Vascular ring

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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]

Contents

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]

Related Research Articles

<span class="mw-page-title-main">Trachea</span> Cartilaginous tube that connects the pharynx and larynx to the lungs

The trachea, also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all air-breathing animals with lungs. The trachea extends from the larynx and branches into the two primary bronchi. At the top of the trachea the cricoid cartilage attaches it to the larynx. The trachea is formed by a number of horseshoe-shaped rings, joined together vertically by overlying ligaments, and by the trachealis muscle at their ends. The epiglottis closes the opening to the larynx during swallowing.

<span class="mw-page-title-main">Esophagus</span> Vertebrate organ through which food passes to the stomach

The esophagus or oesophagus, colloquially known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the stomach. The esophagus is a fibromuscular tube, about 25 cm (10 in) long in adults, that travels behind the trachea and heart, passes through the diaphragm, and empties into the uppermost region of the stomach. During swallowing, the epiglottis tilts backwards to prevent food from going down the larynx and lungs. The word oesophagus is from Ancient Greek οἰσοφάγος (oisophágos), from οἴσω (oísō), future form of φέρω + ἔφαγον.

<span class="mw-page-title-main">Esophageal atresia</span> Congenital discontinuity of the oesophagus

Esophageal atresia is a congenital medical condition that affects the alimentary tract. It causes the esophagus to end in a blind-ended pouch rather than connecting normally to the stomach. It comprises a variety of congenital anatomic defects that are caused by an abnormal embryological development of the esophagus. It is characterized anatomically by a congenital obstruction of the esophagus with interruption of the continuity of the esophageal wall.

Tracheomalacia is a condition or incident where the cartilage that keeps the airway (trachea) open is soft such that the trachea partly collapses especially during increased airflow. This condition is most commonly seen in infants and young children. The usual symptom is stridor when a person breathes out. This is usually known as a collapsed windpipe.

<span class="mw-page-title-main">Brachiocephalic artery</span>

The brachiocephalic artery is an artery of the mediastinum that supplies blood to the right arm and the head and neck. It was previously known as the innominate artery, meaning unnamed artery.

<span class="mw-page-title-main">Aortic dissection</span> Injury to the innermost layer of the aorta

Aortic dissection (AD) occurs when an injury to the innermost layer of the aorta allows blood to flow between the layers of the aortic wall, forcing the layers apart. In most cases, this is associated with a sudden onset of severe chest or back pain, often described as "tearing" in character. Also, vomiting, sweating, and lightheadedness may occur. Other symptoms may result from decreased blood supply to other organs, such as stroke, lower extremity ischemia, or mesenteric ischemia. Aortic dissection can quickly lead to death from insufficient blood flow to the heart or complete rupture of the aorta.

<span class="mw-page-title-main">Subclavian artery</span> Major arteries of the upper thorax, below the clavicle

In human anatomy, the subclavian arteries are paired major arteries of the upper thorax, below the clavicle. They receive blood from the aortic arch. The left subclavian artery supplies blood to the left arm and the right subclavian artery supplies blood to the right arm, with some branches supplying the head and thorax. On the left side of the body, the subclavian comes directly off the aortic arch, while on the right side it arises from the relatively short brachiocephalic artery when it bifurcates into the subclavian and the right common carotid artery.

<span class="mw-page-title-main">Patent ductus arteriosus</span> Condition wherein the ductus arteriosus fails to close after birth

Patent ductus arteriosus (PDA) is a medical condition in which the ductus arteriosus fails to close after birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs through the aorta, which has a higher blood pressure, to the pulmonary artery, which has a lower blood pressure. Symptoms are uncommon at birth and shortly thereafter, but later in the first year of life there is often the onset of an increased work of breathing and failure to gain weight at a normal rate. With time, an uncorrected PDA usually leads to pulmonary hypertension followed by right-sided heart failure.

<span class="mw-page-title-main">Stenosis</span> Abnormal narrowing of a blood vessel or other tubular organ or structure

A stenosis is an abnormal narrowing in a blood vessel or other tubular organ or structure such as foramina and canals. It is also sometimes called a stricture.

<span class="mw-page-title-main">Coarctation of the aorta</span> Medical condition

Coarctation of the aorta, also called aortic narrowing, is a congenital condition whereby the aorta is narrow, usually in the area where the ductus arteriosus inserts. The word coarctation means "pressing or drawing together; narrowing". Coarctations are most common in the aortic arch. The arch may be small in babies with coarctations. Other heart defects may also occur when coarctation is present, typically occurring on the left side of the heart. When a patient has a coarctation, the left ventricle has to work harder. Since the aorta is narrowed, the left ventricle must generate a much higher pressure than normal in order to force enough blood through the aorta to deliver blood to the lower part of the body. If the narrowing is severe enough, the left ventricle may not be strong enough to push blood through the coarctation, thus resulting in a lack of blood to the lower half of the body. Physiologically its complete form is manifested as interrupted aortic arch.

<span class="mw-page-title-main">Recurrent laryngeal nerve</span> Nerve in the human body

The recurrent laryngeal nerve (RLN) is a branch of the vagus nerve that supplies all the intrinsic muscles of the larynx, with the exception of the cricothyroid muscles. There are two recurrent laryngeal nerves, right and left. The right and left nerves are not symmetrical, with the left nerve looping under the aortic arch, and the right nerve looping under the right subclavian artery then traveling upwards. They both travel alongside the trachea. Additionally, the nerves are among the few nerves that follow a recurrent course, moving in the opposite direction to the nerve they branch from, a fact from which they gain their name.

Stridor is a high-pitched extra-thoracic breath sound resulting from turbulent air flow in the larynx or lower in the bronchial tree. It is different from a stertor which is a noise originating in the pharynx. Stridor is a physical sign which is caused by a narrowed or obstructed airway. It can be inspiratory, expiratory or biphasic, although it is usually heard during inspiration. Inspiratory stridor often occurs in children with croup. It may be indicative of serious airway obstruction from severe conditions such as epiglottitis, a foreign body lodged in the airway, or a laryngeal tumor. Stridor should always command attention to establish its cause. Visualization of the airway by medical experts equipped to control the airway may be needed.

<span class="mw-page-title-main">Aortic arch</span> Part of the aorta

The aortic arch, arch of the aorta, or transverse aortic arch is the part of the aorta between the ascending and descending aorta. The arch travels backward, so that it ultimately runs to the left of the trachea.

<span class="mw-page-title-main">Aortic arches</span>

The aortic arches or pharyngeal arch arteries are a series of six paired embryological vascular structures which give rise to the great arteries of the neck and head. They are ventral to the dorsal aorta and arise from the aortic sac.

<span class="mw-page-title-main">Aberrant subclavian artery</span> Medical condition

Aberrant subclavian artery, or aberrant subclavian artery syndrome, is a rare anatomical variant of the origin of the right or left subclavian artery. This abnormality is the most common congenital vascular anomaly of the aortic arch, occurring in approximately 1% of individuals.

<span class="mw-page-title-main">Ortner's syndrome</span> Medical condition

Ortner's syndrome is a rare cardiovocal syndrome and refers to recurrent laryngeal nerve palsy from cardiovascular disease. It was first described by Norbert Ortner (1865–1935), an Austrian physician, in 1897.

Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. There are three types of interrupted aortic arch, with type B being the most common. Interrupted aortic arch is often associated with DiGeorge syndrome.

<span class="mw-page-title-main">Thoracic aorta injury</span> Medical condition

Injury of the thoracic aorta refers to any injury which affects the portion of the aorta which lies within the chest cavity. Injuries of the thoracic aorta are usually the result of physical trauma; however, they can also be the result of a pathological process. The main causes of this injury are deceleration and crush injuries. There are different grades to injuries to the aorta depending on the extent of injury, and the treatment whether surgical or medical depends on that grade. It is difficult to determine if a patient has a thoracic injury just by their symptoms, but through imaging and a physical exam the extent of injury can be determined. All patients with a thoracic aortic injury need to be treated either surgically with endovascular repair or open surgical repair or with medicine to keep their blood pressure and heart rate in the appropriate range. However, most patients that have a thoracic aortic injury do not live for 24 hours.

Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Most commonly there is a larger (dominant) right arch behind and a smaller (hypoplastic) left aortic arch in front of the trachea/esophagus. The two arches join to form the descending aorta which is usually on the left side. In some cases the end of the smaller left aortic arch closes and the vascular tissue becomes a fibrous cord. Although in these cases a complete ring of two patent aortic arches is not present, the term ‘vascular ring’ is the accepted generic term even in these anomalies.

<span class="mw-page-title-main">Right-sided aortic arch</span> Medical condition

Right-sided aortic arch is a rare anatomical variant in which the aortic arch is on the right side rather than on the left. During normal embryonic development, the aortic arch is formed by the left fourth aortic arch and the left dorsal aorta. In people with a right-sided aortic arch, instead the right dorsal aorta persists and the distal left aorta disappears.

References

  1. "Vascular rings - Overview - Mayo Clinic". www.mayoclinic.org. Retrieved 2022-06-12.
  2. Sahni, Deepank; Franklin, Wayne H. (2022), "Vascular Ring Double Aortic Arch", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID   32644364 , 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.