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Ann Thorac Surg 2001;71:710-712
© 2001 The Society of Thoracic Surgeons

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Case report

Late-onset dysphagia lusoria

^a Division of Cardiothoracic Surgery, Joseph B. Whitehead Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA

Accepted for publication March 27, 2000.

Address reprint requests to Dr Miller, Section of General Thoracic Surgery, Crawford Long Hospital, The Emory Clinic, 25 Prescott St, NE, Suite 3420, Atlanta, GA 30308
e-mail: tracy.starkey{at}surgery.eushc.org

	Abstract

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Symptomatic vascular rings are uncommon in adults. We report a case of dysphagia in a 48-year-old woman from a right-sided aortic arch with aberrant left subclavian artery and left-sided ligamentum arteriosum. Through a left posterolateral thoracotomy, the ligamentum was divided, relieving the patient’s dysphagia. Twenty-three cases of adult-onset dysphagia lusoria attributable to this anatomy have been reported. We recommend a left thoracotomy with division of the ligamentum as treatment.

	Introduction

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The syndrome of esophageal compression from an abnormal vascular structure was first described by Bayford in 1794 [1]. During the autopsy of a starved, previously dysphagic 62-year-old woman, Bayford identified a misplaced right subclavian artery encroaching on her esophagus [1, 2]. Today, the term dysphagia lusoria is applied to dysphagia resulting from any type of vascular ring, with symptoms usually occurring in infants and children. We report a case of a right-sided aortic arch with aberrant left subclavian artery and left ligamentum (RAA/ALSC) that became symptomatic in an adult.

A 48-year-old woman presented with a 20-year history of gastroesophageal reflux disease and a 6-month history of dysphagia, first to solids, then to liquids. The physical exam had normal results.

A chest roentgenogram revealed a right-sided aortic arch, and the possibility of a vascular ring was entertained. A barium esophagogram showed a well-circumscribed indentation in the posterior aspect of the thoracic esophagus (Fig 1). An aortogram demonstrated the aberrant left subclavian artery as the last structure originating from a small Kommerell’s diverticulum (Fig 2).

View larger version (125K): [in this window] [in a new window]   Fig 1. Barium esophagogram demonstrating indentation in the posterior thoracic esophagus.

View larger version (183K): [in this window] [in a new window]   Fig 2. Aortogram showing the right-sided aortic arch and aberrant left subclavian artery.

At 24 hours, a barium swallow showed a normal esophagus. The patient tolerated liquids by the third postoperative day and solid food by the fifth. She was subsequently discharged and remains symptom free at 3 months.

	Comment

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The term vascular ring describes malformations of the aortic arch that render the esophagus or trachea partially or completely entrapped. The RAA/ALSC is a form of complete vascular ring that can be explained embryologically by recalling that humans begin with six aortic arches [3]. Edwards [3] hypothesized that these arches form paired segmental structures with a double arch and bilateral ductus arteriosi. Normal development occurs upon involution and preservation of certain segments. RAA/ALSC results from a persistent right fourth arch and absent left arch, which is interrupted between the left common carotid and left subclavian [4].

RAA/ALSC is the second most common vascular ring requiring surgery, occurring in an estimated 1 in 1,000 individuals in the general population [2]. It causes 25 to 30% of cases of vascular rings, typically presenting in early infancy or childhood with respiratory symptoms [5]. Adults primarily complain of dysphagia. Although the major cause of impingement in our case was the ligamentum, atherosclerotic disease or age-related dilatation may contribute to late-onset dysphagia in some instances [1].

The workup of the patient with dysphagia should always include a chest roentgenogram, which will reveal the right-sided arch. An esophagogram is likewise necessary, and it will show a distinct concavity in the thoracic esophagus [6]. Aortography is an important test, but one should remember that the aortogram may not distinguish a double aortic arch with atretic left arch from RAA/ALSC [7].

Since Hallman and Cooley [8] reported their pediatric series, the most common approach to a RAA/ALSC has been a left posterolateral thoracotomy. This incision provides the best access to the middle and posterior mediastina [5, 7, 8]. The ligamentum should be divided, and the esophagus, aberrant artery, trachea, and all fascial bands encircling these structures should be dissected free [1, 4, 5, 8]. The decision to ligate the aberrant subclavian or to reimplant it remains an intraoperative one. Experience in children demonstrates some recurrence of symptoms if the subclavian is not divided, and many authors advise proceeding with this step, with most suggesting reimplantation to avoid steal syndrome [1, 8]. In adults, the division of the ligamentum with dissection of mediastinal structures allows the vessels to assume a less constricting pattern. Division of the retroesophageal artery is often unnecessary, as it was in this case [4].

Adult presentation of dysphagia lusoria is rare; 23 cases secondary to RAA/ALSC have been reported in the world literature. Operative repair should occur in an adult with progressive symptoms. We recommend left posterolateral thoracotomy with division of the ligamentum.

	References

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1. Drucker M.H., Symbas P.N. Right aortic arch with aberrant left subclavian artery: symptomatic in adulthood. Am J Surg 1980;139:432-434.[Medline]
2. McNally P.R., Rak K.M. Dysphagia lusoria caused by persistent right aortic arch with aberrant left subclavian artery and diverticulum of Kommerell. Dig Dis Sci 1992;37:144-149.[Medline]
3. Edwards J.E. Anomalies of the derivatives of the aortic arch system. Med Clin N Am 1948;32:925-949.[Medline]
4. Adkins R.B., Maples M.D., Graham B.S., Witt T.T., Davies J. Dysphagia associated with aortic arch anomaly in adults. Am Surg 1986;52:238-245.[Medline]
5. Van Son J.A., Julsrud P.R., Hagler D.J., et al. Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc 1993;68:1056-1063.[Medline]
6. Jung J.Y., Almond C.H., Saab S.B., Lababidi Z. Surgical repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. J Thorac Cardiovasc Surg 1978;75:237-243.[Abstract]
7. Chun K., Colombani P.M., Dudgeon D.L., Haller J.A. Diagnosis and management of congenital vascular rings: a 22 year experience. Ann Thorac Surg 1992;53:597-603.[Abstract/Free Full Text]
8. Hallman G.L., Cooley D.A. Congenital aortic vascular rings: surgical considerations. Arch Surg 1964;88:666-675.

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				A. Gupta and M. C Winslet Tortuous common carotid artery as a cause of dysphagia J R Soc Med, June 1, 2005; 98(6): 275 - 276. [Full Text] [PDF]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Kirk R. Kanter Joseph I. Miller, Jr Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Morris, C. D. Articles by Miller, J. I. Search for Related Content PubMed PubMed Citation Articles by Morris, C. D. Articles by Miller, J. I., Jr Related Collections Great vessels Esophagus - other