Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology

Case 13

Clinical History:
The patient was a 1 month old female with biphasic stridor since birth that increases in intensity when the child is agitated or feeding.

Clinical Physical Exam:
Biphasic stridor, most pronounced over the upper trachea.

Clinical Labs:
Non-contributory

Clinical Differential Diagnosis:
Vascular ring due to a double aortic arch or a left aortic arch with an aberrant right subclavian artery

Imaging Findings:
An upper GI exam demonstrated a left sided aortic arch with a posterior indentation on the esophagus. An echocardiogram performed later demonstrated a double aortic arch with both limbs being of fairly equal caliber.

Images 1 and 2

Imaging Differential Diagnosis:
Left aortic arch with aberrant right subclavian artery, double aortic arch.

Operative Findings:
Esophagoscopy and laryngoscopy was performed one day after the Upper GI and demonstrated compression of the anterior wall of the trachea by a pulsating vessel, felt to be innominate artery. Compression of the posterior wall of the mid-esophagus was also seen and felt to be caused by a pulsating blood vessel. The vascular ring was electively divided 6 days later via a left posterolateral thoracotomy. The posterior aortic arch was divided, causing an impressive release of the ring with the 2 ends of the posterior arch separating by 2 to 3 centimeters. The ligamentum arteriosum was then divided.

Image 3

Image 4

Pathological Findings:
None

Final Diagnosis:
Double Aortic Arch, Complete Vascular Ring

Follow-up and Prognosis:
The patient had an uncomplicated post-operative course.

Similar Cases:
Case 2

References:

• Paediapedia

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