Radiology Teaching Files > Case 3131364

Contributed by: J P.
Patient: 4 month old male
History: Patient presented with prolonged history of "raspy breathing, especially when crying".

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CXR: Tracheal narrowing.

Recons: Right sided arch with left innominate artery, which branches into Left subclavian and left ICA, and PDA.


Right sided aortic arch.


A right sided arch does not necessarily cause symptoms, but if the physiological abnormalities described are present, the diagnosis of vascular ring is virtually certain. Two types of right sided aortic arch can be distinguished: those with "mirror image branching," in which a left innominate artery arises as the first branch off the aortic arch, dividing into a left common carotid and left subclavian artery, and those with an aberrant left subclavian artery, which arises as a fourth branch off the aortic arch. The first type is often associated with cyanotic heart disease, whereas the second one is usually associated with a normal heart.3 An aberrant left subclavian artery can be confirmed by barium swallow but is not of itself pathological and may be a normal variant in 1% of the population. A left sided ductus arteriosus (or ligamentum arteriosum) completes the ring, and it is this combination that can cause symptoms. In one series of 26 patients with this type of vascular ring, six had symptoms of tracheo-oesophageal compression.4 Another study reported 15 children, three of whom required division of the ring.5 If the importance of these vascular abnormalities remains unclear, a fibreoptic bronchoscopy allowing direct inspection of the airway should be performed. Surgery entails dividing the ligamentum arteriosum, thereby breaking the constricting circle. Freeing up the surrounding connective tissues from the trachea, oesophagus, and aorta further enhances this manoeuvre. After surgery, secondary tracheomalacia is almost always present after the prolonged period of airway compression. Symptoms may not resolve completely, and abnormalities of the flow volume loop may persist.

Delayed diagnosis of a vascular ring can result in unnecessary investigations and prolonged periods of ineffective treatment. Such treatment may be harmful. Inhaled corticosteroids are an effective treatment for asthma but may have adverse effects on growth, bone density, and adrenal function if used inappropriately.6 Prolonged airway compression may also be harmful. Recognition of the correct diagnosis, with consequent surgery, leads to an improvement in symptoms and withdrawal of unnecessary treatment. Consideration of alternative diagnoses, with careful inspection of the chest x ray film, is essential in the management of children with persistent respiratory symptoms.

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Additional Details:

Case Number: 3131364Last Updated: 11-15-2005
Anatomy: Vascular/Lymphatic   Pathology: Congenital
Modality: CT, Conventional RadiographExam Date: 10-14-2005Access Level: Readable by all users
Keywords: aortic arch aberrant left subclavian

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