Digestive Diseases and Sciences, VoL 37, No. 1 (January 1992), pp. 144-149
CASE REPORT
Dysphagia Lusoria Caused by Persistent Right Aortic Arch with Aberrant Left Subclavian Artery and Diverticulum of Kommerell MAJOR PETER R. MCNALLY, DO, and MAJOR KEVIN M. RAK, MD KEY WORDS: aorta; esophagus;dysphagia;congenitalanomalies.
Developmental anomalies of the aortic arch and its major branches are relatively common, 3% by autopsy series (1), but they areusually asymptomatic and only confront the clinician as a roentgenographic curiosity. Aortic arch anomalies may become symptomatic when they completely "ring" the trachea and esophagus or when coincident congenital heart defects occur (1-3). Symptomatic aortic arch anomalies clinically present in a bimodal fashion (4). The trachea is compressible during infancy, and these patients typically present when solid foods are introduced, with respiratory symptoms: stridor, wheezing, cyanosis, or recurrent pneumonia (4). In adults, the esophagus is more likely to be comPressed, as the trachea is rigid, causing patients to present with esophageal dysphagia (5). There have been over a dozen different aortic arch anomalies reported to ring the esophagus and trachea and cause symptoms (1). In 1794, Dr. David Bayford described the autopsy findings of a woman with lifelong dysphagia who eventually died of starvation, because of esophageal obstruction (6). At autopsy, he found that a right subclavian Manuscript received October 3, 1990; revised manuscriPt received January 22, 1991; accepted January28, 1991. From the GastroenterologyService, Departmentof Medicine, and Departmentof Radiology,FitzsimonsArmyMedicalCenter, Aurora, Colorado, 80045-5001. The opinions and assertions containedherein are the private ones of the authors and are not to be construedas officialpolicy or reflecting the views of the Army or the Department of Defense. Address for reprint requests." Major Peter R. McNally, DO, FACP, GastroenterologyService,Departmentof Medicine,B!dg 403-W, Fitzsimons Army Medical Center, Aurora, Colorado 80045-5001. 144
artery passed aberrantly from the left aortic arch behind the esophagus---causing the woman's dysphagia. Dr. Bayford referred to this extraordinary disposition of the right subclavian artery as, lusus nature or sport or freak of nature. Since then the term dysphagia lusoria has been used to collectively refer to all aortic root anomalies causing esophageal dysphagia. We report a case of dysphagia lusoria caused by a right aortic arch with a large diverticulum of Kommerell and an aberrant left subclavian artery. Endoscopic and radiographic findings of this form of dysphagia lusoria are described, and the therapeutic alternatives are discussed. CASE REPORT
A 37-year-old female presented to the emergency room at Fort George Leonard Wood Community Hospital in April 1985 after an apparent 10-min episode of food impaction. During a hurried meal of hamburger and fried potatoes, the patient experienced food sticking at the level of the suprasternal notch. Before arrival at the emergency room, the patient relieved the acute dysphagia by repeated forced swallows of liquid. The patient was born in the Republic of South Korea and was unaware of respiratory or swallowing problems as an infant or a child. One year after emigrating to the United States (I977), the patient began to experience symptoms of heartburn and waterbrash that required self-administration of antacids for relief. About six months later the patient began to notice intermittent dysphagia to solids when meals were hurried. Episodes of dysphagia were never prolonged, always 20 mm Hg fall in pressure after arterial ligation, as debilitating symptoms of "steal" phenomenon are otherwise common (19-21). SUMMARY
It requires a high index of suspicion to make the diagnosis of dysphagia lusoria. Clinically, these adults will present with symptoms of intermittent solid food dysphagia, and a mediastinal abnormality may be seen on chest x-ray. Noninvasive imaging of the chest with either computerized tomography or magnetic resonance scanning are 148
excellent methods for evaluating the mediastinum for solid tumors or vascular anomalies that can cause extrinsic esophageal compression. Dysphagia lusoria caused by a persistence of the right embryologic aortic arch and diverticulum of Kommerell with an aberrant left subclavian artery may be satisfactorily managed by dietary modification when the symptoms are mild. ACKNOWLEDGMENTS To Mary Cord for preparation of illustrations used in this paper. REFERENCES 1. Shuford WH, Sybers RG: The Aortic Arch and its Malformations with Emphasis on the Angiographic Features. Springfield, Illinois, Charles C Thomas, 1974, pp 18-92 2. Klinkhamer AC: Aberrant right subclavian artery: Clinical and roentgenologic aspects. Am J Roentgenol 97:438-446, 1966 3. Edwards JE: Anomalies of the derivatives of the aortic arch system. Med Clin North Am 32:925-949, 1948 4. Lincoln JCR, Deverall PB, Stark J, Aberdeen E, Waterston DJ: Vascular anomalies compressing the esophagus and trachea. Thorax 24:295-306, 1969 5. Lichter I: Treatment of dysphagia lusoria in the adult. Br J Surgery 50:793-796, 1963 6. Asherson N: David Bayford: His syndrome and sign of dysphagia lusoria. Ann R Coil Surg Eng 61:63-67, 1979 7. Shannon JM: Aberrant right subclavian artery with Kommerell's diverticulum: Report of a case. J Thorac Cardiovasc Sur 41:408-411, 1961 8. Klinkenhamer AC: Esophagography in Anomalies of the Aortic Arch System. Baltimore, Williams and Wilkins, 1969, pp 16-30 Digestive Diseases and Sciences, Vol. 37, No. 1 (January 1992)
DYSPHAGIA L U S O R I A 9. Shuford WH, Sybers RG, Edwards FK: The three types of aortic arch. Am J Roentgenol 109:67-83, 1970 10. Berenzweig H, Baue AE, McCallum RW: Dysphagia lusoria: Report of a case and a review of the diagnostic and surgical approach. Dig Dis Sci 25:630-636, 1980 11. Palmer E: Dysphagia lusoria: Clinical aspects in the adult. Ann Intern Med 42:1173-1180, 1955 12. Rahiah FA: Dysphagia lusoria in adults. Int Surg 48:466469, 1967 13. Blackstone MO: Uncommon esophageal appearances. In Endoscopic Interpretation. New York, Raven Press, 1984, pp 76-77 14. Holinger PH, Schild JA: Anomalies of the esophagus. In Gastroenterology, 3rd ed. HL Bockus (ed). Philadelphia, WB Saunders, 1974, pp 183-190 15. Haaga JR, Alfidi RJ: Computed Tomography of the Whole Body. St Louis, CV Mosby, 1988, pp 586-648
Digestive Diseases and Sciences, Vol. 37, No. 1 (January 1992)
16. Lee JKT, Sagel SS, Stanley RJ: Computed Body Tomography with MRI correlation. New York, Raven Press, 1989, pp 223-225 17. Hahn D: Mediastinum and lung. In Magnetic Resonance Imaging. DD Stark, WG Bradley (eds). St Louis, CV Mosby, 1988, pp 804-833 18. Skinner DB and Belsey RHR: Management of Esophageal Disease. Philadelphia, WB Saunders, 1988, pp 378-385 19. Valentine JR, Carter DJ, Clagett GP: A modified extrathoracic approach to the treatment of dysphagia lusoria. J Vasc Surg 5:498-500, 1987 20. Karlson KJ, Heiss FW, Ellis FH: Adult dysphagia lusoria: Treatment by arterial division and re-establishment of vascular continuity. Chest 87:684-686, 1985 21. Leonardi HK, Naggar CZ, Ellis FH: Dysphagia due to aortic arch anomaly: Diagnostic and therapeutic considerations. Arch Surg 115:1229-1232, 1980
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CASE REPORT
Dysphagia Lusoria Caused by Persistent Right Aortic Arch with Aberrant Left Subclavian Artery and Diverticulum of Kommerell MAJOR PETER R. MCNALLY, DO, and MAJOR KEVIN M. RAK, MD KEY WORDS: aorta; esophagus;dysphagia;congenitalanomalies.
Developmental anomalies of the aortic arch and its major branches are relatively common, 3% by autopsy series (1), but they areusually asymptomatic and only confront the clinician as a roentgenographic curiosity. Aortic arch anomalies may become symptomatic when they completely "ring" the trachea and esophagus or when coincident congenital heart defects occur (1-3). Symptomatic aortic arch anomalies clinically present in a bimodal fashion (4). The trachea is compressible during infancy, and these patients typically present when solid foods are introduced, with respiratory symptoms: stridor, wheezing, cyanosis, or recurrent pneumonia (4). In adults, the esophagus is more likely to be comPressed, as the trachea is rigid, causing patients to present with esophageal dysphagia (5). There have been over a dozen different aortic arch anomalies reported to ring the esophagus and trachea and cause symptoms (1). In 1794, Dr. David Bayford described the autopsy findings of a woman with lifelong dysphagia who eventually died of starvation, because of esophageal obstruction (6). At autopsy, he found that a right subclavian Manuscript received October 3, 1990; revised manuscriPt received January 22, 1991; accepted January28, 1991. From the GastroenterologyService, Departmentof Medicine, and Departmentof Radiology,FitzsimonsArmyMedicalCenter, Aurora, Colorado, 80045-5001. The opinions and assertions containedherein are the private ones of the authors and are not to be construedas officialpolicy or reflecting the views of the Army or the Department of Defense. Address for reprint requests." Major Peter R. McNally, DO, FACP, GastroenterologyService,Departmentof Medicine,B!dg 403-W, Fitzsimons Army Medical Center, Aurora, Colorado 80045-5001. 144
artery passed aberrantly from the left aortic arch behind the esophagus---causing the woman's dysphagia. Dr. Bayford referred to this extraordinary disposition of the right subclavian artery as, lusus nature or sport or freak of nature. Since then the term dysphagia lusoria has been used to collectively refer to all aortic root anomalies causing esophageal dysphagia. We report a case of dysphagia lusoria caused by a right aortic arch with a large diverticulum of Kommerell and an aberrant left subclavian artery. Endoscopic and radiographic findings of this form of dysphagia lusoria are described, and the therapeutic alternatives are discussed. CASE REPORT
A 37-year-old female presented to the emergency room at Fort George Leonard Wood Community Hospital in April 1985 after an apparent 10-min episode of food impaction. During a hurried meal of hamburger and fried potatoes, the patient experienced food sticking at the level of the suprasternal notch. Before arrival at the emergency room, the patient relieved the acute dysphagia by repeated forced swallows of liquid. The patient was born in the Republic of South Korea and was unaware of respiratory or swallowing problems as an infant or a child. One year after emigrating to the United States (I977), the patient began to experience symptoms of heartburn and waterbrash that required self-administration of antacids for relief. About six months later the patient began to notice intermittent dysphagia to solids when meals were hurried. Episodes of dysphagia were never prolonged, always 20 mm Hg fall in pressure after arterial ligation, as debilitating symptoms of "steal" phenomenon are otherwise common (19-21). SUMMARY
It requires a high index of suspicion to make the diagnosis of dysphagia lusoria. Clinically, these adults will present with symptoms of intermittent solid food dysphagia, and a mediastinal abnormality may be seen on chest x-ray. Noninvasive imaging of the chest with either computerized tomography or magnetic resonance scanning are 148
excellent methods for evaluating the mediastinum for solid tumors or vascular anomalies that can cause extrinsic esophageal compression. Dysphagia lusoria caused by a persistence of the right embryologic aortic arch and diverticulum of Kommerell with an aberrant left subclavian artery may be satisfactorily managed by dietary modification when the symptoms are mild. ACKNOWLEDGMENTS To Mary Cord for preparation of illustrations used in this paper. REFERENCES 1. Shuford WH, Sybers RG: The Aortic Arch and its Malformations with Emphasis on the Angiographic Features. Springfield, Illinois, Charles C Thomas, 1974, pp 18-92 2. Klinkhamer AC: Aberrant right subclavian artery: Clinical and roentgenologic aspects. Am J Roentgenol 97:438-446, 1966 3. Edwards JE: Anomalies of the derivatives of the aortic arch system. Med Clin North Am 32:925-949, 1948 4. Lincoln JCR, Deverall PB, Stark J, Aberdeen E, Waterston DJ: Vascular anomalies compressing the esophagus and trachea. Thorax 24:295-306, 1969 5. Lichter I: Treatment of dysphagia lusoria in the adult. Br J Surgery 50:793-796, 1963 6. Asherson N: David Bayford: His syndrome and sign of dysphagia lusoria. Ann R Coil Surg Eng 61:63-67, 1979 7. Shannon JM: Aberrant right subclavian artery with Kommerell's diverticulum: Report of a case. J Thorac Cardiovasc Sur 41:408-411, 1961 8. Klinkenhamer AC: Esophagography in Anomalies of the Aortic Arch System. Baltimore, Williams and Wilkins, 1969, pp 16-30 Digestive Diseases and Sciences, Vol. 37, No. 1 (January 1992)
DYSPHAGIA L U S O R I A 9. Shuford WH, Sybers RG, Edwards FK: The three types of aortic arch. Am J Roentgenol 109:67-83, 1970 10. Berenzweig H, Baue AE, McCallum RW: Dysphagia lusoria: Report of a case and a review of the diagnostic and surgical approach. Dig Dis Sci 25:630-636, 1980 11. Palmer E: Dysphagia lusoria: Clinical aspects in the adult. Ann Intern Med 42:1173-1180, 1955 12. Rahiah FA: Dysphagia lusoria in adults. Int Surg 48:466469, 1967 13. Blackstone MO: Uncommon esophageal appearances. In Endoscopic Interpretation. New York, Raven Press, 1984, pp 76-77 14. Holinger PH, Schild JA: Anomalies of the esophagus. In Gastroenterology, 3rd ed. HL Bockus (ed). Philadelphia, WB Saunders, 1974, pp 183-190 15. Haaga JR, Alfidi RJ: Computed Tomography of the Whole Body. St Louis, CV Mosby, 1988, pp 586-648
Digestive Diseases and Sciences, Vol. 37, No. 1 (January 1992)
16. Lee JKT, Sagel SS, Stanley RJ: Computed Body Tomography with MRI correlation. New York, Raven Press, 1989, pp 223-225 17. Hahn D: Mediastinum and lung. In Magnetic Resonance Imaging. DD Stark, WG Bradley (eds). St Louis, CV Mosby, 1988, pp 804-833 18. Skinner DB and Belsey RHR: Management of Esophageal Disease. Philadelphia, WB Saunders, 1988, pp 378-385 19. Valentine JR, Carter DJ, Clagett GP: A modified extrathoracic approach to the treatment of dysphagia lusoria. J Vasc Surg 5:498-500, 1987 20. Karlson KJ, Heiss FW, Ellis FH: Adult dysphagia lusoria: Treatment by arterial division and re-establishment of vascular continuity. Chest 87:684-686, 1985 21. Leonardi HK, Naggar CZ, Ellis FH: Dysphagia due to aortic arch anomaly: Diagnostic and therapeutic considerations. Arch Surg 115:1229-1232, 1980
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