Case Report Aneurysm of an Aberrant Right Subclavian Artery: Case Report and Review of the Literature PAUL D. KIERNAN, M.D., JOSEPH DEARANI, M.D., WILLIAM D. BYRNE, M.D., THOMAS EHRLICH, M.D., WILLIAM CARTER, M.D., GARY KRASICKY, M.D., AND WILLIAM HARSHAW, M.D. In this article, we describe a case of a surgically treated aneurysm of an aberrant right subclavian artery. The historical literature to date is summarized, as are the key concepts relative to the anatomy, embryology, diagnosis, and treatment of this uncommonly occurring entity. Although the topic might be expected to be of concern to only a few specialists, all physicians should be aware that a patient with an enlarging aneurysm of an aberrant subclavian artery may experience dyspnea, dysphagia, or sudden collapse from rupture as the initial manifestations. An asymptomatic patient may have a mediastinal mass detected by roentgenography. The diagnosis may be confirmed with computed tomography or magnetic resonance imaging. As with most aneurysms, surgical treatment is recom- mended, and the benefit-to-risk analysis depends on individual case factors. HISTORY In 1735, Hunauld' first reported dysphagia attributed to an aberrant right subclavian artery. Subsequently, Bayford/ defined the anatomic features and coined the term "dysphagia lusoria" (dysphagia due to a freak of nature). McCallen and Schaff first described aneurysmal change in such a vessel 20 years after Kommerell' highlighted the existence of a diverticulum at the origin of some aberrant right subclavian arteries. Although the incidence of aberrant right subclavian artery is reported to be between 0.5 and 1.0%,5 aneurysms of such arteries are uncommon. A review of the literature in 1985 yielded 32 cases, only 20 of which were treated surgically." Because of the relative paucity of such cases, we describe herein a surgically treated aneurysm of an aberrant right subclavian artery. Important embryologic, diagnostic, and From the Section of Thoracic Surgery (P.D.K., W.D.B.), Section of Family Practice Medicine (T.E., W.c.), and Section of Radiology (G.K., W.H.), Fairfax Hospital, INOVA Health Care Systems, Annandale, Virginia, and Department of General Surgery (J.D.), Georgetown University Medical Center, Washington, DC. Address reprint requests to Dr. P. D. Kiernan, Section of Thoracic Surgery, Fairfax Hospital, Suite 210, 3301 Woodburn Road, Annandale, VA 22003. therapeutic facets are reviewed as well as the English-lan- guage literature on the subject to date. EMBRYOLOGY AND ANATOMY On the basis of the conceptual embryologic double aortic arch reported by Stewart, Kincaid, and Edwards," left- and right-sided aortic arches and respective aberrant right and left subclavian arteries are easily explained and visualized. Numerous other variations and anomalies are also clear- ly explained, dependent on the site of arch agenesis or regression. Moreover, incomplete arch agenesis clarifies the origin of Kommerell's diverticulum, as noted in up to 60% of cases." When aberrant subclavian arteries occur, they arise as the last brachiocephalic branch off the proximal descending aorta. The site of origin is usually the posteromedial aspect of the descending aorta (Fig. 1),9 Investigators currently believe that such vessels are almost always posterior to the esophagus, where they course in a caudad to cephalad man- ner and create a characteristic retroesophageal indentation on esophagograrns.'? The ipsilateral recurrent laryngeal nerve, which has no embryologic vessels to negotiate around, courses directly to the larynx. The anatomic site of the thoracic duct may similarly be anomalous; often, it Mayo Clin Proc 1993; 68:468-474 468 © 1993 Mayo Foundation for Medical Education and Research
Aneurysm of an Aberrant Right Subclavian Artery: Case Report and Review of the Literature
Dec 07, 2022
Welcome message from author
This document is posted to help you gain knowledge. Please leave a comment to let me know what you think about it! Share it to your friends and learn new things together.
Transcript
Aneurysm of an Aberrant Right Subclavian Artery: Case Report and Review of the LiteratureCase Report
Aneurysm of an Aberrant Right Subclavian Artery: Case Report and Review of the Literature
PAUL D. KIERNAN, M.D., JOSEPH DEARANI, M.D., WILLIAM D. BYRNE, M.D., THOMAS EHRLICH, M.D.,
WILLIAM CARTER, M.D., GARY KRASICKY, M.D., AND WILLIAM HARSHAW, M.D.
In this article, we describe a case of a surgically treated aneurysm of an aberrant right subclavian artery. The historical literature to date is summarized, as are the key concepts relative to the anatomy, embryology, diagnosis, and treatment of this uncommonly occurring entity. Although the topic might be expected to be of concern to only a few specialists, all physicians should be aware that a patient with an enlarging aneurysm of an aberrant subclavian artery may experience dyspnea, dysphagia, or sudden collapse from rupture as the initial manifestations. An asymptomatic patient may have a mediastinal mass detected by roentgenography. The diagnosis may be confirmed with computed tomography or magnetic resonance imaging. As with most aneurysms, surgical treatment is recom mended, and the benefit-to-risk analysis depends on individual case factors.
HISTORY In 1735, Hunauld' first reported dysphagia attributed to an aberrant right subclavian artery. Subsequently, Bayford/ defined the anatomic features and coined the term "dysphagia lusoria" (dysphagia due to a freak of nature). McCallen and Schaff first described aneurysmal change in such a vessel 20 years after Kommerell' highlighted the existence of a diverticulum at the origin of some aberrant right subclavian arteries.
Although the incidence of aberrant right subclavian artery is reported to be between 0.5 and 1.0%,5 aneurysms of such arteries are uncommon. A review of the literature in 1985 yielded 32 cases, only 20 of which were treated surgically." Because of the relative paucity of such cases, we describe herein a surgically treated aneurysm of an aberrant right subclavian artery. Important embryologic, diagnostic, and
From the Section of Thoracic Surgery (P.D.K., W.D.B.), Section of Family Practice Medicine (T.E., W.c.), and Section of Radiology (G.K., W.H.), Fairfax Hospital, INOVA Health Care Systems, Annandale, Virginia, and Department of General Surgery (J.D.), Georgetown University Medical Center, Washington, DC.
Address reprint requests to Dr. P. D. Kiernan, Section of Thoracic Surgery, Fairfax Hospital, Suite 210, 3301 Woodburn Road, Annandale, VA 22003.
therapeutic facets are reviewed as well as the English-lan guage literature on the subject to date.
EMBRYOLOGY AND ANATOMY On the basis of the conceptual embryologic double aortic arch reported by Stewart, Kincaid, and Edwards," left- and right-sided aortic arches and respective aberrant right and left subclavian arteries are easily explained and visualized. Numerous other variations and anomalies are also clear ly explained, dependent on the site of arch agenesis or regression. Moreover, incomplete arch agenesis clarifies the origin of Kommerell's diverticulum, as noted in up to 60% of cases."
When aberrant subclavian arteries occur, they arise as the last brachiocephalic branch off the proximal descending aorta. The site of origin is usually the posteromedial aspect of the descending aorta (Fig. 1),9 Investigators currently believe that such vessels are almost always posterior to the esophagus, where they course in a caudad to cephalad man ner and create a characteristic retroesophageal indentation on esophagograrns.'? The ipsilateral recurrent laryngeal nerve, which has no embryologic vessels to negotiate around, courses directly to the larynx. The anatomic site of the thoracic duct may similarly be anomalous; often, it
Mayo Clin Proc 1993; 68:468-474 468 © 1993 Mayo Foundation for Medical Education and Research
Mayo Clin Proc, May 1993, Vol 68
Esophagus
Left subclavian
Fig. I. Diagram (superior view) of left aortic arch and anomalous right subclavian artery. (Redrawn from Grant.")
passes through the mediastinum to the right of its usual position. I I
REPORT OF CASE A right superior mediastinal shadow (Fig. 2), noted within the retrotracheal triangle" on a chest roentgenogram ob tained before a planned cholecystectomy, was the first clue to diagnosis in this otherwise asymptomatic 54-year-old man. The patient denied having dysphagia, and a roentgeno gram of the upper aspect of the gastrointestinal tract showed norma) findings. Computed tomograms of the chest (Fig. 3) obtained with use of contrast medium revealed a uniformly
ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY 469
enhancing retroesophageal mass contiguous with the aortic arch, which ascended along the expected course of the sub clavian artery. A 6-cm-diameter aneurysm of the aberrant right subclavian artery was diagnosed. No other thoracic aortic aneurysm was detected, and follow-up abdominal ultrasonography disclosed normal caliber of the abdominal aorta.
The patient was then referred for surgical consultation. Angiography was performed to confirm the diagnosis as well as to plan optimal exposure for control of the vessel off the descending thoracic aorta and for revascularization of the right upper extremity. With demonstration of the site of origin off the posteromedial aspect of the descending tho racic aorta (Fig. 4), we heeded the advice of a surgeon (Bematz PE. Personal communication) who previously had performed exposure of a similar vessel by means of a left thoracotomy and had concluded that a right-sided approach would likely be superior.
A right posterolateral thoracotomy, through the unresected fifth rib, provided optimal exposure (Fig. 5) of the descending aorta, the proximal Komrnerell diverticu lum as it merged into the aneurysm of the aberrant right subclavian artery, and the distal right subclavian artery where it coursed over the right first rib. The descending aorta was partially cross-clamped, and the distal subclavian artery was cross-clamped by means of a counterincision at the second intercostal space. The aneurysm sac was opened, the thrombus (Fig. 5 insert) was removed, and the caliber of the right subclavian artery was normal at the level of the first rib. Kommerell's diverticulum was oversewn
Fig.2. Chest roentgenograms of 54-year-old man obtained some years before development of aneurysm of aberrant right subclavian artery (Al and at time of diagnosis (B).
470 ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY Mayo Clio Proc, May 1993, Vol 68
Fig. 3. A and B, Computed tomograms of chest of 54-year-old man, showing origin of aneurysm of aberrant right subclavian artery arising from descending thoracic aorta.
with use of large pledgets and 3-0 polypropylene suture material.
Although a bypass from the ascending aorta to the right subclavian artery had been planned, an 8-mm woven Dacron graft was attached to the descending aorta, distal to the oversewn diverticulum (Fig. 6). Access to the .descending aorta was easier than to the ascending aorta. We originated the graft directly off the descending aorta rather than from the Kommerell diverticulum to minimize the likelihood of confusion with formation of a pseudoaneurysm during any subsequent investigations.
With excellent hemostasis, double ligation of two acces sory lymph ducts, and strong digital pulses achieved in the right upper extremity, the graft was separated from the adja cent esophagus and lung by the aneurysm sac and a mobi lized pleural flap. After an uncomplicated convalescence, the patient was dismissed on the sixth postoperative day.
REVIEW OF THE LITERATURE In 1985, Austin and Wolfe? published a case report and a review of the literature in which 31 of 32 patients had a left aortic arch. One patient had a right aortic arch and an aneurysm of an aberrant left subclavian artery. Of the 32 pa tients, 20 underwent a surgical procedure. Ten patients underwent left thoracotomy, all but two without reconstruc tion of the subclavian artery. The related mortality was 11% (one of nine patients for whom survival data were reported).
No deaths occurred among the six patients in Austin and Wolfe's review who underwent right thoracotomy. Of these patients, three underwent revascularization. In two other patients, a median sternotomy was used for exploration; neither patient underwent revascularization, and both died. Finally, two other patients underwent surgical treatment, but neither the exposure nor the facts relevant to re vascularization were described.
For 5 of the 12 nonoperative patients in Austin and Wolfe's series, information about the incidence of rupture and survival was lacking. Of the other seven patients, only two survived; five (71%) died of arterial rupture-four of rupture of the aneurysms of the subclavian artery and one of a ruptured abdominal aortic aneurysm.
Fig. 4. Aortogram of 54-year-old man, illustrating aneurysm of aberrant right subclavian artery emanating from a Kommerell di verticulum off descending thoracic aorta, without visualization of ipsilateral vertebral artery. Normal caliber of right subclavian artery is shown distally.
Mayo Clin Proc, May 1993, Vol 68
Esophagus
Descending aorta
Fig. 5. Diagram depicting method of cross-clamping by using partial occlusion clamp across origin of Kommerell's diverticulum off descending thoracic aorta and cross-clamping of right subcla vian artery through second intercostal space. Insert depicts throm bus retrieved from aneurysm. a. = artery; v. =vein.
An additional 21 cases have been culled from the English language literature during the 8 years since Austin and Wolfe's report, and 1 as yet unpublished case has been described to us (Cherry KJ, Bower TC. Personal communi cation) (Table 1). Of these 22 patients, 14 (64%) underwent surgical repair, 3 because of rupture. Although those three patients died, all other surgically treated patients for whom mortality data were reported have survived.
The mortality rates relative to operative and medical man agement for the cases reported to date are summarized in Table 2. The comparison of rates relative to type of opera tive exposure is included. Despite lack of longitudinal fol low-up, the risk of rupture of an aneurysm-of either the subclavian artery or another artery-in nonoperative patients exceeded 30% (at least 6 of 18 patients) (Table 1). This figure emphasizes the substantial risk of rupture of large aneurysms of aberrant subclavian arteries.
DISCUSSION Although the embryologic, anatomic, and diagnostic details of aneurysms of an aberrant right subclavian artery were discussed earlier in this report, a few additional comments seem appropriate. As in the current case, barium studies of the upper part of the gastrointestinal tract may not elicit a complete esophagogram. Undoubtedly, a complete esoph agogram would have illustrated the classic oblique retro esophageal indentation.
Computed tomography (or magnetic resonance imaging) is probably the best means of determining the presence of an
ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY 471
aneurysm, particularly in distinguishing the normal widened lumen of a Kommerell diverticulum from an actual aneu rysm. Arteriography is no substitute in this regard," al though we think that angiography is appropriate in preparing for surgical treatment.
As noted in our review of the literature, rupture of an aneurysm of an aberrant subclavian artery is as substantial a threat as rupture of any other aneurysm. 51 Thus, we strongly disagree with those investigators" who advise that only pa tients with symptomatic aneurysms or those in whom rupture seems imminent should be candidates for surgical treatment. Moreover, up to 25% of patients with aneurysms of an aberrant right subclavian artery harbor another aneurysm or aneurysms," and such patients are a high-risk group.
In past reports, the advisability of revascularization of surgically treated aneurysms of aberrant subclavian arteries has been debated; however, contemporary discussion relates more to the "how" than to the "whether"-because of a report" of an unacceptably high incidence of ischemia in those patients who do not undergo revascularization. Vari ous technical options are appropriate for the exposure cho sen.20,37,52 We favor repair by means of a thoracotomy ipsi lateral to the aneurysm, as was originally reported by Campbell" and subsequently advocated by Austin and Wolfe." With this approach, the closure of the site of aortic origin is easily exposed in light of the embryologic and
Fig. 6. Diagram depicting Dacron graft reconstruction of descend ing aorta to right subclavian artery. Kommerell's diverticulum is oversewn with pledgets.
472 ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY Mayo Clin Proc, May 1993, Vol 68
Table I.-Outcome of Patients After Medical or Surgical Treatment of Aneurysms of Aberrant Subclavian Arteries*
Revascu- Limb Case Operation Approach Survived larization ischemia Comment Reference
No Yes Died 21h yr later, 3 ruptured AAA
2 No No Rupture 13 3 Yes L thor No No ND 14 4 Yes Lthor Yes No ND 15 5 No No Rupture 16 6 Yes L thor Yes No ND 17 7 Yes Median' No No ND Rupture 18 8 Yes Median No No ND 19 9 Yes Lthor Yes No ND 19
10 Yes L thor Yes No ND 19 II Yes R thor Yes Yes ND 20 12 Yes ND No ND ND 21 13 No Yes 21 14 Yes R thor Yes No Yes 22 15 Yes Lthor No ND 23 16 No Yes 24 17 No No Rupture 25 18 Yes Lthor Yes Yes ND 26 19 No No Rupture 27 20 No No 28 21 Yes ND No ND ND Rupture 28 22 No No 28 23 No No 28 24 ND ND ND ND ND 29 25 Yes R thor Yes No ND 30 26 Yes Lthor Yes No ND 31 27 Yes R thor Yes Yes ND 32 28 Yes L thor Yes No ND 33 29 Yes R thor Yes No ND 34 30 No ND 35 31 Yes Lthor Yes Yes ND 36 32 Yes R thor Yes Yes ND 6 33 Yes Median Yes Yes No 37 34 No ND 38 35 Yes L thor Yes Yes ND 39 36 Yes ND No Yes ND Rupture 40 37 No ND 41 38 ND ND ND ND ND 42 39 No ND 43 40 Yes L thor Yes Yes No 44 41 Yes Median Yes No No 45 42 Yes R thor, Yes No No 46
median 43 Yes Median Yes Yes No 46 44 Yes Median Yes Yes No 47 45 No No Rupture 48 46 Yes ND ND ND ND 49 47 No ND ND 49 48 No ND ND 49 49 No ND ND 49 50 Yes L thor No Yes No Rupture 50 51 Yes Lthor No ND ND Rupture 51t 52 Yes Lthor Yes Yes No 51 53 Yes R thor Yes Yes No Current case 54 Yes L thor Yes Yes No :j:
*AAA =abdominal aortic aneurysm; L =left; median =median sternotomy; ND =not described; R =right; thor =thoracotomy.
tThe third case of this series had been published previously by Gomes and associates. I?
:j:Cherry KJ, Bower TC. Personal communication.
MayoClin Proc, May 1993, Vol68
Table 2.-Mortality Related to Management of Aneurysms of Aberrant Subclavian Arteries
Type of No. of Mortality management patients No. %
Surgical 34 8 24 Left thoracotomy 16 3 19 Right thoracotomy 8 0 0 Median sternotomy 6 2 33 Not described 4 3 75
Medical 18 8 44 Not described 2
Total 54 16 30
anatomic features as herein reviewed. Moreover, re vascularization necessitates no separate incision and may originate off the descending or the ascending aorta. Finally, our review of the literature seems to substantiate the safety and efficacy of such an approach.
CONCLUSION The methods for diagnosing aneurysms of an aberrant sub clavian artery have become less invasive and more sophisti cated. With increased use of computed tomography and magnetic resonance imaging techniques, these uncommon aneurysms will likely be diagnosed earlier and more fre quently than in the past. Earlier diagnosis should allow more timely surgical intervention before complications occur. Al though various surgical strategies have been devised, repair by means of a thoracotomy ipsilateral to the aneurysm offers advantages for exposure and safety as well as the capability of accomplishing revascularization easily through the same approach.
REFERENCES I. Hunauld M. Examen de quelques parties d'un singe. Hist
Acad Roy Sci 1735; 2:516-523 2. Bayford D. An account of a singular cause of obstructed
deglutition. Mem Med Soc Lond 1794; 2:275-286 3. McCallen AM, Schaff B. Aneurysm of an anomalous right
subclavian artery. Radiology 1956; 66:561-563 4. Kommerell B. Verlagerung des Osophagus durch eine
abnorm verlaufende Arteria subclavia dextra (Arteria lusoria). Fortschr Geb Rontgenstr 1936; 54:590-595
5. McDonald JJ, Anson BJ. Variations in the origin of arteries derived from the aortic arch, in American whites and negroes. Am J Phys Anthropol 1940; 27:91-107
6. Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of the literature. J Vase Surg 1985; 2:571-577
7. Stewart JR, Kincaid OW, Edwards IE. An Atlas of Vascular Rings and Related Malformations of the Aortic Arch System. Springfield (IL): Charles C Thomas, 1964
ANEURYSM OFABERRANT RIGHT SUBCLAVIAN ARTERY 473
8. Salomonowitz E, Edwards JE, Hunter DW, Castaneda Zuniga WR, Lund G, Cragg AH, et al. The three types of aortic diverticula. AJR Am J Roentgenol 1984; 142:673 679
9. Grant JCB. An Atlas of Anatomy. 5th ed. Baltimore: Williams & Wilkins, 1962
10. Beabout JW, Stewart JR, Kincaid OW. Aberrant right sub clavian artery: dispute of commonly accepted concepts. AJR Am J Roentgenol 1964; 92:855-864
11. Raider L. Aberrant right subclavian artery. South Med J 1967; 60:145-151
12. Raider L. The retrotracheal triangle. Chest 1973; 63:835 838
13. Richards WCD, Elliott CEo Aneurysm of an anomalous right subclavian artery. Br Heart J 1957; 19:141-143
14. Shannon JM. Aberrant right subclavian artery with Kommerell's diverticulum: report of a case. J Thorac Cardiovasc Surg 1961; 41:408-411
15. Candardjis G, Desbaillets P, Saegesser F. Coarctation simultanee de l' aorte et d'une arteria lusoria avec anevrisme, Radiol Clin (Basel) 1961; 30:308-322
16. Ryan JA. An unusual case of traumatic mediastinal aneurysm in a closed chest injury. Br J Surg 1962; 50:210-218
17. Gomes MMR, Bernatz PE, Forth RJ. Arteriosclerotic aneu rysm of an aberrant right subclavian artery. Dis Chest 1968; 54:549-552
18. Lynn RB. Kommerell's diverticulum with esophago-arterial fistula. Can J Surg 1969; 12:331-333
19. Hunter JA, Dye WS, Javid H, Najafi H, Julian Oc. Arterio sclerotic aneurysm of anomalous right subclavian artery. J Thorac Cardiovasc Surg 1970; 59:754-758
20. Campbell CF. Repair of an aneurysm of an aberrant retroesophageal right subclavian artery arising from Kommerell's diverticulum. J Thorac Cardiovasc Surg 1971; 62:330-334
21. Engelman RM, Madayag M. Aberrant right subclavian artery aneurysm: a rare cause of a superior mediastinal tumor. Chest 1972; 62:45-47
22. Sarot IA. Discussion of Engelman RM, Madayag M, Spencer FC. Aneurysm of aberrant right subclavian artery: rare cause of superior mediastinal tumor. N Y State J Med 1973; 73:290-292
23. Sakurai H, Lukban S, Litwak RS. Coarctation of aorta asso ciated with anomalous right subclavian artery and its aneu rysm. NY State J Med 1973; 73:292-296
24. Lui AHF. Aneurysm of anomalous right subclavian artery: report of a case. Am Surg 1973; 39:187-190
25. Dikman SH, Baron M, Gordon AJ. Right aortic arch with ruptured aneurysm of anomalous left subclavian artery. Am J Cardiol 1974; 34:245-249
26. Stoney WS, Alford WC Jr, Burrus GR, Thomas CS Jf. Aber rant right subclavian artery aneurysm. Ann Thorac Surg 1975; 19:460-467
27. Buades Reynes J, Aguirre Errasti C, Bilboa Ercoreca FJ, Onate Landa A. Aneurysm of aberrant right subclavian ar tery with esophageal perforation [letter]. Chest 1976; 70: 105
28. Wagner T, Thiry S. Aneurysma der Arteria 1usoria: Mitteilung von vier Fallen. Schweiz Med Wochenschr 1976; 106:15-21
29. Cunningham Gc. Radiologic case presentation. Cardiovasc Dis 1977; 4:4-6
474 ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY
30. Rodgers BM, Talbert JL, Hollenbeck JI. Aneurysm of anomalous subclavian artery: an unusual cause of dysphagia lusoria in childhood. Ann Surg 1978; 187:158-160
31. Dartevelle P, Bouquet P, Planche C, Merlier M. Anevrysme atheromateux d'une artere sous-claviere droite aberrante: a propos d'un cas revele par une dysphagie et opere chez une femme de 73 ans, Chirurgie 1978; 104:141-147
32. Jourdan J, Schmitt R, Kreitman P, Hatchuel P, Blaive B, Dor V. Anevrysme sur arteria lusoria: aspects cliniques, diagnostiques; resultats post-operatoires apres resection et retablissernent de la continuite arterielle. Arch Mal Coeur 1980; 73:221-226
33. Schmidt FE, Hewitt RL, Flores AA Jr. Aneurysm of anoma lous right subclavian artery. South Med J 1980; 73:255-256
34. McIntyre MD, Lynn RB. Kommerell's diverticulum. Can…
Aneurysm of an Aberrant Right Subclavian Artery: Case Report and Review of the Literature
PAUL D. KIERNAN, M.D., JOSEPH DEARANI, M.D., WILLIAM D. BYRNE, M.D., THOMAS EHRLICH, M.D.,
WILLIAM CARTER, M.D., GARY KRASICKY, M.D., AND WILLIAM HARSHAW, M.D.
In this article, we describe a case of a surgically treated aneurysm of an aberrant right subclavian artery. The historical literature to date is summarized, as are the key concepts relative to the anatomy, embryology, diagnosis, and treatment of this uncommonly occurring entity. Although the topic might be expected to be of concern to only a few specialists, all physicians should be aware that a patient with an enlarging aneurysm of an aberrant subclavian artery may experience dyspnea, dysphagia, or sudden collapse from rupture as the initial manifestations. An asymptomatic patient may have a mediastinal mass detected by roentgenography. The diagnosis may be confirmed with computed tomography or magnetic resonance imaging. As with most aneurysms, surgical treatment is recom mended, and the benefit-to-risk analysis depends on individual case factors.
HISTORY In 1735, Hunauld' first reported dysphagia attributed to an aberrant right subclavian artery. Subsequently, Bayford/ defined the anatomic features and coined the term "dysphagia lusoria" (dysphagia due to a freak of nature). McCallen and Schaff first described aneurysmal change in such a vessel 20 years after Kommerell' highlighted the existence of a diverticulum at the origin of some aberrant right subclavian arteries.
Although the incidence of aberrant right subclavian artery is reported to be between 0.5 and 1.0%,5 aneurysms of such arteries are uncommon. A review of the literature in 1985 yielded 32 cases, only 20 of which were treated surgically." Because of the relative paucity of such cases, we describe herein a surgically treated aneurysm of an aberrant right subclavian artery. Important embryologic, diagnostic, and
From the Section of Thoracic Surgery (P.D.K., W.D.B.), Section of Family Practice Medicine (T.E., W.c.), and Section of Radiology (G.K., W.H.), Fairfax Hospital, INOVA Health Care Systems, Annandale, Virginia, and Department of General Surgery (J.D.), Georgetown University Medical Center, Washington, DC.
Address reprint requests to Dr. P. D. Kiernan, Section of Thoracic Surgery, Fairfax Hospital, Suite 210, 3301 Woodburn Road, Annandale, VA 22003.
therapeutic facets are reviewed as well as the English-lan guage literature on the subject to date.
EMBRYOLOGY AND ANATOMY On the basis of the conceptual embryologic double aortic arch reported by Stewart, Kincaid, and Edwards," left- and right-sided aortic arches and respective aberrant right and left subclavian arteries are easily explained and visualized. Numerous other variations and anomalies are also clear ly explained, dependent on the site of arch agenesis or regression. Moreover, incomplete arch agenesis clarifies the origin of Kommerell's diverticulum, as noted in up to 60% of cases."
When aberrant subclavian arteries occur, they arise as the last brachiocephalic branch off the proximal descending aorta. The site of origin is usually the posteromedial aspect of the descending aorta (Fig. 1),9 Investigators currently believe that such vessels are almost always posterior to the esophagus, where they course in a caudad to cephalad man ner and create a characteristic retroesophageal indentation on esophagograrns.'? The ipsilateral recurrent laryngeal nerve, which has no embryologic vessels to negotiate around, courses directly to the larynx. The anatomic site of the thoracic duct may similarly be anomalous; often, it
Mayo Clin Proc 1993; 68:468-474 468 © 1993 Mayo Foundation for Medical Education and Research
Mayo Clin Proc, May 1993, Vol 68
Esophagus
Left subclavian
Fig. I. Diagram (superior view) of left aortic arch and anomalous right subclavian artery. (Redrawn from Grant.")
passes through the mediastinum to the right of its usual position. I I
REPORT OF CASE A right superior mediastinal shadow (Fig. 2), noted within the retrotracheal triangle" on a chest roentgenogram ob tained before a planned cholecystectomy, was the first clue to diagnosis in this otherwise asymptomatic 54-year-old man. The patient denied having dysphagia, and a roentgeno gram of the upper aspect of the gastrointestinal tract showed norma) findings. Computed tomograms of the chest (Fig. 3) obtained with use of contrast medium revealed a uniformly
ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY 469
enhancing retroesophageal mass contiguous with the aortic arch, which ascended along the expected course of the sub clavian artery. A 6-cm-diameter aneurysm of the aberrant right subclavian artery was diagnosed. No other thoracic aortic aneurysm was detected, and follow-up abdominal ultrasonography disclosed normal caliber of the abdominal aorta.
The patient was then referred for surgical consultation. Angiography was performed to confirm the diagnosis as well as to plan optimal exposure for control of the vessel off the descending thoracic aorta and for revascularization of the right upper extremity. With demonstration of the site of origin off the posteromedial aspect of the descending tho racic aorta (Fig. 4), we heeded the advice of a surgeon (Bematz PE. Personal communication) who previously had performed exposure of a similar vessel by means of a left thoracotomy and had concluded that a right-sided approach would likely be superior.
A right posterolateral thoracotomy, through the unresected fifth rib, provided optimal exposure (Fig. 5) of the descending aorta, the proximal Komrnerell diverticu lum as it merged into the aneurysm of the aberrant right subclavian artery, and the distal right subclavian artery where it coursed over the right first rib. The descending aorta was partially cross-clamped, and the distal subclavian artery was cross-clamped by means of a counterincision at the second intercostal space. The aneurysm sac was opened, the thrombus (Fig. 5 insert) was removed, and the caliber of the right subclavian artery was normal at the level of the first rib. Kommerell's diverticulum was oversewn
Fig.2. Chest roentgenograms of 54-year-old man obtained some years before development of aneurysm of aberrant right subclavian artery (Al and at time of diagnosis (B).
470 ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY Mayo Clio Proc, May 1993, Vol 68
Fig. 3. A and B, Computed tomograms of chest of 54-year-old man, showing origin of aneurysm of aberrant right subclavian artery arising from descending thoracic aorta.
with use of large pledgets and 3-0 polypropylene suture material.
Although a bypass from the ascending aorta to the right subclavian artery had been planned, an 8-mm woven Dacron graft was attached to the descending aorta, distal to the oversewn diverticulum (Fig. 6). Access to the .descending aorta was easier than to the ascending aorta. We originated the graft directly off the descending aorta rather than from the Kommerell diverticulum to minimize the likelihood of confusion with formation of a pseudoaneurysm during any subsequent investigations.
With excellent hemostasis, double ligation of two acces sory lymph ducts, and strong digital pulses achieved in the right upper extremity, the graft was separated from the adja cent esophagus and lung by the aneurysm sac and a mobi lized pleural flap. After an uncomplicated convalescence, the patient was dismissed on the sixth postoperative day.
REVIEW OF THE LITERATURE In 1985, Austin and Wolfe? published a case report and a review of the literature in which 31 of 32 patients had a left aortic arch. One patient had a right aortic arch and an aneurysm of an aberrant left subclavian artery. Of the 32 pa tients, 20 underwent a surgical procedure. Ten patients underwent left thoracotomy, all but two without reconstruc tion of the subclavian artery. The related mortality was 11% (one of nine patients for whom survival data were reported).
No deaths occurred among the six patients in Austin and Wolfe's review who underwent right thoracotomy. Of these patients, three underwent revascularization. In two other patients, a median sternotomy was used for exploration; neither patient underwent revascularization, and both died. Finally, two other patients underwent surgical treatment, but neither the exposure nor the facts relevant to re vascularization were described.
For 5 of the 12 nonoperative patients in Austin and Wolfe's series, information about the incidence of rupture and survival was lacking. Of the other seven patients, only two survived; five (71%) died of arterial rupture-four of rupture of the aneurysms of the subclavian artery and one of a ruptured abdominal aortic aneurysm.
Fig. 4. Aortogram of 54-year-old man, illustrating aneurysm of aberrant right subclavian artery emanating from a Kommerell di verticulum off descending thoracic aorta, without visualization of ipsilateral vertebral artery. Normal caliber of right subclavian artery is shown distally.
Mayo Clin Proc, May 1993, Vol 68
Esophagus
Descending aorta
Fig. 5. Diagram depicting method of cross-clamping by using partial occlusion clamp across origin of Kommerell's diverticulum off descending thoracic aorta and cross-clamping of right subcla vian artery through second intercostal space. Insert depicts throm bus retrieved from aneurysm. a. = artery; v. =vein.
An additional 21 cases have been culled from the English language literature during the 8 years since Austin and Wolfe's report, and 1 as yet unpublished case has been described to us (Cherry KJ, Bower TC. Personal communi cation) (Table 1). Of these 22 patients, 14 (64%) underwent surgical repair, 3 because of rupture. Although those three patients died, all other surgically treated patients for whom mortality data were reported have survived.
The mortality rates relative to operative and medical man agement for the cases reported to date are summarized in Table 2. The comparison of rates relative to type of opera tive exposure is included. Despite lack of longitudinal fol low-up, the risk of rupture of an aneurysm-of either the subclavian artery or another artery-in nonoperative patients exceeded 30% (at least 6 of 18 patients) (Table 1). This figure emphasizes the substantial risk of rupture of large aneurysms of aberrant subclavian arteries.
DISCUSSION Although the embryologic, anatomic, and diagnostic details of aneurysms of an aberrant right subclavian artery were discussed earlier in this report, a few additional comments seem appropriate. As in the current case, barium studies of the upper part of the gastrointestinal tract may not elicit a complete esophagogram. Undoubtedly, a complete esoph agogram would have illustrated the classic oblique retro esophageal indentation.
Computed tomography (or magnetic resonance imaging) is probably the best means of determining the presence of an
ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY 471
aneurysm, particularly in distinguishing the normal widened lumen of a Kommerell diverticulum from an actual aneu rysm. Arteriography is no substitute in this regard," al though we think that angiography is appropriate in preparing for surgical treatment.
As noted in our review of the literature, rupture of an aneurysm of an aberrant subclavian artery is as substantial a threat as rupture of any other aneurysm. 51 Thus, we strongly disagree with those investigators" who advise that only pa tients with symptomatic aneurysms or those in whom rupture seems imminent should be candidates for surgical treatment. Moreover, up to 25% of patients with aneurysms of an aberrant right subclavian artery harbor another aneurysm or aneurysms," and such patients are a high-risk group.
In past reports, the advisability of revascularization of surgically treated aneurysms of aberrant subclavian arteries has been debated; however, contemporary discussion relates more to the "how" than to the "whether"-because of a report" of an unacceptably high incidence of ischemia in those patients who do not undergo revascularization. Vari ous technical options are appropriate for the exposure cho sen.20,37,52 We favor repair by means of a thoracotomy ipsi lateral to the aneurysm, as was originally reported by Campbell" and subsequently advocated by Austin and Wolfe." With this approach, the closure of the site of aortic origin is easily exposed in light of the embryologic and
Fig. 6. Diagram depicting Dacron graft reconstruction of descend ing aorta to right subclavian artery. Kommerell's diverticulum is oversewn with pledgets.
472 ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY Mayo Clin Proc, May 1993, Vol 68
Table I.-Outcome of Patients After Medical or Surgical Treatment of Aneurysms of Aberrant Subclavian Arteries*
Revascu- Limb Case Operation Approach Survived larization ischemia Comment Reference
No Yes Died 21h yr later, 3 ruptured AAA
2 No No Rupture 13 3 Yes L thor No No ND 14 4 Yes Lthor Yes No ND 15 5 No No Rupture 16 6 Yes L thor Yes No ND 17 7 Yes Median' No No ND Rupture 18 8 Yes Median No No ND 19 9 Yes Lthor Yes No ND 19
10 Yes L thor Yes No ND 19 II Yes R thor Yes Yes ND 20 12 Yes ND No ND ND 21 13 No Yes 21 14 Yes R thor Yes No Yes 22 15 Yes Lthor No ND 23 16 No Yes 24 17 No No Rupture 25 18 Yes Lthor Yes Yes ND 26 19 No No Rupture 27 20 No No 28 21 Yes ND No ND ND Rupture 28 22 No No 28 23 No No 28 24 ND ND ND ND ND 29 25 Yes R thor Yes No ND 30 26 Yes Lthor Yes No ND 31 27 Yes R thor Yes Yes ND 32 28 Yes L thor Yes No ND 33 29 Yes R thor Yes No ND 34 30 No ND 35 31 Yes Lthor Yes Yes ND 36 32 Yes R thor Yes Yes ND 6 33 Yes Median Yes Yes No 37 34 No ND 38 35 Yes L thor Yes Yes ND 39 36 Yes ND No Yes ND Rupture 40 37 No ND 41 38 ND ND ND ND ND 42 39 No ND 43 40 Yes L thor Yes Yes No 44 41 Yes Median Yes No No 45 42 Yes R thor, Yes No No 46
median 43 Yes Median Yes Yes No 46 44 Yes Median Yes Yes No 47 45 No No Rupture 48 46 Yes ND ND ND ND 49 47 No ND ND 49 48 No ND ND 49 49 No ND ND 49 50 Yes L thor No Yes No Rupture 50 51 Yes Lthor No ND ND Rupture 51t 52 Yes Lthor Yes Yes No 51 53 Yes R thor Yes Yes No Current case 54 Yes L thor Yes Yes No :j:
*AAA =abdominal aortic aneurysm; L =left; median =median sternotomy; ND =not described; R =right; thor =thoracotomy.
tThe third case of this series had been published previously by Gomes and associates. I?
:j:Cherry KJ, Bower TC. Personal communication.
MayoClin Proc, May 1993, Vol68
Table 2.-Mortality Related to Management of Aneurysms of Aberrant Subclavian Arteries
Type of No. of Mortality management patients No. %
Surgical 34 8 24 Left thoracotomy 16 3 19 Right thoracotomy 8 0 0 Median sternotomy 6 2 33 Not described 4 3 75
Medical 18 8 44 Not described 2
Total 54 16 30
anatomic features as herein reviewed. Moreover, re vascularization necessitates no separate incision and may originate off the descending or the ascending aorta. Finally, our review of the literature seems to substantiate the safety and efficacy of such an approach.
CONCLUSION The methods for diagnosing aneurysms of an aberrant sub clavian artery have become less invasive and more sophisti cated. With increased use of computed tomography and magnetic resonance imaging techniques, these uncommon aneurysms will likely be diagnosed earlier and more fre quently than in the past. Earlier diagnosis should allow more timely surgical intervention before complications occur. Al though various surgical strategies have been devised, repair by means of a thoracotomy ipsilateral to the aneurysm offers advantages for exposure and safety as well as the capability of accomplishing revascularization easily through the same approach.
REFERENCES I. Hunauld M. Examen de quelques parties d'un singe. Hist
Acad Roy Sci 1735; 2:516-523 2. Bayford D. An account of a singular cause of obstructed
deglutition. Mem Med Soc Lond 1794; 2:275-286 3. McCallen AM, Schaff B. Aneurysm of an anomalous right
subclavian artery. Radiology 1956; 66:561-563 4. Kommerell B. Verlagerung des Osophagus durch eine
abnorm verlaufende Arteria subclavia dextra (Arteria lusoria). Fortschr Geb Rontgenstr 1936; 54:590-595
5. McDonald JJ, Anson BJ. Variations in the origin of arteries derived from the aortic arch, in American whites and negroes. Am J Phys Anthropol 1940; 27:91-107
6. Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of the literature. J Vase Surg 1985; 2:571-577
7. Stewart JR, Kincaid OW, Edwards IE. An Atlas of Vascular Rings and Related Malformations of the Aortic Arch System. Springfield (IL): Charles C Thomas, 1964
ANEURYSM OFABERRANT RIGHT SUBCLAVIAN ARTERY 473
8. Salomonowitz E, Edwards JE, Hunter DW, Castaneda Zuniga WR, Lund G, Cragg AH, et al. The three types of aortic diverticula. AJR Am J Roentgenol 1984; 142:673 679
9. Grant JCB. An Atlas of Anatomy. 5th ed. Baltimore: Williams & Wilkins, 1962
10. Beabout JW, Stewart JR, Kincaid OW. Aberrant right sub clavian artery: dispute of commonly accepted concepts. AJR Am J Roentgenol 1964; 92:855-864
11. Raider L. Aberrant right subclavian artery. South Med J 1967; 60:145-151
12. Raider L. The retrotracheal triangle. Chest 1973; 63:835 838
13. Richards WCD, Elliott CEo Aneurysm of an anomalous right subclavian artery. Br Heart J 1957; 19:141-143
14. Shannon JM. Aberrant right subclavian artery with Kommerell's diverticulum: report of a case. J Thorac Cardiovasc Surg 1961; 41:408-411
15. Candardjis G, Desbaillets P, Saegesser F. Coarctation simultanee de l' aorte et d'une arteria lusoria avec anevrisme, Radiol Clin (Basel) 1961; 30:308-322
16. Ryan JA. An unusual case of traumatic mediastinal aneurysm in a closed chest injury. Br J Surg 1962; 50:210-218
17. Gomes MMR, Bernatz PE, Forth RJ. Arteriosclerotic aneu rysm of an aberrant right subclavian artery. Dis Chest 1968; 54:549-552
18. Lynn RB. Kommerell's diverticulum with esophago-arterial fistula. Can J Surg 1969; 12:331-333
19. Hunter JA, Dye WS, Javid H, Najafi H, Julian Oc. Arterio sclerotic aneurysm of anomalous right subclavian artery. J Thorac Cardiovasc Surg 1970; 59:754-758
20. Campbell CF. Repair of an aneurysm of an aberrant retroesophageal right subclavian artery arising from Kommerell's diverticulum. J Thorac Cardiovasc Surg 1971; 62:330-334
21. Engelman RM, Madayag M. Aberrant right subclavian artery aneurysm: a rare cause of a superior mediastinal tumor. Chest 1972; 62:45-47
22. Sarot IA. Discussion of Engelman RM, Madayag M, Spencer FC. Aneurysm of aberrant right subclavian artery: rare cause of superior mediastinal tumor. N Y State J Med 1973; 73:290-292
23. Sakurai H, Lukban S, Litwak RS. Coarctation of aorta asso ciated with anomalous right subclavian artery and its aneu rysm. NY State J Med 1973; 73:292-296
24. Lui AHF. Aneurysm of anomalous right subclavian artery: report of a case. Am Surg 1973; 39:187-190
25. Dikman SH, Baron M, Gordon AJ. Right aortic arch with ruptured aneurysm of anomalous left subclavian artery. Am J Cardiol 1974; 34:245-249
26. Stoney WS, Alford WC Jr, Burrus GR, Thomas CS Jf. Aber rant right subclavian artery aneurysm. Ann Thorac Surg 1975; 19:460-467
27. Buades Reynes J, Aguirre Errasti C, Bilboa Ercoreca FJ, Onate Landa A. Aneurysm of aberrant right subclavian ar tery with esophageal perforation [letter]. Chest 1976; 70: 105
28. Wagner T, Thiry S. Aneurysma der Arteria 1usoria: Mitteilung von vier Fallen. Schweiz Med Wochenschr 1976; 106:15-21
29. Cunningham Gc. Radiologic case presentation. Cardiovasc Dis 1977; 4:4-6
474 ANEURYSM OF ABERRANT RIGHT SUBCLAVIAN ARTERY
30. Rodgers BM, Talbert JL, Hollenbeck JI. Aneurysm of anomalous subclavian artery: an unusual cause of dysphagia lusoria in childhood. Ann Surg 1978; 187:158-160
31. Dartevelle P, Bouquet P, Planche C, Merlier M. Anevrysme atheromateux d'une artere sous-claviere droite aberrante: a propos d'un cas revele par une dysphagie et opere chez une femme de 73 ans, Chirurgie 1978; 104:141-147
32. Jourdan J, Schmitt R, Kreitman P, Hatchuel P, Blaive B, Dor V. Anevrysme sur arteria lusoria: aspects cliniques, diagnostiques; resultats post-operatoires apres resection et retablissernent de la continuite arterielle. Arch Mal Coeur 1980; 73:221-226
33. Schmidt FE, Hewitt RL, Flores AA Jr. Aneurysm of anoma lous right subclavian artery. South Med J 1980; 73:255-256
34. McIntyre MD, Lynn RB. Kommerell's diverticulum. Can…
Related Documents
