Figure 1
Barium oesophagram
Sitophobia secondary to aberrant right subclavian artery
SectionChest imaging
Case TypeClinical Cases
AuthorsTae Do Choi; Supriya Gupta, MD1; Jayanth H. Keshavamurthy, MD1; Sridhar Subbaramiah, MD2
Connected authors
17 years, female
Clinical History
A 17-year-old female patient who was concurrently being evaluated for syncope presented with worsening dysphagia towards solids that caused her sitophobia, fear of eating. Echocardiogram showed unusual anatomical anomalies. Barium oesophagram and Computed Tomography Angiography (CTA) confirmed the anatomical anomalies. She eventually underwent surgical revision by paediatric cardiothoracic surgeons
Imaging Findings
Barium oesophagogram (Panel A) demonstrated posterior narrowing of the oesophagus indicative of possible obstruction. CTA (Panel B) confirmed left aortic arch with aberrant right subclavian artery resulting in mild compression of the posterior wall of the oesophagus.
Discussion
Compression of the oesophagus by anomalies of aortic root and arch vasculature defines the term, dysphagia lusoria. The most common anomaly of the aortic arch is aberrant origin and retroesophageal course of the right subclavian artery, which has an incidence ranging from 0.5 to 1.8%. Regression of the fourth vascular arch, between right CCA and right SCA, and right dorsal aorta with persistence of seventh intersegmental artery results in aberrant right SCA also known as arteria lusoria. [1]
In 80% of the cases, the aberrant artery crosses between the oesophagus and vertebral column, such as in our patient. In rare cases, the aberrant artery may originate from a broad base known as Kommerell’s diverticulum. Anatomic and physiologic changes to this diverticulum may explain delayed clinical presentations in some patients. Also, up to 37% of cases show coexisting vascular abnormalities. [2] Our patient was found to have bicuspid valve contributing to her syncope. However, most of vascular arch anomalies are clinically asymptomatic with up to 60 – 80% of patient remaining asymptomatic in their lifespan. [1]
Most cases are found incidentally or secondary to dysphagia and respiratory symptoms. Initial evaluation is barium oesophagram, but diagnosis is made with CT angiography which also helps in planning for surgery. [2]
Since 1965, numerous surgical approaches have been instituted to correct the aberrant flow in symptomatic patients. Our patient underwent left posteriolateral thoracotomy and division of aberrant right SCA with reimplantation to the left SCA by the paediatric cardiothoracic surgery service and is doing well [3]
This case highlights the importance of recognizing symptomatic presentation in an often clinically silent anatomical anomaly.
In 80% of the cases, the aberrant artery crosses between the oesophagus and vertebral column, such as in our patient. In rare cases, the aberrant artery may originate from a broad base known as Kommerell’s diverticulum. Anatomic and physiologic changes to this diverticulum may explain delayed clinical presentations in some patients. Also, up to 37% of cases show coexisting vascular abnormalities. [2] Our patient was found to have bicuspid valve contributing to her syncope. However, most of vascular arch anomalies are clinically asymptomatic with up to 60 – 80% of patient remaining asymptomatic in their lifespan. [1]
Most cases are found incidentally or secondary to dysphagia and respiratory symptoms. Initial evaluation is barium oesophagram, but diagnosis is made with CT angiography which also helps in planning for surgery. [2]
Since 1965, numerous surgical approaches have been instituted to correct the aberrant flow in symptomatic patients. Our patient underwent left posteriolateral thoracotomy and division of aberrant right SCA with reimplantation to the left SCA by the paediatric cardiothoracic surgery service and is doing well [3]
This case highlights the importance of recognizing symptomatic presentation in an often clinically silent anatomical anomaly.
Differential Diagnosis List
Dysphagia lusoria secondary to aberrant right SCA, incomplete vascular ring
Oesophageal stricture
Achalasia
Oesophageal web
Final Diagnosis
Dysphagia lusoria secondary to aberrant right SCA, incomplete vascular ring
References
[1] Levitt B, Richter JE (2007) Dysphagia lusoria: a comprehensive review. Dis Esophagus 20(6):455-60 (PMID: 17958718)
[2] Kellenberger CJ (2010) Aortic Arch Malformations. Pediatr Radiol 40(6):876-84 (PMID: 20354848)
[3] Carrizo GJ, Marjani MA (2004) Dysphagia Lusoria caused by an abberrant right subclavian artery. Texas Heart Institute Journal 31:168-171 (PMID: 15212130)
Case information
| URL: | https://eurorad.org/case/14619 |
| DOI: | 10.1594/EURORAD/CASE.14619 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
CT angiography
Aberrant right subclavian artery is visualized coursing posterior to the trachea (red arrow).
There is mild compression of oesophagus between aortic arch, right subclavian artery, and trachea (Red-dotted arrow). This corresponds to the site of kinking on oesophagus as noted on barium study.
Abberrant subclavian artery has large origin known as Kommerell’s diverticulum (asterisk).
Barium oesophagram
Barium oesophagogram demonstrating kinking of the oesophagus on frontal and lateral views (red arrow), secondary to crossing vessel.
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
Barium oesophagogram demonstrating kinking of the oesophagus on frontal and lateral views (red arrow), secondary to crossing vessel.
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
CT angiography
Aberrant right subclavian artery is visualized coursing posterior to the trachea (red arrow).
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
There is mild compression of oesophagus between aortic arch, right subclavian artery, and trachea (Red-dotted arrow). This corresponds to the site of kinking on oesophagus as noted on barium study.
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
Abberrant subclavian artery has large origin known as Kommerell’s diverticulum (asterisk).
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA
Gupta S, Department of Radiology, Medical College of Georgia at Augusta University, Augusta, GA