Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 4  |  Page : 435-437

Kommerell diverticulum with right-sided aortic arch with aberrant left subclavian artery


Department of Cardiothoracic and Vascular Surgery, Sawai Man Singh Medical College, Jaipur, Rajasthan, India

Date of Submission29-Jun-2020
Date of Decision07-Jul-2020
Date of Acceptance04-Aug-2020
Date of Web Publication24-Dec-2020

Correspondence Address:
Sourabh Mittal
Department of Cardiothoracic and Vascular Surgery, Sawai Man Singh Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_91_20

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  Abstract 


The right aortic arch with aberrant left subclavian artery is the most common cause of vascular ring and can be either asymptomatic or symptomatic owing to mass effect. Removal of Kommerell diverticulum and division of the ligamentum arteriosum through a left thoracotomy is currently advocated in symptomatic patients and patients with large diverticulum (>5 cm) to avoid inherent complications.

Keywords: Kommerell diverticulum, right-sided aortic arch, left subclavian artery, dysphagia lusoria, dyspnea


How to cite this article:
Mittal S, Sharma A, Dixit S, Sharma M. Kommerell diverticulum with right-sided aortic arch with aberrant left subclavian artery. Indian J Vasc Endovasc Surg 2020;7:435-7

How to cite this URL:
Mittal S, Sharma A, Dixit S, Sharma M. Kommerell diverticulum with right-sided aortic arch with aberrant left subclavian artery. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2021 Jan 16];7:435-7. Available from: https://www.indjvascsurg.org/text.asp?2020/7/4/435/304645




  Introduction Top


Kommerell diverticulum with a right-sided aortic arch and aberrant left subclavian artery (LSCA) is a rare congenital variation of vascular structures, which can be either asymptomatic or symptomatic owing to its mass effect. David Bayford first described this condition in the 1700s where an aberrant right subclavian artery (RSCA) compressed the esophagus and gave the term “dysphagia lusoria.”[1] Kommerell was the first one to diagnose this entity in 1936.[2]

The origin of LSCA and diverticulum is considered due to the faulty regression of the fourth primitive aortic arch. It can result from dilation of the anomalous origin of the subclavian artery in the following situations: left aortic arch (LAA) with anomalous origin of RSCA (0.5%–2.0%), or right aortic arch with anomalous origin of the LSCA (0.05%–0.1%).[3] Surgical intervention is warranted in symptomatic patients or asymptomatic patients with large diverticulum due to possible inherent risks of dissection and rupture.

We report a case of the right-sided aortic arch, Kommerell diverticulum, and left aberrant subclavian artery presenting to the department of cardiothoracic and vascular surgery, in a tertiary care center in North India, and review the available literature and management protocols.


  Case Report Top


We report a case of an 18-year-old male presenting with a history of progressive dysphagia, more with solids for 6 years. The patient also had complaints of dyspnea on exertion, choking sensations, and anxiety episodes. There was no history of syncope or limb weakness.

The patient was evaluated at a local hospital for the above-mentioned symptomatology and was referred to our department after a tentative diagnosis of aberrant LSCA was made in two-dimensional (2D) echocardiography.

On examination, the amplitude of the pulse was lower in the left upper limb (LUL) as compared to the right upper limb (RUL). Blood pressure in LUL was 100/64 mm of mercury and RUL was 118/64 mm of mercury.

The patient was evaluated as per departmental protocol. Routine blood investigations were within the normal limits. Electrocardiography findings were within the normal limits. Two-dimensional echocardiography was repeated and findings were confirmed and structural heart defects were ruled out.

The chest radiograph was showed a right-sided aorta and possibility of an upper mediastinal mass. Contrast esophagogram was suggestive of indentation in the upper esophagus [Figure 1]. Upper GI endoscopy was suggestive of a bulge in the posterior wall of the esophagus possibly due to extrinsic compression. Computed tomography (CT) aortogram showed a smooth margined saccular structure in the upper part of the posterior mediastinum with a mass effect on the esophagus with a possibility of Kommerell diverticulum with a right-sided aortic arch and aberrant LSCA [Figure 2].
Figure 1: Contrast esophagogram showing indentation (marked with an an arrow) due to diverticulum

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Figure 2: Preoperative computed tomography angiogram showing diverticulum with the origin of the left subclavian artery (marked with an arrow)


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After the necessary evaluation and optimization, the patient was taken up for surgery. The patient was induced under general anesthesia on double-lumen endotracheal tube and nasogastric tube in place. Left posterolateral thoracotomy was done through fourth intercostal space and a transpleural approach was taken. Vagus and recurrent laryngeal nerves were identified and preserved and were retracted away from the field. A bulbous diverticulum measuring 6 cm × 5 cm and the ligamentum arteriosum were seen behind the esophagus and were causing extrinsic compression over it. The ligamentum arteriosum was doubly ligated and transected. After permissive hypotension, side biting vascular clamp was applied over diverticulum and it was sharply cut [Figure 3]. Interposition dacron graft of 6 mm diameter was used for repositioning of the subclavian artery over a divided stump [Figure 4]. On releasing the vascular clamp, distal pulsation to graft and radial pulsation were confirmed by the anesthesiologist. The patient was reversed and extubated and was shifted to the intensive care unit. Postoperative period was uneventful. The patient was orally allowed on the postoperative day (POD-2) and was shifted to ward on POD-3. The intercostal drainage tube was removed on the POD-4, and the patient was discharged on POD-8. On a close follow-up of 1 year, the patient is symptom-free with no recurrence of dysphagia, and pulsation in LUL is of the same amplitude as of RUL. Postoperative CT angiogram has not been done yet and will be planned in case of recurrence of symptoms and change in characteristics of pulse.
Figure 3: Intra-operative image showing tied ductus remnant and side biting clamp over diverticulum with control of the subclavian artery

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Figure 4: Intra-operative image showing an end-to-side anastomosis of the subclavian artery to aorta after diverticulum excision with interposed Dacron graft

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  Discussion Top


Left aberrant subclavian artery is a rare vascular anomaly that originates from right-sided aortic arch as its fourth branch, passes behind the esophagus, and crosses the posterior part of mediastinum, and drains into LUL. Kommerell diverticulum is a bulbous swelling at the point of origin of the subclavian artery. It can exist in various aortic arch anomalies but most frequently seen in cases of right-sided aortic arch with aberrant LSCA. Embryo logically, it is due to regression of fourth LAA between the left carotid and LSCA. The presence of a left ductus arteriosus or a ligamentum arteriosum between the LSCA and the left pulmonary artery in such patients results in a vascular ring. This is the most common type of vascular ring (except for that which occurs with a double aortic arch). The ring is generally loose; therefore, symptoms of tracheal or oesophageal compression is often mild or absent.[1],[4]

The location of the diverticulum is posterior to the esophagus in 80% of cases, between the trachea and the esophagus in 15% of cases, and anterior to the trachea in 5% of cases.[5]

The original description of this diverticulum was in a patient with LAA and aberrant RSCA. Patients are usually asymptomatic.

Another arch anomaly with diverticulum is LAA with the right descending aorta and right ligamentum arteriosum. It is not associated with cardiac anomalies but can produce symptoms of dysphagia.

Right-sided aortic arch without a simultaneous congenital heart defect is extremely rare. In 5%–10% of cases, it is related to congenital heart diseases, including tetralogy of Fallot, pulmonary stenosis with ventricular septal defect, tricuspid atresia, and patent ductus arteriosus. This particular patient did not have any congenital heart disease.[6]

Most commonly, patients are mildly symptomatic and this anomaly is an incidental finding. Progressive symptoms related to compression of the esophagus (dysphagia) or trachea (stridor) may be observed in cases with large dilatations or with localized diverticula at specific sites. Cardiovascular symptoms can also occur as a result of associated structural cardiac malformations, early atherosclerotic lesions, or abnormal artery dissection.[7],[8] Our patient had both dysphagia and dyspnea, which can be attributed to this vascular malformation.

The requirement of surgical intervention is decided by the size of the diverticulum and symptomatology of the patients. A Kommerell diverticulum with a diameter >5 cm or in symptomatic patients not relieved by medical management; can lead to complications such as tracheomalacia, oesophageal dilation, or rupture of the aneurysm.[9]

Our diagnosis was based on clinical assessment and a battery of tests, i.e., 2D echocardiography, contrast esophagogram, CT aortogram, and upper GI endoscopy. Considering the size of the diverticulum and poor quality of life, the decision for surgical intervention was taken in this patient. There are only a few case reports of such anomalies and other variants in literature. Standard management options of Kommerell diverticulum are not yet established because of the rarity of this anomaly. There are no established guidelines, but only experiences, for management of such patients.


  Conclusion Top


Right-sided aortic arch with aberrant LSCA is a rare vascular condition, which is incidental finding most of the time. They remain asymptomatic unless diverticulum causes compression of the esophagus or trachea or due to vascular ring formation. Decision-making in such patient poses a challenge for the surgeons due to other associated anomalies and its proximity to vital structures. Due to a lack of literature in such cases, treatment execution has to be individualized considering associated anomalies. There is always a risk associated with surgical intervention immediately and on follow-up which has to be carefully weighed upon.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Miller JM, Miller KS. A note on the historical aspects of dysphagia lusoria. Am Surg 1992;58:502-3.  Back to cited text no. 1
    
2.
Kommerell B. Displacement of the esophagus through an abnormally running arteria subclavia dextra (arteria lusoria). Fortschr Geb Roentgenstrahlen 1936;54:590-5.  Back to cited text no. 2
    
3.
Mubarak MY, Kamarul AT, Noordini MD. Right-sided Aortic Arch with Aberrant Left Subclavian Artery from Kommerell's Diverticulum. Iran J Radiol 2011;8:103-6.  Back to cited text no. 3
    
4.
Backer CL, Ilbawi MN, Idriss FS, DeLeon SY. Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg 1989;97:725-31.  Back to cited text no. 4
    
5.
van Son JA, Julsrud PR, Hagler DJ, Sim EK, Pairolero PC, Puga FJ, et al. Surgical treatment of vascular rings: The Mayo Clinic experience. Mayo Clin Proc 1993;68:1056-63.  Back to cited text no. 5
    
6.
Cinà CS, Althani H, Pasenau J, Abouzahr L. Kommerell's diverticulum and right-sided aortic arch: A cohort study and review of the literature. J Vasc Surg 2004;39:131-9.  Back to cited text no. 6
    
7.
Ahmadi A, Sonbolestan SA. A rare anomalous origin of left subclavian artery from the circle of Willis in combination with right sided aortic arch: A case report. ARYA Atheroscler 2013;9:303-5.  Back to cited text no. 7
    
8.
Ota T, Okada K, Takanashi S, Yamamoto S, Okita Y. Surgical treatment for Kommerell's diverticulum. J Thorac Cardiovasc Surg 2006;131:574-8.  Back to cited text no. 8
    
9.
Rosa P, Gillespie DL, Goff JM, O'donnell SD, Starnes B. Aberrant right subclavian artery syndrome: A case of chronic cough. J Vasc Surg 2003;37:1318-21.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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