Vascular ring

Specialty| Vascular 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]

## Contents

* 1 Signs and symptoms
* 2 Diagnosis
* 3 Treatment
* 4 References
* 5 External links

## Signs and symptoms[edit | edit source]

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]

## Diagnosis[edit | edit source]

a-c)Preoperative computed tomograms of vascular rings

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[edit | edit source]

It is sometimes treated with surgery.[4]

## References[edit | edit source]

1. ↑ "Vascular rings - Overview - Mayo Clinic". www.mayoclinic.org. Archived from the original on 2022-06-02. Retrieved 2022-06-12.
2. ↑ Sahni, Deepank; Franklin, Wayne H. (2022), "Vascular Ring Double Aortic Arch", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 32644364, archived from the original on 2022-08-12, retrieved 2022-06-12
3. ↑ Shah R, Mora B, Bacha E, Sena L, Buonomo C, Del Nido P, Rahbar R (Oct 2006). "The presentation and management of vascular rings: an otolaryngology perspective". Int. J. Pediatr. Otorhinolaryngol. 71 (1): 57–62. doi:10.1016/j.ijporl.2006.08.025. PMID 17034866.
4. ↑ Bonnard A, Auber F, Fourcade L, Marchac V, Emond S, Révillon Y (April 2003). "Vascular ring abnormalities: a retrospective study of 62 cases". J. Pediatr. Surg. 38 (4): 539–43. doi:10.1053/jpsu.2003.50117. PMID 12677561.`{{cite journal}}`: CS1 maint: uses authors parameter (link)

## External links[edit | edit source]

Classification|

* ICD-10: Q25.4
* ICD-9-CM: 747.21

|

External resources|

* MedlinePlus: 007318
* eMedicine: med/2981

* v
* t
* e

Congenital vascular defects / Vascular malformation (Q25–Q28, 747)

Great arteries/
other arteries|

| Aorta|

* Patent ductus arteriosus
* Coarctation of the aorta
* Interrupted aortic arch
* Double aortic arch
* Right-sided aortic arch
* Overriding aorta
* Aneurysm of sinus of Valsalva
* Vascular ring

|

Pulmonary artery|

* Pulmonary atresia
* Stenosis of pulmonary artery

Subclavian artery|

* Aberrant subclavian artery

Umbilical artery|

* Single umbilical artery

Great veins|

| Superior/inferior vena cava|

* Congenital stenosis of vena cava
* Persistent left superior vena cava

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Pulmonary vein|

* Anomalous pulmonary venous connection (Total, Partial)
* Scimitar syndrome

Arteriovenous malformation|

* Cerebral arteriovenous malformation

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