Asian Cardiovasc Thorac Ann 2006;14:e45-e47
© 2006 Asia Publishing EXchange Ltd
Adult Dysphagia Associated with Type B Double Aortic Arch
Ganesh Shanmugam, FRCS,
James Pollock, FRCS
Department of Cardiac Surgery, Royal Hospital for Sick Children, Glasgow, United Kingdom
For reprint information contact: James Pollock, FRCS Tel: 44 141 201 0269 Fax: 44 141 201 9204 Email: jim.pollock{at}yorkhill.scot.nhs.uk., Department of Cardiac Surgery, Royal Hospital for Sick Children, Dalnair Street, Glasgow G3 8SJ, Scotland, United Kingdom.
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ABSTRACT
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Esophageal compression secondary to a double aortic arch is rare in adults due to its propensity to cause severe symptoms in infancy or childhood. We report four adult patients with dysphagia associated with a double aortic arch. Chromosome analysis in one of these patients revealed a balanced translocation between chromosomes 4 and 18 – this has not been previously reported. Surgical division is indicated in all symptomatic patients and provides excellent operative results.
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INTRODUCTION
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Vascular rings are congenital abnormalities of the aortic arch that result in complete or partial encirclement of the trachea or esophagus, and have been well documented to cause respiratory and gastrointestinal symptoms in infants and children. However, few studies clearly document symptomatic vascular rings in adults. Double aortic arch (DAA) is a rare vascular anomaly, particularly in adults.
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CASE REPORT
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The first patient was a 28-year-old lady presenting with dysphagia. Chest X-Ray showed an indeterminate arch. A Barium swallow showed bilateral esophageal indentations (Figure 1
), suggestive of DAA. Esophagoscopy revealed inflammation at the site of compression. This patient had an obstetric history characterized by multiple miscarriages. Chromosome analysis revealed a translocation between chromosomes 4 and 18.
The three other patients were males, aged 28, 29 and 31 years respectively, all presenting with dysphagia. Computed tomography (CT) demonstrated DAA with a dominant right arch in all cases.
Operative access in all four patients was through a left fourth space thoracotomy. The left arch between the left subclavian and the left sided descending thoracic aorta (DTA) was atretic. The atretic arch (Figure 2) constricting the mid-esophagus was divided between vascular clamps, between the left subclavian and the DTA. The divided stumps were oversewn with prolene. The ligamentum arteriosum was divided. All four patients had normal postoperative recoveries, were discharged home at a median of 5 days, and reported complete resolution of the dysphagia at a median follow-up of 11 years.
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DISCUSSION
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The hypothetic DAA system proposed by Edwards explains aortic arch development.1 Six pairs of aortic arches link the ventral and dorsal aortas. The right fourth arch involutes while the left arch persists as the aortic arch.
Classic DAA anatomy develops when the distal right fourth arch persists and along with the ventral and dorsal aortae encircles and entraps the tracheo-esophageal axis. Double aortic arch is sometimes associated with a band 22q11 deletion. Brzezinska-Kolarz and coworkers reported familial recurrence of DAA, supporting a genetic etiology for this anomaly.2 Cytogenetic examination of their patients disclosed a pericentric inversion of chromosome 9. Chromosome analysis in one of our patients revealed a translocation between chromosomes 4 and 18. It is not known whether this anomaly was coincidental or was a causal factor in the etiology of the double arch. To our knowledge, this has not been previously reported.
Patients with early respiratory symptoms tend to have very tight rings, while those with loose rings can present with dysphagia during adulthood. Two of our patients had mild dysphagia as children, but were diagnosed to have DAA only when their symptoms became severe during adulthood.
The ascending aorta divides into a left anterior and a right posterior arch, which encircle the trachea and esophagus and join posteriorly to form the DTA. The retroesophageal right arch crosses the midline posteriorly, and compresses the esophagus, causing dysphagia. The right arch gives rise to the right carotid and subclavian vessels. The left carotid and subclavian arteries arise from the left arch. The DTA is usually left sided. In patients with a right descending aorta, the arches are right anterior and left posterior.
A monograph from the Mayo Clinic divided DAA into: type A – DAA with both arches patent; type B – DAA with left arch atresia distal to the origin of the left subclavian artery and left ligamentum.3 The patients in this study all had type B – DAA. Usually both arches are patent. Right arch dominance with left arch atresia is less common. This subtype is further classified according to the site of atresia:4
Subtype 4: Proximal to the left common carotid artery;
Subtype 3: Between the left carotid and the left subclavian arteries;
Subtype 2: Between the left subclavian and the ligamentum arteriosum;
Subtype 1: Between the ligamentum and the DTA.
Right arch atresia is extremely rare. All our patients had subtype 1, according to this classification. This classification helps determine the site of arch division.
The recent literature demonstrates a degree of variability regarding the preferred strategy for the diagnosis of vascular rings. The frontal esophagogram shows two esophageal indentations – the right being higher and larger.4 The lateral view demonstrates a fixed posterior indentation. The extant anatomy of a vascular ring, necessary for accurate planning of the surgical approach, cannot however be delineated by esophagography. Computed tomography identifies vascular structures and tracheo-bronchial anatomy. In patients with DAA, there are four separate bracheocephalic vessels (instead of the normal three) in the superior mediastinum grouped around the trachea. This is called the "four vessel" sign. The ability of MRI to demonstrate vascular channels due to the flow-void phenomenon makes it particularly well suited to diagnose vascular rings.5 Volume reconstruction produces 3 dimensional images, thus providing enhanced demonstration of the anatomy. Due to its non-invasive nature, and the multiplanar imaging capability, MRI with magnetic resonance angiography is now considered the study of choice to delineate vascular rings.
Other causes of adult dysphagia secondary to vascular compression include an aberrant right subclavian artery, a right aortic arch with an aberrant left subclavian artery and a right arch with a left ligamentum arteriosum.
A left posterolateral thoracotomy provides excellent access to the middle and posterior mediastina. The phrenic, vagus, and recurrent laryngeal nerves should be identified and preserved. The left subclavian, and left common carotid arteries, the left aortic arch, the ligamentum, the DTA, and if possible, the extremity of the right arch, should be located.6 Simple ligation and division can cause ligature slippage and catastrophic hemorrhage. The ligamentum is always divided. The trachea and the esophagus must be carefully dissected to eliminate potentially constrictive fibrous bands. It is important to avoid damaging the esophagus, which lies in front of the right posterior arch. Video assisted thoracoscopic surgery (VATS) is useful in cases where an atretic arch or a ligamentum requires division.7 Video assisted thoracoscopic surgery division of a patent arch is controversial. Complications of unoperated rings include dissection and aorto-esophageal fistula.8 Postoperative complications include neural injury and chylothorax. Operative intervention results in significant improvement in most symptomatic patients.
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REFERENCES
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- Edwards JE. Anomalies of derivatives of aortic arch system. Med Clin North Am 1948;32:925–49.[Medline]
- Brzezinska-Kolarz B, Sanak M, Musial J, Szczeklik A. Double aortic arch associated with pericentric inversion of chromosome 9. Polish Heart Journal 1997;Vol XLVII, Nr 1.
- Stewart JR, Kincaid OW, Edwards JE. An atlas of vascular rings and related malformation of the aortic arch system. Charles C Thomas. Springfield: 1964.
- Jaffe RB. Radiographic manifestations of congenital anomalies of the aortic arch. Radiol Clin North Am 1991;29:319–34.[Medline]
- van Son JA, Julsrud PR, Hagler DJ, Sim EK, Puga FJ, Schaff HV, et al. Imaging strategies for vascular rings. Ann Thorac Surg 1994;57:604–10.[Abstract]
- Langlois J, Binet JP, De Brux JL, Hvass U, Planche C. Aortic Arch Anomalies. In MC Fallis, RM Filler and G Lemoine Editors. Pediatric Thoracic Surgery. Elsevier Science Publishing Co, New York 1991:172–191.
- Burke RP, Rosenfeld HM, Wernovsky G, Jonas RA. Video-assisted thoracoscopic vascular ring division in infants and children. J Am Coll Cardiol 1995;25:943–7.[Abstract]
- Midulla PS, Dapunt OE, Sadeghi AM, Quintana CS, Griepp RB. Aortic dissection involving a double aortic arch with a right descending aorta. Ann Thorac Surg 1994;58:874–5.[Abstract]
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ABSTRACT
INTRODUCTION
