Pulmonary Vascular Disease: Thoracic Vascular Disorders |

The Unlucky Triad: Vascular Ring Due to Extremely Rare Right Sided Aortic Arch With Ductus Diverticulum and Retro-Esophageal Ligamentum Causing Severe Dysphagia FREE TO VIEW

Ankur Sinha, MBBS; Hitesh Raheja, MBBS; Bridget Gallagher, MD; Sunil Abrol, MD; Vijay Shetty, MD
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Maimonides Medical Center, Brooklyn, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1231A. doi:10.1016/j.chest.2016.08.1340
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SESSION TITLE: Thoracic Vascular Disorders

SESSION TYPE: Student/Resident Case Report Slide

PRESENTED ON: Monday, October 24, 2016 at 11:00 AM - 12:00 PM

INTRODUCTION: Right-sided aortic arch is a rare anomaly occuring in 0.1% of population. A complete right-sided aortic arch is an incidental finding on X- Ray while a ductus diverticulum is an out-pouching at the aorto-ductal junction. The ductus arteriosus closes and the ductus diverticulum shrinks away to a “Ductus bump”. The ligamentum arteriosum is a major source of trache-esophageal constriction, and a retro-esophageal position causes severe dysphagia. We present a case of severe dysphagia secondary to what we would like to refer as the “Unlucky Triad” of a Right sided aortic arch, a large ductus diverticulum and a retro-esophageal ligamentum.

CASE PRESENTATION: 49-year old African American male presented with a 3-month history of difficulty swallowing both solid and liquida with a feeling of “food getting stuck”. He had retrosternal discomfort with severe retching with food and mucus regurgitating and severe halitosis. Chest X-ray revealed a right sided aortic arch with a tracheal shift to the left. A swallow study with iohexol confirmed a fixed esophageal narrowing at T6. CT Angiogram of the chest and neck with IV contrast revealed a mirror image right aortic arch with a left sided cardiac apex, and a prominent ductus diverticulum measuring 1.7 cm by 1.8cm. This structure extended posteriorly to the mid esophagus, indenting it. Surgical intervention was planned and succesfully executed relieving the obstruction.

DISCUSSION: A right-sided aortic arch has been associated with many variations. based on relation to surrounding anatomy, it may present with variable symptoms. In our patient the aortic arch showed a mirror image branching where the arch passed over the right main stem bronchus and continued on as the descending aorta while giving out branches. A ductus diverticulum is a remnant of the infundibular part of the ductus arteriosus. On the lateral view it can be seen as a soft tissue mass in the aorto-pulmonary window. The vascular ring comprised of 3 structures indenting the esophagus in our case. We have referred to this chance occurrence as the “unlucky triad”. The first structure was the ascending right-sided aortic arch anteriorly; the second structure was the ductus diverticulum laterally. The third and posterior component was a retro-esophageal ligamentum arteriosum.

CONCLUSIONS: The triad of right-sided aortic arch anteriorly; the ductus diverticulum laterally and a retro-esophageal ligamentum arteriosum is an extremely rare occurence with significant morbidity due to severe dysphagia and failure to thrive.

Reference #1: Batheeb N.A., Habbab L.M., and Qattan N.M.: Symptomatic stenosis of left subclavian artery from Kommerell's diverticulum. Asian Cardiovasc Thorac Ann 2014; undefined:

Reference #2: Cina CS, Arena GO, Bruin G, Clase CM. Kommerell’s diverticulum and aneurysmal right-sided aortic arch: a case report and review of the literature. J Vasc Surg 2000;32: 1208-14

DISCLOSURE: The following authors have nothing to disclose: Ankur Sinha, Hitesh Raheja, Bridget Gallagher, Sunil Abrol, Vijay Shetty

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