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Eight-month-old boy with irritability after feeding.

Our appreciation is extended to Dr. Jeremy Cardinal,
Indiana University Department of Radiology,
for contributing this case.

History:  Eight-month-old boy with irritability after feeding.
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Which choice best localizes the most salient abnormality?

Anterior mediastinum.Posterior mediastinum.Pulmonary vascularity.Diaphragm.Vertebral column.
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Eight-month-old boy with irritability after feeding.

Here are some images from a fluoroscopic swallow study. Click to enlarge.

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Which choice is most appropriate?

Extrinsic compression.Intraluminal foreign body.Intraluminal neoplasm.Duplication.Fistula.
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Eight-month-old boy with irritability after feeding.



Same fluoroscopy images are again shown below. Which choice is most likely responsible for the extrinsic compression?

Adjacent diverticulum.Neuroenteric cyst.Bronchogenic cyst.Neurogenic tumor.Vessel.Click for galleryClick for galleryClick for gallery
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Eight-month-old boy with irritability after feeding.

Here are some CT images. Click to enlarge.

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Findings:  Lateral CXR demonstrates a posterior impression (anterior displacement) on the trachea. The upper GI study demonstrates an oblique impression on the posterior aspect of the esophagus, coursing from the right inferior to left superior. The CT chest demonstrates a right aortic arch with aberrant left subclavian artery.


Differential diagnosis:

  • Right aortic arch with aberrant left subclavian artery
  • Left aortic arch with aberrant right subclavian artery
  • Right aortic arch with mirror image branching
  • Double aortic arch
Diagnosis:  Right aortic arch with aberrant left subclavian artery
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Eight-month-old boy with irritability after feeding.


Key points:
 

  • Right aortic arch with aberrant left subclavian artery is usually an incidental finding, estimated to be symptomatic in 5% of cases. Airway related symptoms, are most common in infancy. Dysphagia is rare.
  • A vascular ring is formed, with the trachea and esophagus bounded by the ascending aorta anteriorly, the aortic arch on the right, the descending aorta and aberrant left subclavian artery posteriorly, and the ligamentum arteriosum and left pulmonary artery on the left
  • When associated with a tightly constricting ligamentum arteriosum, may cause congenital stridor.
  • In 60% of cases, there is dilation of the origin of the aberrant left subclavian artery (the diverticulum of Kommerell).
  • Congenital heart disease is present in 10-15% of right sided arch.

References:

  1. Westra, Sjirk J, MD. "Right Arch with Aberrant Left SCA." Statdx. https://my.statdx.com/#dxContent;right_arch_with_aberrant_left_sca.
  2. Bhimji, Shabir, MD, PhD. "Vascular Rings." Emedicine. Oct 22, 2008.
  3. http://emedicine.medscape.com/article/426233-overview#a0102.
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Eight-month-old boy with irritability after feeding.


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