Ann Thorac Surg 1995;60:1411-1413
© 1995 The Society of Thoracic Surgeons
Case Reports
Vascular Ring Causing Symptomatic Tracheal Compression in Adulthood
Elie Fadel, MD,
Alain R. Chapelier, MD,
Jacques Cerrina, MD,
Paolo Macchiarini, MD,
Philippe G. Dartevelle, MD
Department of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Hôpital Marie-Lannelongue, Paris-Sud University, Le Plessis Robinson, France
Accepted for publication July 25, 1995.
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Abstract
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A 32-year-old woman was referred for evaluation of a 3-year history of exertion dyspnea. An anomalous vascular ring created by a right aortic arch with mirror-image branching, left descending aorta, and aortic diverticulum narrowed the trachea circumferentially. This feature was surgically approached through a right posterolateral thoracotomy, and simple amputation of the aortic diverticulum resulted in immediate symptomatic relief.
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Introduction
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Symptomatic vascular ring due to aortic arch abnormalities revealed during adult life are uncommon [1]. We report a case of mirror-image right aortic arch (RAA) with a left-sided descending aorta and aortic diverticulum that became symptomatic late in the third decade of life.
A 32-year-old woman was referred to our institution for evaluation of a 3-year history of exertion dyspnea. Physical examination revealed an alert, oriented woman whose only complaint was severe exertion dyspnea. Chest roentgenography showed a right-sided aortic arch without any other lung or heart abnormalities. Bronchial fibroscopy revealed no recurrent nerve palsy but an extrinsic compression of the right posterolateral tracheal wall situated 4 cm above the carina; it narrowed almost 50% of the trachea's lumen. The remaining tracheobronchial tree was normal. Pulmonary function tests revealed a mild restriction; her predicted forced expiratory volume in the first second and predicted forced vital capacity values were both 70%.
Contrast-enhanced computed tomographic scan of the thorax demonstrated a vascular ring, narrowing the trachea, that was formed by three elements: (1) anteriorly, a right-sided aortic arch, (2) posteriorly, a left-sided descending aorta, and (3) on the left, an aortic diverticulum (Fig 1
). The aortic arch incorporated the right common carotid and subclavian arteries and a left brachiocephalic artery, branching a left common carotid and a subclavian artery; the latter was morphologically normal and without an aberrant course. The descending aorta was behind the esophagus and on the left side of the spine. An aortic diverticulum, measuring 3.5 cm in its maximal diameter, was located at the junction of the right-sided aortic arch and descending aorta, and prolonged between the vertebral column and esophagus. Single-contrast esophagography showed a right-sided posterior indentation of the esophagus. Aortography showed a right-sided aortic arch with mirror-image branching and an upper descending aortic diverticulum (Fig 2).
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Fig 1. . Contrast-enhanced computed tomogram of the chest showing a narrowed trachea by a vascular ring formed by a right aortic arch (AA), descending aorta (DA), and aortic diverticulum (star).
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Fig 2. . Lateral aortogram detailing the right aortic arch with mirror-image branching and the aortic diverticulum.
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A right posterolateral thoracotomy in the fourth intercostal space was performed. After division between ligatures of a normal right-sided azygos vein, the distal aortic arch and descending aorta beyond the origin of the right subclavian artery were dissected free. By extending the dissection toward the posterior mediastinum behind the trachea, a diverticulum developing from the left lateral aspect of the aorta was found; its transverse diameter paralleled that of the aorta. After its mobilization, the diverticulum was selectively stapled at its base with a TA-30 surgical device. This step generated an immediate retraction of the distal diverticular stump that leaved an overture into the posterior mediastinum. Inspection of the distal aortic diverticulum showed that it was closed. Intraoperative fiberoptic examination confirmed the relief of the tracheal compression. The esophagus and trachea were then dissected free, and the pleural cavity closed as usual.
Postoperatively, the patient did well and had a complete symptomatic relief. Computed tomographic scan of the thorax 1 month later revealed no extrinsic compression.
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Comment
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Edwards [2] postulated that a double aortic arch system represents the embryologic cornerstone from which all aortic arch abnormalities occur. This double aortic arch system includes an aortic arch and a ductus arteriosus on each side and a descending aorta in the midline. In human beings, the normal development comprises interruption of the dorsal segment of the right arch between the right subclavian artery and descending aorta with regression of the ductus arteriosus. All aortic arch malformations derive from interruption of this arch system at different locations. Among them, RAA abnormalities include: (1) RAA with mirror-image branching of the major arteries, (2) RAA with an aberrant left subclavian artery, and (3) RAA with the left subclavian artery isolated from the aorta [3].
Right aortic arch abnormalities are more commonly diagnosed and treated during childhood; less frequently, they cause symptomatic tracheoesophageal compression during the adult life [1]. Although the RAA abnormality observed in the present case is not included in the classification of Edwards [2], we speculate that its origin was related to the interruption of the left aortic arch between the left subclavian artery and the ductus arteriosus. This last remained attached to the remnant of the posterior part of the left arch from which the aortic diverticulum developed. Consequently, the vascular ring was composed anteriorly by the RAA, posteriorly by the left-sided descending aorta, and on the left by the aortic diverticulum. The ductus or ligament arteriosus ran from the anterior aspect of the diverticulum to the left pulmonary artery.
The unusual peculiarity of this case is that the proximal descending aorta had an abrupt circumflex course to the left side of the vertebral column; as far as we know, only 4 similar cases have been reported [4]. However, 3 of them occurred in childhood; the last was diagnosed at autopsy. The reasons for the late clinical onset observed in the present case might be extrapolated from the theory of D'Souza and associates [4]. The traction of the aortic diverticulum induced by the ductus ligament was unilateral and rendered it mobile until the full development of the left-sided descending aorta, the oblique course of which might have progressively increased the contact area of the posterior aspect of the aortic diverticulum with the trachea.
The management of this type of disease usually has been simple division of the ductus ligamentum through a left thoracotomy, leaving the aortic diverticulum intact. However, it might be criticized because it can be associated with late symptomatic relapses, as has been observed after RAA associated with aberrant left subclavian artery and Kommerel diverticulum [1]. Although in case of aberrant right subclavian artery disease the treatment we advocate is always through a left thoracotomy [5], here we preferred a right thoracotomy that allows a more direct visualization and full mobilization of the aortic diverticulum and safe lateral clamping of the aorta. The use of the stapler was justified by the absence of atherosclerotic aortic disease.
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Footnotes
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Address reprint requests to Dr Dartevelle, Department of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Hôpital Marie-Lannelongue, 133, Ave de la Resistance, 92350 Le Plessis Robinson, France.
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References
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- Georges JL, De Brux JL, Langlois J, et al. Les anomalies des arcs aortiques chez l'adulte. Une cause rare de compression trachéo-oesophagienne: a propos de deux cas. Ann Chir 1989;43:99–104.[Medline]
- Edwards JE. Anomalies of the derivatives of the aortic arch system. Med Clin North Am 1948;32:925–49.[Medline]
- Shuford WH, Sybers RG, Edwards FK. The three types of right aortic arch. AJR 1970;109:67–74.[Abstract]
- D'Souza VD, Velasquez G, Glass TA, Formanek AG. Mirror-image right aortic arch: a proposed mechanism in symptomatic vascular ring. Cardiovasc Intervent Radiol 1985;8:134–6.[Medline]
- Dartevelle P, Bouquet P, Planché C, Merlier M. Anévrysme athéromateux d'une artère sous-clavière droite aberrante. Chirurgie 1978;104:141–8.[Medline]
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Abstract
Introduction
