Dysphagia lusoria in a young patient with a concomitant aortic arch anomaly, successfully treated with surgery. A case report.

doi:10.21203/rs.3.rs-1646758/v1

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Dysphagia lusoria in a young patient with a concomitant aortic arch anomaly, successfully treated with surgery. A case report.

https://doi.org/10.21203/rs.3.rs-1646758/v1

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Background

Dysphagia is a relatively common problem in clinical practice, however, an unusual type of dysphagia due to compression of the esophagus by an abnormal right subclavian artery may be discovered in a rare subset of patients. The prognosis and treatment will depend on the severity of the symptoms and the compromise of surrounding structures.

Case Presentation

We present the case of an 18-year-old female without past medical history. She suffered a two-month gradually progressive dysphagia accompanied by mild reflux. At first, it was treated as gastroesophageal reflux disease, without result. Dysphagia became severe and additional exams were required. An aberrant right subclavian artery that compressed the esophagus was discovered along with a truncus bicaroticus. She was successfully treated with surgery without any complications. On follow-ups, she's doing well.

Conclusions

A symptomatic aberrant right subclavian artery, although rare, should always be corrected after diagnosis to solve symptoms and prevent possible complications. In addition, when necessary, reconstruction of the blood flow to the upper extremity to maintain the arm’s viability and keep the spinal and vertebrobasilar circulation is of paramount importance.

Dysphagia lusoria

Aberrant right subclavian artery

Truncus bicaroticus

Aberrant right subclavian artery is a rare congenital anomaly of the aortic arch, and on some occasions, depending on its severity, the patient may develop troublesome complications. (1, 2) Surveillance of this condition can be variable: non-severe cases can be handled entirely with dietary modifications, yet patients with severe symptoms are best handled with surgery. (1, 3)

We present the case of an 18-year-old female; she presented to our hospital with progressive dysphagia, after clinical evaluations and imaging tests. An aberrant right subclavian artery that compressed the esophagus was discovered along with a truncus bicaroticus. After successful surgery the patient fully recovered

This report has been written in accordance with the care guidelines (11).

Patient is an 18-year-old female without past medical history. She suffered a two-month gradually progressive dysphagia accompanied by mild reflux. At first, it was treated as gastroesophageal reflux disease (GERD), and proton pump inhibitors were given with poor results. Dysphagia became so severe at one point that she could not drink any liquid, including saliva; therefore, she presented at our hospital. Clinical examination, including palpation of her peripheral pulses, was unremarkable; therefore, additional exams were required. An esophagogram (Fig. 1) revealed an extrinsic compression above the level of the aortic arch. With these findings, a computed tomography angiography was requested and demonstrated aberrant anatomy of the aortic arch; the carotid arteries arise from one common trunk, the truncus bicaroticus, and an aberrant right subclavian artery (ARSA) was found compressing the esophagus as it crossed behind it. (Supplementary Video) There was no evidence of a Kommerell's diverticulum, aneurysmal dilation, or other abnormalities. (Fig. 2A, 2B)

Cardiothoracic assessment was required, and after discussing the surgical procedure and its potential complications, surgery was decided. A right posterolateral thoracotomy was done through the third intercostal space; this gave adequate exposure of the mediastinum. First, the esophagus was dissected to expose the ARSA when it was found, the dissection continued proximally to find the aortic arch and distally to have good view and control of the aberrant vessel. Then, exposure was gained toward the patient's right to view the full extent of the aortic arch and its branches. The right subclavian artery arose from the posterior aspect of the aortic arch and was dissected entirely from the esophagus. Once it was free, the right subclavian artery was clamped, and the right upper limb was observed; adequate circulation in the right arm and hand was proved after monitoring blood pressure, oxygen saturation, and clinically confirming good distal capillary refill time and limb color.

Once adequate blood supply of the right upper limb has been confirmed, the right subclavian artery was divided with a vascular mechanical stapler (Covidien, Medtronic Dublin, Ireland), thereby achieving auto-retraction of the artery and freeing the esophagus. Afterward, the lung was re-expanded entirely, a pleural drain was placed, and the chest was appropriately closed. (Fig. 3)

Her postoperative course was uneventful, the right upper limb was closely monitored, and she had no vascular or motor impairment, her dysphagia disappeared and was able to eat a regular diet. She was discharged without any complications. On follow-ups, a right upper limb doppler was done, finding adequate circulation of the extremity.

Congenital anomalies of the aortic arch, although rare (3%), can be associated with vascular rings, congenital heart disease, and anatomical abnormalities. (1) They can have important clinical implications since these variants can encircle and compress many organs, including the trachea or esophagus. (1, 2)

The most common defect found in the adult population is the ARSA (0.3 to 3%). (1, 3) This anomaly, which was first described in 1735 by Hunauld et al., occurs due to the involution of the fourth vascular arch and the right dorsal aorta, leaving the seventh intersegmental artery attached to the descending aorta; in some circumstances, the root of this aberrant artery can have a broad base, usually called Kommerell’s diverticulum. (3) The anatomical course of this anomalous artery can vary as it passes from the chest into the right arm. (1, 4) In most cases (80%), it will cross between the esophagus and the spine. (2) As it happened to our patient. In 15% of the cases, it travels between the esophagus and the trachea, and in 5% of cases, it passes anterior to both the trachea and esophagus. (1, 2, 5).

ARSA can be associated with a concomitant congenital anomaly of the carotid arteries, either with a shared trunk or a close origin; if this happens, it will lead to a decreased distensibility of the esophagus as it is compressed posteriorly by the aberrant vessel leading to dysphagia, most commonly known as dysphagia lusoria. (1, 3) Bayford first described this disease in a young woman who died of permanent malnutrition due to long-term dysphagia. (2, 6) Other abnormalities associated with ARSA include; the ipsilateral vertebral artery arising from the common carotid artery, anomalous point of entrance of the vertebral artery into the cervical spine, thoracic duct ending in the right subclavian, and non-recurrent inferior laryngeal nerve. (1, 6) In our patient, truncus bicaroticus with an aberrant right subclavian artery was discovered.

ARSA is found more often in females, and when present, symptoms usually occur at the two extremes of life if compression of the esophagus or trachea arises. (3, 7) In children, respiratory symptoms are more common, and although dysphagia may be most frequent in adults, children may demonstrate other symptoms like dyspnea, GERD, retrosternal pain, cough, and weight loss. (1) On other occasions, ARSA does not cause any symptoms and can be discovered incidentally (up to 90%). (1, 4) In our patient, progressive dysphagia accompanied by mild reflux was the main symptom.

Diagnosis will depend on the clinical manifestations; some patients will be asymptomatic, although asymmetrical radial pulses may be present if other vascular abnormalities are present. (1, 4) A posterior mass may be visible on X-rays or CT scans. (2) If dysphagia or dyspnea appears, more invasive tests may reveal a posterior, rounded filling defect in the barium swallow or a pulsatile mass on the posterior aspect of the esophageal wall in an upper endoscopy. (1, 7) Nonetheless, the best imaging modalities will be thoracic aortograms, contrast-enhanced CT scans, and magnetic resonance imaging, which are always necessary for preoperative planning. (1, 8) As it was done in our patient.

Treatment will depend on the symptoms; ARSA can be carefully followed without intervention in an asymptomatic patient. (2, 8) However, if symptoms appear or there is a simultaneous aneurysmal formation, treatment should be considered due to the high risk of complications, including aneurysms, rupture, dissection, compression of neighboring structures, and even embolization. (1, 3, 5)

Several treatments have been available since the first successful surgery by Gross in 1946, and now multiple surgical and endovascular therapies are available. (1, 9) The classical approach involves a thoracotomy or thoracoscopy with division and ligation of the right subclavian artery; the use of stents grafts and transposition of the right subclavian artery with carotid subclavian bypass will depend on the associated vascular anomalies like truncus bicaroticus that may compromise blood flow to the right upper limb, spine, and vertebrobasilar circulation. (2, 4, 5) Endovascular or hybrid techniques could be appropriate in particular cases reducing morbidity and mortality; however, this approach is only suitable in cases without aneurysms because the origin of the ARSA cannot be ligated. (1, 10) Also, severe complications, including migration of the plugs, esophageal fistula, embolisms, and stroke, should be considered. (2, 6) In patients who won't tolerate surgery, endoscopic dilation of the esophageal narrowing may be used as a palliative treatment. (1, 2,)

In our case, ARSA was diagnosed and surgery was needed since dysphagia was severe; after surgery, the patient completely recovered and was able to eat and drink as usual and showed no neurological or vascular compromise.

Dysphagia is a common symptom seen in daily clinical practice and regretfully is not usually given the significance it deserves. A high degree of clinical insight is needed if dysphagia lusoria is not to be missed. If ARSA is symptomatic, it should be treated promptly as severe complications can appear. Given the rarity of this condition and limited experience, treatment should be considered on a case-to-case basis to provide the best available care.

Patient perspective

At first, the patient was unsure about her treatment, whether it would hurt, or whether she could be “normal” again; nonetheless, since the surgery was successful, she was grateful to the medical team and was happy since she was able to eat again.

Funding

None

Declaration of competing interest

We have no conflict of interest to declare

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors and has been approved by the ethics committee of our hospital.

Consent

We declare no conflict of interest.

Written informed consent to publish has been received from all participants should appear in the manuscript.

The submission has not been previously published, nor is it with another journal for consideration.

All authors listed on the paper have reviewed and approved the manuscript in its current form and are aware that it has been submitted.

Acknowledgments

None.

Author contribution

SE and GM analyzed and interpreted the patient data and did the surgical treatment AC was a major contributor in writing the manuscript. KG prepared the figures. Revised the manuscript and reviewed all the available data.

All authors read and approved the final manuscript.

Data availability statement

The data generated during the current study are available from the corresponding author on request.

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Dysphagia lusoria in a young patient with a concomitant aortic arch anomaly, successfully treated with surgery. A case report.

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Abstract

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Introduction

Case Report

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