation and division of the aberrant subclavian artery. Reimplantation or bypass of the subclavian artery is essential for upper limb perfusion. In our case, ligation and division of ARSA were performed from a right anterolateral thoracotomy approach. Also, right carotid subclavian bypass operation with a PTFE graft was performed simultaneously. It should be kept in mind that this approach can be used in eligible patients, owing to its excellent exposure and promising surgical results.

In conclusion, in addition to the direct effects of the COVID-19 pandemic, its indirect effects have caused many different clinical pictures. One of these is aphagia lusoria symptom that has not been described before in the literature. As a matter of fact, our case was planned to be treated before proceeding to this situation, but delayed admission caused this result. The fight against the COVID-19 pandemic continues actively all over the world. However, this is only the visible side of the iceberg, and it would take a long time for the consequences of invisible side to appear.

Patient Consent for Publication: A written informed consent was obtained from the patient.

Data Sharing Statement: The data that support the findings of this study are available from the corresponding author upon reasonable request.

Author Contributions: Idea/concept, critical review: F.A., S.G., E.Ş.; Design, analysis and/or interpretation, writing the article, references and fundings: F.A., S.G.; Control/supervision: F.A., E.Ş.; Data collection and/or processing, literature review: F.A., S.G., F.D.

Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Funding: The authors received no financial support for the research and/or authorship of this article.


1) Carrizo GJ, Marjani MA. Dysphagia lusoria caused by an aberrant right subclavian artery. Tex Heart Inst J 2004;31:168-71.

2) Meher R, Sabharwal A, Singh I, Raj A. Dysphagia due to a rare cause. Indian J Surg 2004;66:300.

3) Levitt B, Richter JE. Dysphagia lusoria: A comprehensive review. Dis Esophagus 2007;20:455-60.

4) Hunauld PM. Examen de quelques parties d"un singe. Histoire de l"Acad´emie Royale des Sciences 1735;2:516-23. [Abstract]

5) Bayford D. An account of a singular case of obstructed deglutition. London: Medical Society of London; 1787.

6) Polguj M, Chrzanowski ?, Kasprzak JD, Stefa?czyk L, Topol M, Majos A. The aberrant right subclavian artery (arteria lusoria): The morphological and clinical aspects of one of the most important variations--a systematic study of 141 reports. ScientificWorldJournal 2014;2014:292734.

7) Janssen M, Baggen MG, Veen HF, Smout AJ, Bekkers JA, Jonkman JG, et al. Dysphagia lusoria: Clinical aspects, manometric findings, diagnosis, and therapy. Am J Gastroenterol 2000;95:1411-6.

8) Naqvi SEH, Beg MH, Thingam SKS, Ali E. Aberrant right subclavian artery presenting as tracheoesophagial fistula in a 50-year-old lady: Case report of a rare presentation of a common arch anomaly. Ann Pediatr Cardiol 2017;10:190-3.

9) Kamiya H, Knobloch K, Lotz J, Bog A, Lichtenberg A, Hagl C, et al. Surgical treatment of aberrant right subclavian artery (arteria lusoria) aneurysm using three different methods. Ann Thorac Surg 2006;82:187-90.

10) Karakuş E, Akyüz M, Işık O. Surgical planning of aberrant right subclavian artery and aortic coarctation co-existence. Turk Gogus Kalp Dama 2018;26:163-4.

11) Şahin Y, Aydın Şahin D, Kervancıoğlu M. Aberran sol subklavyen arter basısına bağlı yutma güçlüğü olan bir olgu. ACU Sağlık Bil Derg 2018;9:327-30.

12) Ota T, Okada K, Takanashi S, Yamamoto S, Okita Y. Surgical treatment for Kommerell's diverticulum. J Thorac Cardiovasc Surg 2006;131:574-8.

13) Kouchoukos NT, Masetti P. Aberrant subclavian artery and Kommerell aneurysm: Surgical treatment with a standard approach. J Thorac Cardiovasc Surg 2007;133:888-92.

14) Atay Y, Engin C, Posacioglu H, Ozyurek R, Ozcan C, Yagdi T, et al. Surgical approaches to the aberrant right subclavian artery. Tex Heart Inst J 2006;33:477-81.

Keywords : Aberrant right subclavian artery syndrome, aphagia lusoria, COVID-19, dysphagia lusoria, surgical treatment
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