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Year : 2005  |  Volume : 15  |  Issue : 4  |  Page : 497-501
CT and MR angiography in dysphagia lusoria in adults


Department of Radiodiagnosis, GB Pant Hospital, MAM College, New Delhi-2, India

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Keywords: Angiography, Dysphagia, Esophagus

How to cite this article:
Puri S K, Ghuman S, Narang P, Sharma A, Singh S. CT and MR angiography in dysphagia lusoria in adults. Indian J Radiol Imaging 2005;15:497-501

How to cite this URL:
Puri S K, Ghuman S, Narang P, Sharma A, Singh S. CT and MR angiography in dysphagia lusoria in adults. Indian J Radiol Imaging [serial online] 2005 [cited 2020 Oct 31];15:497-501. Available from: https://www.ijri.org/text.asp?2005/15/4/497/28782

   Introduction Top


Congenital anamolies of the aortic arch and great vessels are rare anamolies which may be encountered incidentally during imaging or present with symptoms, most commonly dysphagia.

The first description of dysphagia secondary to vascular compression of esophagus was by Bayford in 1794 when he noted esophageal compression by an anamolous right subclavian artery at autopsy in a patient with progressive dysphagia.He introduced the term dysphagia lusoria , from " lusus naturae", meaning a freak of nature and named the aberrant right subclavian artery as " arteria lusoria"[1].

The aberrant right subclavian artery is the commonest aortic arch anamoly in adults and occurs in 0.5% of general population. Left aberrant subclavian artery with right sided aortic arch occurs in 0.06% to 0.1% patients [2].We report two cases of aberrant right subclavian artery and one case of aberrant left subclavian artery which were diagnosed by CT and MR angiography. One of the two cases of aberrant right subclavian artery proved to be an incidental finding whereas the other two presented with dysphagia.

CASE 1

A 35-years-old female presented with dysphagia of many years duration.Dysphagia was worse with solids .She had no symptoms suggestive of regurgitation,early satiety or weight loss.

Her physical examination was unremarkable.Endoscopic examination revealed an extrinsic,pulsatile impression over the posterior wall of esophagus, at 20 cm.Barium swallow examination revealed an oblique extrinsic impression over the posterior wall [Figure - 1]a. Mass was pulsatile as seen on fluoroscopy.Spiral CT angiography was performed for aortic arch and great vessels.120 ml 300mg% non-ionic contrast was injected at a rate of 3.5 ml/sec and 3mm sections were taken at 1.3 pitch. 3D MIP and SSD reconstructions were made which revealed an aberrant right subclavian artery ,arising as a fourth branch distal to left subclavian artery [Figure - 1]d.It had a broad base representing diverticulum of Kommerell.The aberrant artery was coursing towards the right side posterior to both the carotids.Axial sections clearly demonstrated the artery coursing posterior to the trachea and esophagus and was indenting the posterior wall of esophagus [Figure - 1]b,c.

Patient was advised dietary modifications in the form of more liquid and semi-solid foods and was managed conservatively

CASE 2

A 52-years -old female with Caries spine involving C7 to D1 vertebrae was having difficulty in swallowing since last 6 months.MRI scan revealed typical features of Caries spine and a prevertbral abscess at the same level.In addition the sagittal and axial TIW, T2W and B-TFE images revealed a signal void lesion representing a vessel coursing posterior to the esophagus and indenting it [Figure - 2]a,b.The vessel could be identified as abberant right subclavian artery. In the same sitting 3D contrast enhanced MR angiography was performed using 20 ml of Gadolinium injected at a rate of 2.5 ml/sec.Scanning was done in coronal plane using TIFFE sequence. MIP images revealed the aberrant subclavian artery arising from distal aortic arch, distal to left subclavian artery and coursing towards right side posterior to carotid arteries [Figure - 2]c.

Patient was first put on anti-tubercular treatment and subsequently as the prevertebral abscess resolved her dysphagia also disappeared suggesting that the aberrant artery was only an incidental finding.

CASE 3

A 30-years-old male patient presented with severe dysphagia since early childhood. Dsysphagia was more with solids as compared to semi-solids and liquids.He also experienced retrosternal cramps and heartburn.Endoscopy showed evidence of esophagitis and a small hiatus hernia.In addition, a pulsatile extrinsic mass was seen indenting the posterior wall at 22 cm. Patient was referred for a barium swallow examination with a probable diagnosis of an aberrant vessel indenting the esophagus.Barium swallow showed a large, smooth, extrinsic indentation of esophagus from right and posterior aspect [Figure - 3]a. Aortic arch was on right side of spine.Spiral CT of upper chest with IV contrast revealed a right sided aortic arch and an aberrant left subclavian artery.There was a large aortic diverticulum arising from arch from which the left aberrant artery was arising.The divertculum was indenting the esophagus from posterior aspect [Figure - 3]b,c.

Patient was put on prokinetic and H2 receptor blockers for esophagitis and hiatus hernia.His symptoms improved moderately and was therefore managed conservatively with advise to take more of liquid and semi-solid foods.


   Discussion Top


The "lusarian artery" or aberrant right subclavian artery is the most common malformation of the aortic arch and occurs in approximately 0.5% of the population [2].In this anamoly the right subclavian artery does not arise from the brachiocephalic artery but instead develops as a fourth branch of the aortic arch that is distal to the left subclavian artery. Relating this abnormality to the hypothetical embryological double aortic arch system of Edward's, it is caused by regression of the right fourth branchial arch segment between the right common carotid artery and right subclavian artery, instead of the segment distal to right subclavian artery. In most cases, the aberrant right subclavian artery courses posterior to the esophagus ,but it courses between the trachea and esophagus in 18% cases and in front of trachea in 4% cases.The aberrant right subclavian artery may arise from a diverticulum like structure, called divertculum of Kommerell, representing a persistence of the embryological right aortic arch.This may simulate an aneurysm and knowledge of the entity is important to avoid misinterpretation[1],[3].

The right sided aortic arch results from persistence of the entire dorsal arch and involution of a segment of the left arch.The arch lies to the right of the spine before descending on either the right or left of the spine. There are two main types of the right aortic arch.The first is right aortic arch with aberrant left subclavian artery in which the interruption occurs between the left common carotid and left subclavian artery in the embryogenic left arch.This results in four branches of the right aortic arch. Starting proximally they are,in order,the left common carotid artery,right common carotid artery,right subclavian artery and left subclavian artery.The distal portion of the embryogenic left arch usually persists as an aortic diverticulum and gives rise to the aberrant left subclavian artery as the fourth branch of the right aortic arch which courses towards left side,posterior to the esophagus.The second type of right aortic arch is with mirror image branching.The arch gives origin to three branches: left brachiocephalic trunk,right common carotid artery and right subclavian artery.It is therefore mirror image of the normal anatomy.There is a far higher incidence of severe congenital heart disease in the individuals with mirror image right aortic arch with almost all having cyanotic heart disease, usually due to Tetrology of Fallot[4],[5].

Retrospective analysis of patients symptoms and autopsy studies indicate that a substantial proportion,60-70% patients remain free of symptoms during their lives.Symptoms,when present occur at the two extremes of life in these patients .In children tracheal obstruction or dysphagia can occur.The increased frequency of pulmonary infections seen in infants is thought to be due to absence of tracheal rigidity in combination with dyspagia and aspiration.In the adults, congenital vascular anamoly of aortic arch and major branches is a rare cause of dysphagia, classically termed as 'dysphagia lusoria'.Various mechanisms proposed as to why dysphagia occurs in elderly patients are: i) increased rigidity of trachea leading to easy compression of esophagus ii) aneusysm formation especially in presence of Kommerell's diverticulum iii) elongation of the aorta and iv) the combination of an aberrant artery and a truncus bicaroticus or a close origin of carotid arteries from the aorta which limits the anterior displacement of trachea and esophagus [1]. Isolated case reports about rare symptoms produced by aberrant right subclavian artery mention superior vena cava obstruction, gastrointestinal bleeding and distal embolisation secondary to thrombosis[6].

Barium contrast examination of the esophagus shows a characteristic diagonal extrinsic,smooth impression at the level of third and fourth dorsal vertebra.Lateral or oblique views show the extrinsic impression to be posterior ,just above the level of aortic arch in case of aberrant right subclavian artery.Because dysphagia occurs frequently with ingestion of solid foods,including a barium soaked bread bolus may improve localization of the defect.In case of left aberrant subclavian artery, if the descending aorta crosses to the left of spine,it produces a significant anterior displacement of the esophagus.With a right descending aorta, a smaller posterior compression defect is produced by the aortic diverticulum or aberrant left subclavian artery as it courses over towards the left arm[7].

Contrast enhanced CT or MRI can easily confirm the findings of barium swallow and in addition they can demonstrate the exact relationship of the anamolous vessel with surrounding structures like esophagus or trachea etc.More commonly , these anamolies are encountered incidentally when they need to be recognized and distinguished from other serious pathology.Traditionally catheter angiogram has been accepted as a gold standard for detailed pre-operative assessment of the patient.However, today with the availability of 3D spiral CT angiography and 3D contrast enhanced MR angiography, one can non-invasively demonstrate with exquisite details all such vascular anamolies including details like atherosclerotic changes,aneurysm formation and diverticulum etc [7],[8].Hence we can completely do away with invasive catheter angiography, even for pre-operative assessment, in patients selected for surgical correction.

Dysphagia in patients with vascular anamoly, need not be because of vascular compression which may be incidental only and symptoms can be because of co-existing esophageal abnormalities like motility disorder, hiatus hernia,reflux disease or Barrett's esophagus[1].Like in one of our patients the dysphagia was because of prevertebral abscess and the aberrant right subclavian artery was an incidental finding. Therefore it is imperative to first treat these patients with prokinetic or anti-reflux drugs or for any other associated condition which might be responsible for dysphagia and only if the symptoms are intractable should these patients be subjected to surgical correction of the anamoly.In addition to this,the decision to operate also depends on the presence of aneurysm near the root of the artery, and the age of the patient. Kieffer et al have noticed relief of symptoms in 16 out of 19 patients with dysphagia and an aberrant artery[1],[7]. For infants and young children with signs of airway and/or esophageal compression, surgery is the treatment of choice to prevent worsening of symptoms.

 
   References Top

1.Janssen M,Baggen MGA,Veen HF et al. Dysphagia lusoria:Clinical aspects,manometric findings,diagnosis and therapy.Am J Gastroenterol 2000;96: 1411-1416.  Back to cited text no. 1    
2.Van Dyke CW, White RD. Congenital abnormalities of the thoracic aorta presenting in the adult. J Thorac Imaging 1994;9:230-245  Back to cited text no. 2    
3.Kalke BR, Magotra R, Doshi SM. A new surgical approach to the management of symptomatic aberrant right subclavian artery. Ann Thorac Surg 1987;44: 86-89.  Back to cited text no. 3  [PUBMED]  
4.Salomonowitz E,Edwards JE, Hunter DW et al. The three types of aortic diverticula. Am J Roentgenol 1984;142:673-679.  Back to cited text no. 4    
5.Davies M and Guest PJ. Developmental abnormalities of the great vessels of the thorax and their embryological basis. Br J Radiology 2003;76: 491-502.  Back to cited text no. 5    
6.Akers DL Jr,Fowl RJ, Plettner J, Kempezinski PF. Complications of anamolous origin of right subclavian artery.Case report and review of literature.Ann Vasc Surg 1991;5:385-388.  Back to cited text no. 6    
7.NguyenP, Gideon M and Castell D. Dysphagia lusoria in the adult: Associated esophageal manometric findings and diagnostic use of scanning technique. Am J Gastroenterol 1994;89:620-623.  Back to cited text no. 7    
8.Jaffe RB. Radiographic manifestations of congenital anamolies of the aortic arch. Radiol Clin North Am 1991;29:319-334.  Back to cited text no. 8  [PUBMED]  

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Correspondence Address:
S K Puri
Department of Radiodiagnosis GB Pant Hospital, MAM College, New Delhi-110002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.28782

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    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

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