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Aberrant Right Subclavian Artery or Arteria Lusoria: A Rare Cause of Dyspnea in Children
Case Reports Aberrant Right Subclavian Artery or Arteria Lusoria: A Rare Cause of Dyspnea in Children Bilel Derbel, Achref Saaidi, Refk Kasraoui, Nazih Chaouch, Fatma Aouini, Nabil Ben Romdhane, and Jamelleddine Manaa, Tunis, Tunisie
Aberrant right subclavian artery or arteria lusoria (AL) is the most frequent anomaly of the aortic arch, secondary to abnormal embryogenesis. It is usually asymptomatic and fortuitously discovered. It can compress neighboring structures and cause dysphagia or, more rarely, dyspnea. In symptomatic cases or in the presence of an aneurysm of the AL origin, it should be surgically treated. The case herein reported concerns a child presenting with respiratory symptomatology related to an AL that was surgically treated by right supraclavicular approach.
Aberrant right subclavian artery (RSCA) or arteria lusoria (AL) is the most frequent anomaly of the aortic arch, with a 0.5% to 2.5%1 prevalence. Usually, this anomaly is asymptomatic, but it can induce dyspagia (dysphagia lusoria), dyspnea, and/ or chronic cough, thereby requiring surgery. When surgical treatment of an RSCA is advised, the approach has to be chosen (sternotomy, left lateral thoracotomy, and/or right supraclavicular cervicotomy).
CASE REPORT For several weeks, a 4-year-old child had been presenting with chronic cough associated with dyspnea during exercise. Clinical examination was normal. At the upper third part of the esophagus, an oeso-gastro-duodenal transit showed a print on the right side and a bayonet-shaped deviation of the esophagus (Fig. 1). Thoracic angioscan Service de Chirurgie Vasculaire Peripherique et de Transplantation d’Organes, Tunis, Tunisie. Correspondence to: Bilel Derbel, MD, Service de Chirurgie Vasculaire Peripherique et de Transplantation d’Organes, H^opital Militaire Principal D’Instruction de Tunis, Mont Fleury, 1008 Tunis, Tunisie; E-mail: [email protected] Ann Vasc Surg 2012; 26: 419.e1e419.e4 DOI: 10.1016/j.avsg.2011.09.007 Ó Annals of Vascular Surgery Inc. Published online: February 10, 2012
showed an RSCA originating from the downward thoracic artery as well as a common origin of the carotid arteries (Figs. 2e4). Owing to a right supraclavicular cervicotomy, the RSCA was dissected as far as the retroesophageal space (Fig. 5). A ligature of the upward stump close to the aortic arch was performed along with a laterolateral reimplantation of the downward stump of the RSCA on the right primitive artery (Fig. 6). Despite a meticulously carried out lymphostasis, a significant postoperative profuse lymphorrhea appeared on postoperative day 2, which was drained out through the suction drain and required surgical reoperation to perform secondary lymphostasis. After a 1-year followup, the evolution was uneventful, without any further cough or dyspnea. Angioscan showed RSCA patency (Fig. 7).
DISCUSSION AL was described for the first time in 1794 by Bayford.2 It results from an abnormal embryogenesis of the fourth aortic arch.3 Originating on the left and beyond the left subclavian artery, it follows an oblique course, upward and on the right toward the right supraclavicular fossa. It crosses the posterior side of the esophagus in more than 80% of the cases, the trachea and the esophagus in 15% of the cases, and the anterior part of the trachea in 5% of the cases.4 Most patients are asymptomatic (95%).5 419.e1
419.e2 Case reports
Annals of Vascular Surgery
Fig. 3. Thoracic angioscan: the right aberrant subclavian artey in arteria lusoria (arrow AL) at the level of the downward thoracic aorta and beyond the left subclavian artery (arrow). Note the common origin of the right and left primitive carotid arteries. Fig. 1. Oeso-gastro-duodenal transit showing the esophageal print (arrow), crossing site of the arterial anomaly.
Fig. 4. Thoracic angioscan: societal slice, esophageal compression by the arteria lusoria (arrow).
Fig. 2. Thoracic angioscan showing the retroesophageal artery corresponding to the anatomic variation of the arteria lusoria (arrow).
AL becomes symptomatic in three cases: (a) when the esophagus and trachea are situated between the AL at the back and a common origin of the two common carotid arteries at the front6,7
(as observed in our case), (b) when there is an AL aneurysm, and (c) in aging people, most likely when atherosclerotic disease and arterial fibromuscular dysplasia are developing.8 In children, respiratory symptoms are prevalent, mainly dyspnea or chronic coughing. They can also present with repetitive respiratory infections. In adults, the most frequent symptom is dysphagia. The lack of trachea rigidity associated to dysphagia
Vol. 26, No. 3, April 2012
Case reports 419.e3
Fig. 7. Follow-up thoracic angioscan: right subclavian carotid reimplantation is patent (arrow). Fig. 5. Aberrant right subclavian artery situated behind the esophagus. VJD, right jugular vein; RPCA, right common carotid artery.
Fig. 6. Anastomosis between the right subclavian artery (A) and the right common carotid artery (B).
and false routes are often held responsible for respiratory symptomatology in children. AL can also be revealed by the extension of aortic dissection9 or by peripheral arterial embolism.10 Oeso-gastro-duodenal transit shows an esophageal print at the third and fourth vertebrae level, which suggests the diagnosis; this will be confirmed by angiography or, even better, by angioscan. Magnetic resonance imaging can also help in visualizing a complication (associated aneurysm).
In symptomatic and aneurysmal forms, surgical treatment is advisable. In 1946, Gross was the first to treat this anomaly.11 It consisted in a simple RSCA ligature. However, with this treatment, ischemia and right upper-limb claudication cases were observed.12 In 1965, the first arterial restoration was performed by Bailey et al.,13 with a reimplantation of the RSCA distal part in the ascending aorta, close to the origin of the right common carotid artery. Other alternatives have been described. Cooley was the first to reimplant the RSCA distal part at the right primitive carotid artery level.14 In our case, a right supraclavicular approach was performed and enabled a good exposure, for the ligature, close to the aortic arch, of the retroesophageal portion and a right subclavianecarotid reimplantation with a lateroterminal anastomosis. It was described by Valentine et al. in 198715; it is a simplified version of the lower cervicotomy approach initially described by Orvald et al. in 1972,16 which requires an extensive dissection on each side of the esophagus before the ligature of the aberrant RSCA. Kieffer et al.1 noticed anatomic variations associated to aberrant RSCAdright vertebral artery originating directly at the level of the thoracic aorta or of the right primitive carotid artery; a nonrecurring laryngeal nerve; a common origin of the two carotid arteries; and a right thoracic canal. The latter two variations were found in our clinical case. It is important to be preoperatively familiar with them to preoperatively spare nervous and lymphatic elements. Pneumogastric and recurrent nerves must be identified and spared.
419.e4 Case reports
CONCLUSION AL is most often asymptomatic. When it is symptomatic without an aneurysm, surgical treatment by a right supraclavicular approach is advisable. This treatment includes a ligature of the RSCA, close to its origin in the aortic arch, and a right subclavianecarotid reimplantation. This surgical strategy requires a thorough knowledge of the anatomy of the supraclavicular region and of the associated vascular and nervous variations. REFERENCES 1. Kieffer E, Bahnini A, Koskas F. Aberrant subclavian artery: surgical treatment in thirty-three adult patients. J Vasc Surg 1994;19:100e11. 2. Bayford D. An account of a singular case of obstructed deglutition. Memoirs Med Soc London 1794;2:275e86. 3. Yopp A, Abrol S, Cunningham JN Jr, Lazzaro RS. Dysphagia lusoria and aberrant right subclavian artery. J Am Coll Surg 2006;202:198. 4. Miller JM, Miller KS. A note on the historical aspects of dysphagia lusoria. Am Surg 1992;58:502e3. 5. Ozcakar L, Kaymak B, Turan S, et al. Thoracic outlet syndrome. Paget-Schroetter syndrome and aberrant subclavian artery in a young man. Joint Bone Spine 2006;73:770e2. 6. van Son JA, Konstantinov IE, Kommerell BF. Kommerell’s diverticulum. Tex Heart Inst J 2002;29:109e12.
Annals of Vascular Surgery
7. Klinkhamer AC. Aberrant right subclavian artery. Clinical and roentgeno-logic aspects. Am J Roentgenol Radium Ther Nucl Med 1966;97:438e46. 8. Myers PO, Fasel JH, Kalangos A, Gailloud P. Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects. Ann Cardiol Angeiol 2010;59:147e54. 9. Coenegrachts K, Rigauts H, De Letter J, Meeus L. Traumatic pseudoaneurysm and dissection of the thoracic aorta in the presence of an aberrant right subclavian artery: value of multislice helical CT. Eur Radiol 2000;10:1750e3. 10. Vinsonneau U, Castellant P, Traore A, et al. Floating thrombus in an aberrant right subclavian artery: a rare cause of peripheral arterial embolic events [in French]. Rev Med Interne 2008;29:908e11. 11. Gross RE. Surgical treatment for dysphagia lusoria. Ann Surg 1946;124:532e4. 12. Mok CK, Cheung KL, Kong SM, Ong GB. Translocating the aberrant right subclavian artery in dysphagia lusoria. Br J Surg 1979;66:113e6. 13. Bailey CP, Hirose T, Alba J. Re-establishment of the continuity of the anomalous right subclavian artery after operation for dysphagia lusoria. Angiology 1965;16: 509e13. 14. Cooley DA. Surgical treatment of aortic aneurysms. Philadelphia, PA: WB Saunders, 1986. 15. Valentine RJ, Carter DJ, Clagget GP. A modified extrathoracic approach to the treatment of dysphagia lusoria. J Vasc Surg 1987;5:498e500. 16. Orvald TO, Scheerer R, Jude JR. A single cervical approach to aberrant right subclavian artery. Surgery 1972;71: 227e30.
Aberrant right subclavian artery or arteria lusoria (AL) is the most frequent anomaly of the aortic arch, secondary to abnormal embryogenesis. It is usually asymptomatic and fortuitously discovered. It can compress neighboring structures and cause dysphagia or, more rarely, dyspnea. In symptomatic cases or in the presence of an aneurysm of the AL origin, it should be surgically treated. The case herein reported concerns a child presenting with respiratory symptomatology related to an AL that was surgically treated by right supraclavicular approach.
Aberrant right subclavian artery (RSCA) or arteria lusoria (AL) is the most frequent anomaly of the aortic arch, with a 0.5% to 2.5%1 prevalence. Usually, this anomaly is asymptomatic, but it can induce dyspagia (dysphagia lusoria), dyspnea, and/ or chronic cough, thereby requiring surgery. When surgical treatment of an RSCA is advised, the approach has to be chosen (sternotomy, left lateral thoracotomy, and/or right supraclavicular cervicotomy).
CASE REPORT For several weeks, a 4-year-old child had been presenting with chronic cough associated with dyspnea during exercise. Clinical examination was normal. At the upper third part of the esophagus, an oeso-gastro-duodenal transit showed a print on the right side and a bayonet-shaped deviation of the esophagus (Fig. 1). Thoracic angioscan Service de Chirurgie Vasculaire Peripherique et de Transplantation d’Organes, Tunis, Tunisie. Correspondence to: Bilel Derbel, MD, Service de Chirurgie Vasculaire Peripherique et de Transplantation d’Organes, H^opital Militaire Principal D’Instruction de Tunis, Mont Fleury, 1008 Tunis, Tunisie; E-mail: [email protected] Ann Vasc Surg 2012; 26: 419.e1e419.e4 DOI: 10.1016/j.avsg.2011.09.007 Ó Annals of Vascular Surgery Inc. Published online: February 10, 2012
showed an RSCA originating from the downward thoracic artery as well as a common origin of the carotid arteries (Figs. 2e4). Owing to a right supraclavicular cervicotomy, the RSCA was dissected as far as the retroesophageal space (Fig. 5). A ligature of the upward stump close to the aortic arch was performed along with a laterolateral reimplantation of the downward stump of the RSCA on the right primitive artery (Fig. 6). Despite a meticulously carried out lymphostasis, a significant postoperative profuse lymphorrhea appeared on postoperative day 2, which was drained out through the suction drain and required surgical reoperation to perform secondary lymphostasis. After a 1-year followup, the evolution was uneventful, without any further cough or dyspnea. Angioscan showed RSCA patency (Fig. 7).
DISCUSSION AL was described for the first time in 1794 by Bayford.2 It results from an abnormal embryogenesis of the fourth aortic arch.3 Originating on the left and beyond the left subclavian artery, it follows an oblique course, upward and on the right toward the right supraclavicular fossa. It crosses the posterior side of the esophagus in more than 80% of the cases, the trachea and the esophagus in 15% of the cases, and the anterior part of the trachea in 5% of the cases.4 Most patients are asymptomatic (95%).5 419.e1
419.e2 Case reports
Annals of Vascular Surgery
Fig. 3. Thoracic angioscan: the right aberrant subclavian artey in arteria lusoria (arrow AL) at the level of the downward thoracic aorta and beyond the left subclavian artery (arrow). Note the common origin of the right and left primitive carotid arteries. Fig. 1. Oeso-gastro-duodenal transit showing the esophageal print (arrow), crossing site of the arterial anomaly.
Fig. 4. Thoracic angioscan: societal slice, esophageal compression by the arteria lusoria (arrow).
Fig. 2. Thoracic angioscan showing the retroesophageal artery corresponding to the anatomic variation of the arteria lusoria (arrow).
AL becomes symptomatic in three cases: (a) when the esophagus and trachea are situated between the AL at the back and a common origin of the two common carotid arteries at the front6,7
(as observed in our case), (b) when there is an AL aneurysm, and (c) in aging people, most likely when atherosclerotic disease and arterial fibromuscular dysplasia are developing.8 In children, respiratory symptoms are prevalent, mainly dyspnea or chronic coughing. They can also present with repetitive respiratory infections. In adults, the most frequent symptom is dysphagia. The lack of trachea rigidity associated to dysphagia
Vol. 26, No. 3, April 2012
Case reports 419.e3
Fig. 7. Follow-up thoracic angioscan: right subclavian carotid reimplantation is patent (arrow). Fig. 5. Aberrant right subclavian artery situated behind the esophagus. VJD, right jugular vein; RPCA, right common carotid artery.
Fig. 6. Anastomosis between the right subclavian artery (A) and the right common carotid artery (B).
and false routes are often held responsible for respiratory symptomatology in children. AL can also be revealed by the extension of aortic dissection9 or by peripheral arterial embolism.10 Oeso-gastro-duodenal transit shows an esophageal print at the third and fourth vertebrae level, which suggests the diagnosis; this will be confirmed by angiography or, even better, by angioscan. Magnetic resonance imaging can also help in visualizing a complication (associated aneurysm).
In symptomatic and aneurysmal forms, surgical treatment is advisable. In 1946, Gross was the first to treat this anomaly.11 It consisted in a simple RSCA ligature. However, with this treatment, ischemia and right upper-limb claudication cases were observed.12 In 1965, the first arterial restoration was performed by Bailey et al.,13 with a reimplantation of the RSCA distal part in the ascending aorta, close to the origin of the right common carotid artery. Other alternatives have been described. Cooley was the first to reimplant the RSCA distal part at the right primitive carotid artery level.14 In our case, a right supraclavicular approach was performed and enabled a good exposure, for the ligature, close to the aortic arch, of the retroesophageal portion and a right subclavianecarotid reimplantation with a lateroterminal anastomosis. It was described by Valentine et al. in 198715; it is a simplified version of the lower cervicotomy approach initially described by Orvald et al. in 1972,16 which requires an extensive dissection on each side of the esophagus before the ligature of the aberrant RSCA. Kieffer et al.1 noticed anatomic variations associated to aberrant RSCAdright vertebral artery originating directly at the level of the thoracic aorta or of the right primitive carotid artery; a nonrecurring laryngeal nerve; a common origin of the two carotid arteries; and a right thoracic canal. The latter two variations were found in our clinical case. It is important to be preoperatively familiar with them to preoperatively spare nervous and lymphatic elements. Pneumogastric and recurrent nerves must be identified and spared.
419.e4 Case reports
CONCLUSION AL is most often asymptomatic. When it is symptomatic without an aneurysm, surgical treatment by a right supraclavicular approach is advisable. This treatment includes a ligature of the RSCA, close to its origin in the aortic arch, and a right subclavianecarotid reimplantation. This surgical strategy requires a thorough knowledge of the anatomy of the supraclavicular region and of the associated vascular and nervous variations. REFERENCES 1. Kieffer E, Bahnini A, Koskas F. Aberrant subclavian artery: surgical treatment in thirty-three adult patients. J Vasc Surg 1994;19:100e11. 2. Bayford D. An account of a singular case of obstructed deglutition. Memoirs Med Soc London 1794;2:275e86. 3. Yopp A, Abrol S, Cunningham JN Jr, Lazzaro RS. Dysphagia lusoria and aberrant right subclavian artery. J Am Coll Surg 2006;202:198. 4. Miller JM, Miller KS. A note on the historical aspects of dysphagia lusoria. Am Surg 1992;58:502e3. 5. Ozcakar L, Kaymak B, Turan S, et al. Thoracic outlet syndrome. Paget-Schroetter syndrome and aberrant subclavian artery in a young man. Joint Bone Spine 2006;73:770e2. 6. van Son JA, Konstantinov IE, Kommerell BF. Kommerell’s diverticulum. Tex Heart Inst J 2002;29:109e12.
Annals of Vascular Surgery
7. Klinkhamer AC. Aberrant right subclavian artery. Clinical and roentgeno-logic aspects. Am J Roentgenol Radium Ther Nucl Med 1966;97:438e46. 8. Myers PO, Fasel JH, Kalangos A, Gailloud P. Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects. Ann Cardiol Angeiol 2010;59:147e54. 9. Coenegrachts K, Rigauts H, De Letter J, Meeus L. Traumatic pseudoaneurysm and dissection of the thoracic aorta in the presence of an aberrant right subclavian artery: value of multislice helical CT. Eur Radiol 2000;10:1750e3. 10. Vinsonneau U, Castellant P, Traore A, et al. Floating thrombus in an aberrant right subclavian artery: a rare cause of peripheral arterial embolic events [in French]. Rev Med Interne 2008;29:908e11. 11. Gross RE. Surgical treatment for dysphagia lusoria. Ann Surg 1946;124:532e4. 12. Mok CK, Cheung KL, Kong SM, Ong GB. Translocating the aberrant right subclavian artery in dysphagia lusoria. Br J Surg 1979;66:113e6. 13. Bailey CP, Hirose T, Alba J. Re-establishment of the continuity of the anomalous right subclavian artery after operation for dysphagia lusoria. Angiology 1965;16: 509e13. 14. Cooley DA. Surgical treatment of aortic aneurysms. Philadelphia, PA: WB Saunders, 1986. 15. Valentine RJ, Carter DJ, Clagget GP. A modified extrathoracic approach to the treatment of dysphagia lusoria. J Vasc Surg 1987;5:498e500. 16. Orvald TO, Scheerer R, Jude JR. A single cervical approach to aberrant right subclavian artery. Surgery 1972;71: 227e30.