Medicine:Vascular ring

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Vascular ring
SpecialtyVascular system

A vascular ring is a congenital defect in which there is an abnormal formation of the aorta and/or its surrounding blood vessels. The trachea and esophagus are completely encircled and sometimes compressed by a "ring" formed by these vessels, which can lead to breathing and digestive difficulties.[1][2]

Most often this is because of persistence of the double aortic arch after the second month of fetal life.[citation needed]

Presentation

The two arches surround the esophagus and trachea which, if sufficiently constrictive, may cause breathing or swallowing difficulties despite medical therapies.[citation needed]

A less common ring is present with a right aortic arch instead of the usual left-sided aortic arch. This compresses the esophagus and trachea because of the persistence of a ductal ligament (from fetal circulation) that may connect between the aorta on the front and the left subclavian artery posteriorly going to the left arm.[citation needed]

Symptoms

Diagnosis

Infants with vascular rings typically present before 12 months with respiratory or esophageal symptoms like stridor, wheezing, cough, dysphagia, or difficulty feeding. The stridor improves with neck extension, differentiating from laryngomalacia which is relieved by prone or upright positioning, and will not be relieved with corticosteroids or epinephrine, unlike croup. Diagnosis requires a high degree of clinical suspicion and can be confirmed with barium contrast esophagogram for those with esophageal symptoms, bronchoscopy, or CT or MRI.[3]

Treatment

It is sometimes treated with surgery.[4]

References

  1. "Vascular rings - Overview - Mayo Clinic" (in en). https://www.mayoclinic.org/diseases-conditions/vascular-rings/cdc-20389579. 
  2. Sahni, Deepank; Franklin, Wayne H. (2022), "Vascular Ring Double Aortic Arch", StatPearls (Treasure Island (FL): StatPearls Publishing), PMID 32644364, http://www.ncbi.nlm.nih.gov/books/NBK558938/, retrieved 2022-06-12 
  3. "The presentation and management of vascular rings: an otolaryngology perspective". Int. J. Pediatr. Otorhinolaryngol. 71 (1): 57–62. Oct 2006. doi:10.1016/j.ijporl.2006.08.025. PMID 17034866. 
  4. "Vascular ring abnormalities: a retrospective study of 62 cases". J. Pediatr. Surg. 38 (4): 539–43. April 2003. doi:10.1053/jpsu.2003.50117. PMID 12677561. 

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Classification
External resources