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The hardest pill to swallow: A freak of nature

      Abstract

      Not required

      Keywords

      Abbreviations:

      (CT) (computed tomography), (ARSA) (aberrant right subclavian artery)

      1. Case

      A 74-year-old woman presented for evaluation of intermittent dysphagia to solids for the past year and a foreign body sensation in her chest that started one hour ago, after she took some vitamins. She had a history of coronary artery stenting 5 months ago and was on dual antiplatelet therapy with aspirin and clopidogrel. Before arrival she had an episode of vomiting undigested food with moderate amount of bright red blood. Patient denied abdominal pain, heartburn or reflux, blood in stools or black stools; also denied weight loss or history of cancer in her family.
      She was hemodynamically stable and her physical examination was unremarkable. Laboratory tests revealed anemia (10.6 g/dL) and a high BUN/Creatinine ratio (>20). Initial x-ray of the neck demonstrated air collection in the esophagus at C6-C7 level consistent with distal obstruction (Fig. 1). An upper endoscopy (Fig. 2A) followed by a chest computed tomography (CT) with contrast (Fig. 2B) were obtained for further assessment. What is the diagnosis?
      Fig. 1
      Fig. 1Cervical spine and soft tissue x-ray of the neck, frontal and lateral views.
      Fig. 2
      Fig. 2Upper gastrointestinal endoscopy (A). Computed tomography of the chest with contrast, axial and coronal views (B).

      2. Discussion

      The endoscopy demonstrated a pulsatile lesion in the upper third of the esophagus, attached to a large blood clot that extended throughout the esophagus to the gastroesophageal junction (Fig. 2A). The chest CT showed a distended esophagus filled with non-enhancing dense debris, in addition to a vessel coursing posterior to it and causing extrinsic esophageal compression (Fig. 2B).
      This is a case of an aberrant right subclavian artery (ARSA), a congenital anomaly first described by Bayford as dysphagia lusus naturae or “freak of nature” [
      • Myers P.O.
      • Fasel J.H.
      • Kalangos A.
      • Gailloud P.
      Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects.
      ], that consists of a right subclavian artery arising directly from the aortic arch and passing through the mediastinum, rather than originating from the brachiocephalic artery [
      • Abraham V.
      • Mathew A.
      • Cherian V.
      • Chandran S.
      • Mathew G.
      Aberrant subclavian artery: anatomical curiosity or clinical entity.
      ]. It has a prevalence up to 1.8% and is associated with cardiac anomalies and Down syndrome [
      • Myers P.O.
      • Fasel J.H.
      • Kalangos A.
      • Gailloud P.
      Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects.
      ]. Patients are usually asymptomatic but may develop symptoms in their young adulthood. Late-onset presentation is not uncommon, as in this case, and may be secondary to decreased flexibility of the esophagus due to aging, increased rigidity of the vessel wall due to atherosclerosis, and esophageal compression due to aneurysmal dilatation of the ARSA [
      • Levitt B.
      • Richter J.E.
      Dysphagia lusoria: a comprehensive review.
      ]. Non-invasive angiography is the gold standard for diagnosis and for surgical planning [
      • Abraham V.
      • Mathew A.
      • Cherian V.
      • Chandran S.
      • Mathew G.
      Aberrant subclavian artery: anatomical curiosity or clinical entity.
      ]. Treatment consists of dietary modification and surgery is reserved for severe symptomatic cases [
      • Myers P.O.
      • Fasel J.H.
      • Kalangos A.
      • Gailloud P.
      Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects.
      ]. Our patient underwent bilateral carotid-subclavian transposition and thoracic endovascular aortic repair with resolution of her symptoms.

      Prior presentations

      None.

      Ethics committee approval

      Not applicable.

      Patient consent

      The patient provided signed informed consent for the publication of this manuscript.

      Funding information

      None.

      Declaration of Competing Interest

      The authors declare they have no conflict of interest.

      Acknowledgements

      The authors thank David O. Rahni, MD, Joanna Sesti, MD and Julie Schwegman, PA for their helpful contributions to this report.

      References

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        • Fasel J.H.
        • Kalangos A.
        • Gailloud P.
        Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects.
        Ann Cardiol Angeiol (Paris). 2010; 59 (Jun): 147-154
        • Abraham V.
        • Mathew A.
        • Cherian V.
        • Chandran S.
        • Mathew G.
        Aberrant subclavian artery: anatomical curiosity or clinical entity.
        Int J Surg. 2009; 7 (Apr): 106-109
        • Levitt B.
        • Richter J.E.
        Dysphagia lusoria: a comprehensive review.
        Dis Esophagus. 2007; 20: 455-460