Materiały źródłowe

• #1 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  A vascular ring is an unusual congenital condition in which the anomalous configuration of the arch, associated vessels, or both surrounds the trachea and esophagus, forming a complete or incomplete ring around them. […] In spite of the significant variability in this group of structural abnormalities of the aortic arch, they all possess a common feature: They all produce some degree of compression on the major airway structures, the esophagus, or both. The location and severity of compression varies with the configuration of the lesion. Individuals with anomalies producing more severe compression are likely to present earlier in life. […] Surgical division of a vascular ring is indicated in all symptomatic patients. To avoid serious complications, surgery should not be delayed, especially in patients with symptoms of airway compression. Individuals who have no symptoms from a vascular ring may not require surgical intervention. Surgical repairs in most cases can be performed successfully and without long-term morbidity.

• #2 Vascular Ring Double Aortic Arch – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558938/

  Vascular ring refers to a congenital anomaly where vascular structures encircle and compress the trachea and esophagus, leading to respiratory and gastrointestinal symptoms. […] The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist. The ascending aorta and transverse aortic arch compress the trachea, whereas the right aortic arch compresses the esophagus. […] The ascending aorta further divides into right and left transverse arches in DAA. The right transverse arch courses over the right mainstem bronchus, whereas the left aortic arch is over the left. Because the aorta usually descends towards the left side of the body, the right aortic arch goes posteriorly and inserts into it. Further branches originate from the transverse arches: the left common carotid and subclavian from the left aortic arch and the right common carotid and right subclavian from the right arch.

• #3 Vascular rings and slings – UpToDate

  http://www.uptodate.com/contents/vascular-rings

  Vascular rings are congenital anomalies of the aortic arch that result in compression of the tracheobronchial tree and/or esophagus, leading to respiratory and gastrointestinal symptoms. They can be classified as either complete (when both the trachea and esophagus are fully encircled by a vascular anomaly) or incomplete (without full encirclement of both structures). […] Beginning at the fourth week of embryogenesis, the aortic arch develops from six symmetrical paired aortic arch vessels and the paired dorsal aortae. During the next few weeks of embryogenesis, remodeling and rearrangement of these structures result in the formation of the normal left aortic arch.

• #4 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  The various forms of this anomaly occur very early in embryologic development. They result from the abnormal or incomplete regression of one of the six embryonic branchial arches. […] Abnormal arch development results when one or more of the necessary involutions or other changes in embryonic arch structures does not occur.

• #5 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  The various forms of this anomaly occur very early in embryologic development. They result from the abnormal or incomplete regression of one of the six embryonic branchial arches. […] Abnormal arch development results when one or more of the necessary involutions or other changes in embryonic arch structures does not occur.

• #6

  https://step2.medbullets.com/evidence/31676999

  A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. […] In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. […] For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.

• #7 Vascular Ring Double Aortic Arch – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558938/

  Vascular ring refers to a congenital anomaly where vascular structures encircle and compress the trachea and esophagus, leading to respiratory and gastrointestinal symptoms. […] The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist. The ascending aorta and transverse aortic arch compress the trachea, whereas the right aortic arch compresses the esophagus. […] The ascending aorta further divides into right and left transverse arches in DAA. The right transverse arch courses over the right mainstem bronchus, whereas the left aortic arch is over the left. Because the aorta usually descends towards the left side of the body, the right aortic arch goes posteriorly and inserts into it. Further branches originate from the transverse arches: the left common carotid and subclavian from the left aortic arch and the right common carotid and right subclavian from the right arch.

• #8 Case Based Pediatrics Chapter

  http://hawaii.edu/medicine/pediatrics/pedtext/s07c07.html

  Vascular rings and pulmonary slings are congenital anomalies of the aortic arch and pulmonary artery. They are very important but rare causes for common respiratory symptoms, especially in infants. Their etiology is related to abnormal development of the embryonic aortic arches. When the abnormal blood vessels form a ring around the trachea and esophagus, it is termed vascular ring. These rings may be complete or incomplete. The severity of symptoms depends on the degree of compression of the trachea and esophagus. […] Failure of regression or persistence of normally regressed portions will result in one of many vascular rings or a pulmonary artery sling. […] The classic vascular rings will result from disruption at 4 points in the normal development. The first point is at the right dorsal aorta. This normally will regress but if it persists, a double aortic arch will develop. The second point is at the right 4th aortic arch. This normally will persist and develop into the proximal portion of the subclavian artery on the right. Failure of this to develop will result in the right subclavian artery to arise from the left aortic arch. The third point is at the left dorsal aorta. This normally persists to form the left descending aorta. If this regresses, a right aortic arch will persist. The fourth point is at the left 4th arch. This normally persists and develops into a portion of the left aortic arch. If this regresses, a right aortic arch will persist and the left subclavian will arise from the right arch.

• #9 Case Based Pediatrics Chapter

  http://hawaii.edu/medicine/pediatrics/pedtext/s07c07.html

  Vascular rings and pulmonary slings are congenital anomalies of the aortic arch and pulmonary artery. They are very important but rare causes for common respiratory symptoms, especially in infants. Their etiology is related to abnormal development of the embryonic aortic arches. When the abnormal blood vessels form a ring around the trachea and esophagus, it is termed vascular ring. These rings may be complete or incomplete. The severity of symptoms depends on the degree of compression of the trachea and esophagus. […] Failure of regression or persistence of normally regressed portions will result in one of many vascular rings or a pulmonary artery sling. […] The classic vascular rings will result from disruption at 4 points in the normal development. The first point is at the right dorsal aorta. This normally will regress but if it persists, a double aortic arch will develop. The second point is at the right 4th aortic arch. This normally will persist and develop into the proximal portion of the subclavian artery on the right. Failure of this to develop will result in the right subclavian artery to arise from the left aortic arch. The third point is at the left dorsal aorta. This normally persists to form the left descending aorta. If this regresses, a right aortic arch will persist. The fourth point is at the left 4th arch. This normally persists and develops into a portion of the left aortic arch. If this regresses, a right aortic arch will persist and the left subclavian will arise from the right arch.

• #10 Case Based Pediatrics Chapter

  http://hawaii.edu/medicine/pediatrics/pedtext/s07c07.html

  Vascular rings encompass only 1% to 3% of all congenital heart disease. Some vascular rings are associated with other congenital heart lesions while others are isolated defects. Tracheobronchial anomalies are rarely seen with vascular rings but are common in pulmonary artery slings. […] The most common symptomatic vascular ring is the double aortic arch. This results from persistence of the right dorsal aorta. The right and left aortic arches encircle the trachea and esophagus. The double aortic arch thus forms a ring around the trachea and esophagus compressing both the trachea and esophagus to a variable degree. […] A pulmonary sling is the left pulmonary artery arising from the right pulmonary artery. It is also known as an anomalous pulmonary artery and results from regression/failure of development of the left pulmonary artery.

• #11 Vascular Ring | Thoracic Key

  https://thoracickey.com/vascular-ring/

  Vascular ring refers to a group of anomalies of the aortic arch that cause respiratory symptoms or feeding problems. […] The vascular ring may be divided into two groups, complete (or true) and incomplete. […] Complete vascular ring refers to conditions in which the abnormal vascular structures or their remnants form a complete circle around the trachea and esophagus. […] Incomplete vascular ring refers to vascular anomalies that do not form a complete circle around the trachea and esophagus but do compress the trachea or esophagus. […] Double aortic arch is the most common vascular ring (40%). This anomaly is caused by a failure of regression of both the right and left fourth branchial arches, resulting in right and left aortic arches, respectively. […] The right aortic arch is usually larger than the left arch (seen in 75% of patients), but on rare occasions, partial obstruction or complete atresia of the left arch (with a ligamentous remnant) may occur.

• #12 Vascular Ring Double Aortic Arch – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558938/

  Vascular ring refers to a congenital anomaly where vascular structures encircle and compress the trachea and esophagus, leading to respiratory and gastrointestinal symptoms. […] The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist. The ascending aorta and transverse aortic arch compress the trachea, whereas the right aortic arch compresses the esophagus. […] The ascending aorta further divides into right and left transverse arches in DAA. The right transverse arch courses over the right mainstem bronchus, whereas the left aortic arch is over the left. Because the aorta usually descends towards the left side of the body, the right aortic arch goes posteriorly and inserts into it. Further branches originate from the transverse arches: the left common carotid and subclavian from the left aortic arch and the right common carotid and right subclavian from the right arch.

• #13 Vascular Ring Double Aortic Arch | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/31015

  The ascending aorta further divides into right and left transverse arches in DAA. The right transverse arch courses over the right mainstem bronchus, whereas the left aortic arch is over the left. Because the aorta usually descends towards the left side of the body, the right aortic arch goes posteriorly and inserts into it. Further branches originate from the transverse arches: the left common carotid and subclavian from the left aortic arch and the right common carotid and right subclavian from the right arch. The arterial duct is usually left-sided and generally inserts in the left transverse arch or descending aorta.

• #14 Vascular rings and slings – UpToDate

  http://www.uptodate.com/contents/vascular-rings

  Vascular rings are congenital anomalies of the aortic arch that result in compression of the tracheobronchial tree and/or esophagus, leading to respiratory and gastrointestinal symptoms. They can be classified as either complete (when both the trachea and esophagus are fully encircled by a vascular anomaly) or incomplete (without full encirclement of both structures). […] Beginning at the fourth week of embryogenesis, the aortic arch develops from six symmetrical paired aortic arch vessels and the paired dorsal aortae. During the next few weeks of embryogenesis, remodeling and rearrangement of these structures result in the formation of the normal left aortic arch.

• #15 Vascular rings and slings | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vascular-rings-and-slings?lang=us

  Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels. […] Vascular rings can be classified as complete (mostly symptomatic) or incomplete (mostly asymptomatic). […] Causes of vascular rings and slings commonly include double aortic arch (the most common symptomatic vascular ring), incomplete double aortic arch, right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, aberrant right subclavian artery (most common), and pulmonary sling.

• #16 Vascular rings and slings | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vascular-rings-and-slings?embed_domain=hackmd.io%25252F%252540yIPUAFeCSL2JsU8smR5nJQ%25252Fbnjhjgjghjghjgh&lang=us

  Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels. […] Causes of vascular rings and slings commonly include double aortic arch (the most common symptomatic vascular ring), incomplete double aortic arch, right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, aberrant right subclavian artery (most common), and pulmonary sling. […] Vascular rings can be classified as complete (mostly symptomatic) or incomplete (mostly asymptomatic).

• #17 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3

  A ring is complete or true when there is encirclement on all sides, while it is incomplete or partial when at least one side is not involved. […] The ring may be caused by patent vascular structures, in which case the diagnosis is usually straightforward. However, identifying the ring can be challenging when atretic vessels or the ligamentum arteriosum are involved. […] A right aortic arch occurs in 0.1 % of the population. […] A right aortic arch with an aberrant left subclavian artery is caused by a persistent right fourth arch and regression of the left fourth arch in between the left common carotid and left subclavian arteries. […] A right aortic arch with a retroesophageal left subclavian artery, Kommerell diverticulum and left ductus arteriosus is repaired using a muscle-sparing left thoracotomy.

• #18 Vascular rings and slings | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vascular-rings-and-slings?lang=us

  Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels. […] Vascular rings can be classified as complete (mostly symptomatic) or incomplete (mostly asymptomatic). […] Causes of vascular rings and slings commonly include double aortic arch (the most common symptomatic vascular ring), incomplete double aortic arch, right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, aberrant right subclavian artery (most common), and pulmonary sling.

• #19 Multidetector Computed Tomography for Congenital Anomalies of the Aortic Arch: Vascular Rings – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-multidetector-computed-tomography-for-congenital-articulo-S1885585716300779

  The development of multidetector computed tomography has triggered a revolution in the study of the aorta and other large vessels and has replaced angiography in the diagnosis of congenital anomalies of the aortic arch, particularly vascular rings. […] Vascular rings (VR) are congenital developmental abnormalities of the aortic arch consisting of vascular structures that encircle and sometimes compress the airway and esophagus. […] The exact prevalence of VR is unclear because most data have been derived from historical series that collected data on symptomatic patients. […] The most frequent VR are the double aortic arch (DAA) and the right arch with aberrant left subclavian artery (ALSA). […] Congenital heart disease detection has improved in recent decades due to greater diagnostic accuracy in the prenatal and neonatal periods.

• #20 Vascular rings and slings | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vascular-rings-and-slings?lang=us

  Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels. […] Vascular rings can be classified as complete (mostly symptomatic) or incomplete (mostly asymptomatic). […] Causes of vascular rings and slings commonly include double aortic arch (the most common symptomatic vascular ring), incomplete double aortic arch, right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, aberrant right subclavian artery (most common), and pulmonary sling.

• #21 Vascular ring | Iowa Head and Neck Protocols

  https://medicine.uiowa.edu/iowaprotocols/vascular-ring

  All congenital vascular anomalies that cause compression of the trachea and/or esophagus. […] Severity of symptoms and time of presentation are directly proportional to the tightness of the ring. […] The diverticulum is an important factor in causing compressive symptoms. If there is no diverticulum there is possibly a different etiology for breathing or swallowing issues. […] There are many in cardiothoracic literature who do not see this as a compressive entity and are reluctant to operate and treat an aberrant right subclavian artery. […] The decision for surgery is a joint decision between multiple actors. It will often not be clear that dysphagia or airway difficulty are a direct result of vascular compression. Ultimately, cardiothoracic surgery is responsible to the decision to proceed with treatment but it often involves discussions with pulmonology, cardiology, otolaryngology and the family. […] Surgical outcomes are generally very good (70-90% resolution). Dysphagia almost always resolves and persistent problems after initial repair are often related to tracheal anomalies.

• #22 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  A vascular ring is an unusual congenital condition in which the anomalous configuration of the arch, associated vessels, or both surrounds the trachea and esophagus, forming a complete or incomplete ring around them. […] In spite of the significant variability in this group of structural abnormalities of the aortic arch, they all possess a common feature: They all produce some degree of compression on the major airway structures, the esophagus, or both. The location and severity of compression varies with the configuration of the lesion. Individuals with anomalies producing more severe compression are likely to present earlier in life. […] Surgical division of a vascular ring is indicated in all symptomatic patients. To avoid serious complications, surgery should not be delayed, especially in patients with symptoms of airway compression. Individuals who have no symptoms from a vascular ring may not require surgical intervention. Surgical repairs in most cases can be performed successfully and without long-term morbidity.

• #23 Congenital Defects Tutorial – Congenital Heart Defects | Atlas of Human Cardiac Anatomy

  https://www.vhlab.umn.edu/atlas/congenital-defects-tutorial/anomalies-of-arteries-and-veins/vascular-rings-and-slings.shtml

  Anomalies in aortic arch development are classified as vascular rings and slings, many which may cause compression of the airway and lead to stridor and/or respiratory distress. […] Vascular rings include defects in development of the aortic arch that leave the trachea and esophagus completely encircled by structures which are often fibrous, ligamentous remains of previously patent vessels. […] Infants with a double aortic arch often present with stridor, dyspnea, and a barking cough, all which are due to tracheal compression. […] The symptoms of a right aortic arch and aberrant left subclavian artery depend on whether the vascular ring is loose or tight. […] Clinical manifestations of vascular rings can be traced to tracheal and esophageal compression.

• #24 Vascular Rings | Concise Medical Knowledge

  https://www.lecturio.com/concepts/vascular-rings/

  Abnormal embryologic remodeling of the aortic arch system leads to anomalous vascular structures that encircle and/or compress the trachea and esophagus. […] Direct pressure from these aberrant vessels on the airway and esophagus can cause respiratory and/or gastrointestinal symptoms. […] Activities that increase cardiac demand (e.g., feeding, crying) lead to vascular engorgement, worsening the compression of the trachea and esophagus.

• #25 Congenital Defects Tutorial – Congenital Heart Defects | Atlas of Human Cardiac Anatomy

  https://www.vhlab.umn.edu/atlas/congenital-defects-tutorial/anomalies-of-arteries-and-veins/vascular-rings-and-slings.shtml

  Anomalies in aortic arch development are classified as vascular rings and slings, many which may cause compression of the airway and lead to stridor and/or respiratory distress. […] Vascular rings include defects in development of the aortic arch that leave the trachea and esophagus completely encircled by structures which are often fibrous, ligamentous remains of previously patent vessels. […] Infants with a double aortic arch often present with stridor, dyspnea, and a barking cough, all which are due to tracheal compression. […] The symptoms of a right aortic arch and aberrant left subclavian artery depend on whether the vascular ring is loose or tight. […] Clinical manifestations of vascular rings can be traced to tracheal and esophageal compression.

• #26 Vascular Rings | SpringerLink

  http://link.springer.com/10.1007/978-3-662-43588-5_53

  Vascular rings are rare congenital anomalies caused by an anomalous configuration of the aortic arch or associated vessels surrounding the trachea and esophagus to form a complete or incomplete compressing ring around them. […] A common finding in all forms of vascular rings is the increased occurrence of respiratory infections. […] The goal of surgical therapy in patients with a double aortic arch is to divide the smaller of the two arches at a site that does not compromise the blood flow to the head vessels. […] Excellent results have been achieved in recent years without operative mortality in high-volume centers.

• #27 Vascular Rings | Concise Medical Knowledge

  https://www.lecturio.com/concepts/vascular-rings/

  Abnormal embryologic remodeling of the aortic arch system leads to anomalous vascular structures that encircle and/or compress the trachea and esophagus. […] Direct pressure from these aberrant vessels on the airway and esophagus can cause respiratory and/or gastrointestinal symptoms. […] Activities that increase cardiac demand (e.g., feeding, crying) lead to vascular engorgement, worsening the compression of the trachea and esophagus.

• #28 Congenital Defects Tutorial – Congenital Heart Defects | Atlas of Human Cardiac Anatomy

  https://www.vhlab.umn.edu/atlas/congenital-defects-tutorial/anomalies-of-arteries-and-veins/vascular-rings-and-slings.shtml

  Anomalies in aortic arch development are classified as vascular rings and slings, many which may cause compression of the airway and lead to stridor and/or respiratory distress. […] Vascular rings include defects in development of the aortic arch that leave the trachea and esophagus completely encircled by structures which are often fibrous, ligamentous remains of previously patent vessels. […] Infants with a double aortic arch often present with stridor, dyspnea, and a barking cough, all which are due to tracheal compression. […] The symptoms of a right aortic arch and aberrant left subclavian artery depend on whether the vascular ring is loose or tight. […] Clinical manifestations of vascular rings can be traced to tracheal and esophageal compression.

• #29 Case Based Pediatrics Chapter

  http://hawaii.edu/medicine/pediatrics/pedtext/s07c07.html

  In the group of vascular rings and slings as a whole, respiratory symptoms predominate in the initial presentation. The severity of compression determines the severity of symptoms. About 70% to 90% of patients will have respiratory symptoms. Stridor is present in almost all cases. […] The diagnosis of vascular rings is challenging. A high index of suspicion is necessary because of the relatively low incidence of these anomalies in contrast to the common symptoms the patients exhibit. […] Vascular rings are surgically corrected if the patient is symptomatic. If the patient is asymptomatic or has mild symptoms, he/she can be monitored and treated conservatively. Many mild symptoms can resolve with growth. It is however always indicated to surgically correct patients with pulmonary slings, double aortic arch and right arch with a left ligamentum arteriosum upon diagnosis since these patients will progressively become more symptomatic over time.

• #30 Vascular Ring | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vascular-ring

  A vascular ring occurs when a childs aorta the bodys largest blood vessel or its branches form abnormally, so that they encircle and constrict the trachea and usually the esophagus. This compression can cause breathing and swallowing problems. […] Vascular rings are caused by malformed blood vessels. But the vessels themselves arent really the issue, and some people without symptoms may live their entire lives not even realizing that they have a vascular ring. Instead, symptoms occur when a vascular ring puts pressure on a childs esophagus, trachea, or both. […] Normally, the aorta develops from one in a series of symmetrical arches. By the end of the second month of fetal development, the other arches are naturally broken down or formed into arteries. When a vascular ring occurs, certain arches that should have disappeared still remain and form a ring structure.

• #31 32: Vascular Rings and Slings | Veterian Key

  https://veteriankey.com/32-vascular-rings-and-slings/

  A vascular ring is an anomaly of aortic arch development that results in complete encirclement of the trachea and the esophagus by vascular structures. […] The etiology of most vascular rings is unknown. Chromosome 22q11.2 deletions are associated with isolated aortic arch anomalies, including vascular rings, and also with conotruncal cardiac defects and noncardiac abnormalities. […] Vascular rings can cause varying degrees of compression of the trachea and esophagus. Mild tracheal compression may be asymptomatic. More significant involvement in younger patients may manifest as stridor, dyspnea, and a barking cough, all of which are worse during feeding or exertion. […] The diagnosis of vascular rings requires a high index of suspicion because of the relative infrequency of this entity compared with other conditions that cause respiratory distress in children, such as asthma, respiratory infection, and gastroesophageal reflux.

• #32 32: Vascular Rings and Slings | Veterian Key

  https://veteriankey.com/32-vascular-rings-and-slings/

  A vascular ring is an anomaly of aortic arch development that results in complete encirclement of the trachea and the esophagus by vascular structures. […] The etiology of most vascular rings is unknown. Chromosome 22q11.2 deletions are associated with isolated aortic arch anomalies, including vascular rings, and also with conotruncal cardiac defects and noncardiac abnormalities. […] Vascular rings can cause varying degrees of compression of the trachea and esophagus. Mild tracheal compression may be asymptomatic. More significant involvement in younger patients may manifest as stridor, dyspnea, and a barking cough, all of which are worse during feeding or exertion. […] The diagnosis of vascular rings requires a high index of suspicion because of the relative infrequency of this entity compared with other conditions that cause respiratory distress in children, such as asthma, respiratory infection, and gastroesophageal reflux.

• #33 Congenital Defects Tutorial – Congenital Heart Defects | Atlas of Human Cardiac Anatomy

  https://www.vhlab.umn.edu/atlas/congenital-defects-tutorial/anomalies-of-arteries-and-veins/vascular-rings-and-slings.shtml

  Anomalies in aortic arch development are classified as vascular rings and slings, many which may cause compression of the airway and lead to stridor and/or respiratory distress. […] Vascular rings include defects in development of the aortic arch that leave the trachea and esophagus completely encircled by structures which are often fibrous, ligamentous remains of previously patent vessels. […] Infants with a double aortic arch often present with stridor, dyspnea, and a barking cough, all which are due to tracheal compression. […] The symptoms of a right aortic arch and aberrant left subclavian artery depend on whether the vascular ring is loose or tight. […] Clinical manifestations of vascular rings can be traced to tracheal and esophageal compression.

• #34 32: Vascular Rings and Slings | Veterian Key

  https://veteriankey.com/32-vascular-rings-and-slings/

  A vascular ring is an anomaly of aortic arch development that results in complete encirclement of the trachea and the esophagus by vascular structures. […] The etiology of most vascular rings is unknown. Chromosome 22q11.2 deletions are associated with isolated aortic arch anomalies, including vascular rings, and also with conotruncal cardiac defects and noncardiac abnormalities. […] Vascular rings can cause varying degrees of compression of the trachea and esophagus. Mild tracheal compression may be asymptomatic. More significant involvement in younger patients may manifest as stridor, dyspnea, and a barking cough, all of which are worse during feeding or exertion. […] The diagnosis of vascular rings requires a high index of suspicion because of the relative infrequency of this entity compared with other conditions that cause respiratory distress in children, such as asthma, respiratory infection, and gastroesophageal reflux.

• #35 Double Aortic Arch in an Infant With Persistent Stridor

  https://www.patientcareonline.com/view/double-aortic-arch-infant-persistent-stridor

  Double aortic arch-a vascular ring anomaly in which both embryonic aortic arches persist and encircle the trachea and esophagus-is rare. […] Vascular ring anomalies that compress the lower trachea are associated with respiratory symptoms, including wheezing, dyspnea, cough, chest retractions, recurrent respiratory infection, and apnea. […] The severity of symptoms varies with the degree of tracheoesophageal compression. […] Unlike stridor caused by laryngomalacia, stridor associated with vascular ring anomaly does not worsen when the infant is placed in the supine position or when the infant cries and strains. […] Stridor caused by vascular ring anomalies worsens with feeding, similar to tracheomalacia and tracheoesophageal fistula. […] If the clinical picture supports a diagnosis of vascular ring anomaly, several imaging studies may provide confirmation.

• #36 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  A vascular ring is an unusual congenital condition in which the anomalous configuration of the arch, associated vessels, or both surrounds the trachea and esophagus, forming a complete or incomplete ring around them. […] In spite of the significant variability in this group of structural abnormalities of the aortic arch, they all possess a common feature: They all produce some degree of compression on the major airway structures, the esophagus, or both. The location and severity of compression varies with the configuration of the lesion. Individuals with anomalies producing more severe compression are likely to present earlier in life. […] Surgical division of a vascular ring is indicated in all symptomatic patients. To avoid serious complications, surgery should not be delayed, especially in patients with symptoms of airway compression. Individuals who have no symptoms from a vascular ring may not require surgical intervention. Surgical repairs in most cases can be performed successfully and without long-term morbidity.

• #37 Double Aortic Arch in an Infant With Persistent Stridor

  https://www.patientcareonline.com/view/double-aortic-arch-infant-persistent-stridor

  Double aortic arch-a vascular ring anomaly in which both embryonic aortic arches persist and encircle the trachea and esophagus-is rare. […] Vascular ring anomalies that compress the lower trachea are associated with respiratory symptoms, including wheezing, dyspnea, cough, chest retractions, recurrent respiratory infection, and apnea. […] The severity of symptoms varies with the degree of tracheoesophageal compression. […] Unlike stridor caused by laryngomalacia, stridor associated with vascular ring anomaly does not worsen when the infant is placed in the supine position or when the infant cries and strains. […] Stridor caused by vascular ring anomalies worsens with feeding, similar to tracheomalacia and tracheoesophageal fistula. […] If the clinical picture supports a diagnosis of vascular ring anomaly, several imaging studies may provide confirmation.

• #38 Double Aortic Arch in an Infant With Persistent Stridor

  https://www.patientcareonline.com/view/double-aortic-arch-infant-persistent-stridor

  Echocardiography is used liberally in evaluating patients for vascular ring anomalies because it may be adequate for diagnosis and is less invasive than other modalities. […] The consequences of missing a double aortic arch diagnosis vary. […] In infants, tracheal compression may result in tracheomalacia and associated breathing difficulties. […] Older children often present with frequent lower respiratory tract infections because they have difficulty with clearing secretions from below the compressed portion of the trachea. […] In cases of less severe tracheal compression, a diagnosis of double aortic arch anomalies may be made in adulthood. […] The literature also provides exceedingly rare cases of adults who have double aortic arch with presentations that mimic chronic asthma.

• #39 Double Aortic Arch in an Infant With Persistent Stridor

  https://www.patientcareonline.com/view/double-aortic-arch-infant-persistent-stridor

  Echocardiography is used liberally in evaluating patients for vascular ring anomalies because it may be adequate for diagnosis and is less invasive than other modalities. […] The consequences of missing a double aortic arch diagnosis vary. […] In infants, tracheal compression may result in tracheomalacia and associated breathing difficulties. […] Older children often present with frequent lower respiratory tract infections because they have difficulty with clearing secretions from below the compressed portion of the trachea. […] In cases of less severe tracheal compression, a diagnosis of double aortic arch anomalies may be made in adulthood. […] The literature also provides exceedingly rare cases of adults who have double aortic arch with presentations that mimic chronic asthma.

• #40 Vascular Aortic Arch Ring – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560919/

  Aortic arch anomalies can give rise to vascular rings, a rare vascular anomaly with significant health implications. […] The above embryological knowledge is essential in understanding the formation of vascular rings that can arise from various aortic arch anomalies. […] Since all symptoms result from mechanical compression, there is no role for medical treatment, and surgical correction is the only definitive treatment available with excellent clinical outcomes. […] In children with vascular rings, surgical correction is not associated with any mortality. Rare reported cases of mortality in the vascular ring have been associated with other congenital heart disease or underlying pulmonary pathology. […] More than 70% of patients become asymptomatic after one year after surgical correction. However, respiratory symptoms can persist up to four to six months after surgery. […] The need for reoperation is very low (8%), and the most common reasons for it include persistence of diverticulum, circumflex aortic arch, scarring, and tracheobronchomalacia needing aortopexy.

• #41 Double Aortic Arch in an Infant With Persistent Stridor

  https://www.patientcareonline.com/view/double-aortic-arch-infant-persistent-stridor

  Echocardiography is used liberally in evaluating patients for vascular ring anomalies because it may be adequate for diagnosis and is less invasive than other modalities. […] The consequences of missing a double aortic arch diagnosis vary. […] In infants, tracheal compression may result in tracheomalacia and associated breathing difficulties. […] Older children often present with frequent lower respiratory tract infections because they have difficulty with clearing secretions from below the compressed portion of the trachea. […] In cases of less severe tracheal compression, a diagnosis of double aortic arch anomalies may be made in adulthood. […] The literature also provides exceedingly rare cases of adults who have double aortic arch with presentations that mimic chronic asthma.

• #42 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics

  https://tp.amegroups.org/article/view/114864/html

  Vascular rings and vascular-related aerodigestive compression syndromes encompass a variety of lesions that involve abnormal development of the aortic arch with resultant vascular compression of either the trachea, the esophagus, or both. […] Despite the substantial variability in etiologies and presentations, a multidisciplinary approach to diagnostic evaluation has been standardized at Boston Childrens Hospital. […] The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result. […] The principle mechanisms of failure can be broadly categorized into ongoing vascular compression, development of constricting scar tissue reforming the vascular ring, tracheobronchomalacia, or a combination. […] Ongoing vascular compression may be related to an aberrant subclavian artery if the prior operation was merely division of the ligamentum arteriosum to relieve the vascular ring.

• #43 Double Aortic Arch in an Infant With Persistent Stridor

  https://www.patientcareonline.com/view/double-aortic-arch-infant-persistent-stridor

  Echocardiography is used liberally in evaluating patients for vascular ring anomalies because it may be adequate for diagnosis and is less invasive than other modalities. […] The consequences of missing a double aortic arch diagnosis vary. […] In infants, tracheal compression may result in tracheomalacia and associated breathing difficulties. […] Older children often present with frequent lower respiratory tract infections because they have difficulty with clearing secretions from below the compressed portion of the trachea. […] In cases of less severe tracheal compression, a diagnosis of double aortic arch anomalies may be made in adulthood. […] The literature also provides exceedingly rare cases of adults who have double aortic arch with presentations that mimic chronic asthma.

• #44 Double Aortic Arch in an Infant With Persistent Stridor

  https://www.patientcareonline.com/view/double-aortic-arch-infant-persistent-stridor

  Echocardiography is used liberally in evaluating patients for vascular ring anomalies because it may be adequate for diagnosis and is less invasive than other modalities. […] The consequences of missing a double aortic arch diagnosis vary. […] In infants, tracheal compression may result in tracheomalacia and associated breathing difficulties. […] Older children often present with frequent lower respiratory tract infections because they have difficulty with clearing secretions from below the compressed portion of the trachea. […] In cases of less severe tracheal compression, a diagnosis of double aortic arch anomalies may be made in adulthood. […] The literature also provides exceedingly rare cases of adults who have double aortic arch with presentations that mimic chronic asthma.

• #45 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3

  Symptomatic vascular rings are surgically repaired in the first year of life to avoid complications such as hypoxic spells, sudden death, aneurysm, dissection and erosion of the aorta into the trachea or oesophagus. […] CT has emerged as the preferred imaging examination for the diagnosis and characterisation of vascular rings. […] Abnormalities in this sequential pattern of development and regression result in vascular rings mostly caused by incomplete regression of the distal left fourth arch or an aberrant retroesophageal subclavian artery or a ductus arteriosus originating from the descending thoracic aorta contralateral to the aortic arch. […] A vascular ring is defined by encirclement of the trachea and oesophagus by the aorta, arch branch vessels, pulmonary artery, ductus arteriosus or ligamentum arteriosum.

• #46

  https://step2.medbullets.com/evidence/31676999

  A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. […] In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. […] For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.

• #47 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3

  Symptomatic vascular rings are surgically repaired in the first year of life to avoid complications such as hypoxic spells, sudden death, aneurysm, dissection and erosion of the aorta into the trachea or oesophagus. […] CT has emerged as the preferred imaging examination for the diagnosis and characterisation of vascular rings. […] Abnormalities in this sequential pattern of development and regression result in vascular rings mostly caused by incomplete regression of the distal left fourth arch or an aberrant retroesophageal subclavian artery or a ductus arteriosus originating from the descending thoracic aorta contralateral to the aortic arch. […] A vascular ring is defined by encirclement of the trachea and oesophagus by the aorta, arch branch vessels, pulmonary artery, ductus arteriosus or ligamentum arteriosum.

• #48 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3

  Symptomatic vascular rings are surgically repaired in the first year of life to avoid complications such as hypoxic spells, sudden death, aneurysm, dissection and erosion of the aorta into the trachea or oesophagus. […] CT has emerged as the preferred imaging examination for the diagnosis and characterisation of vascular rings. […] Abnormalities in this sequential pattern of development and regression result in vascular rings mostly caused by incomplete regression of the distal left fourth arch or an aberrant retroesophageal subclavian artery or a ductus arteriosus originating from the descending thoracic aorta contralateral to the aortic arch. […] A vascular ring is defined by encirclement of the trachea and oesophagus by the aorta, arch branch vessels, pulmonary artery, ductus arteriosus or ligamentum arteriosum.

• #49

  https://step2.medbullets.com/evidence/31676999

  A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. […] In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. […] For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.

• #50 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics

  https://tp.amegroups.org/article/view/114864/html

  Vascular rings and vascular-related aerodigestive compression syndromes encompass a variety of lesions that involve abnormal development of the aortic arch with resultant vascular compression of either the trachea, the esophagus, or both. […] Despite the substantial variability in etiologies and presentations, a multidisciplinary approach to diagnostic evaluation has been standardized at Boston Childrens Hospital. […] The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result. […] The principle mechanisms of failure can be broadly categorized into ongoing vascular compression, development of constricting scar tissue reforming the vascular ring, tracheobronchomalacia, or a combination. […] Ongoing vascular compression may be related to an aberrant subclavian artery if the prior operation was merely division of the ligamentum arteriosum to relieve the vascular ring.

• #51 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics

  https://tp.amegroups.org/article/view/114864/html

  Vascular rings and vascular-related aerodigestive compression syndromes encompass a variety of lesions that involve abnormal development of the aortic arch with resultant vascular compression of either the trachea, the esophagus, or both. […] Despite the substantial variability in etiologies and presentations, a multidisciplinary approach to diagnostic evaluation has been standardized at Boston Childrens Hospital. […] The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result. […] The principle mechanisms of failure can be broadly categorized into ongoing vascular compression, development of constricting scar tissue reforming the vascular ring, tracheobronchomalacia, or a combination. […] Ongoing vascular compression may be related to an aberrant subclavian artery if the prior operation was merely division of the ligamentum arteriosum to relieve the vascular ring.

• #52 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics

  https://tp.amegroups.org/article/view/114864/html

  For these patients, reoperation with subclavian-to-carotid transposition is standard. […] Failure to recognize a circumflex aorta or midline descending thoracic aorta at the initial operation may result in residual symptoms, and aortic reconstruction may be necessary. […] Development of scar tissue where previously a ligamentum arteriosum or accessory aortic arch has been divided may result in reformation of the previously repaired vascular ring, and this often is apparent on esophagram. […] Finally, the infrequent application of tracheobronchopexy at the time of vascular ring operation appears to be a distinct mechanism of failure. […] In order to avoid these reoperations, our approach at the initial operation has evolved to include liberal use of tracheobronchopexy under bronchoscopic guidance for patients with tracheobronchomalacia and significant respiratory symptoms.

• #53 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics

  https://tp.amegroups.org/article/view/114864/html

  For these patients, reoperation with subclavian-to-carotid transposition is standard. […] Failure to recognize a circumflex aorta or midline descending thoracic aorta at the initial operation may result in residual symptoms, and aortic reconstruction may be necessary. […] Development of scar tissue where previously a ligamentum arteriosum or accessory aortic arch has been divided may result in reformation of the previously repaired vascular ring, and this often is apparent on esophagram. […] Finally, the infrequent application of tracheobronchopexy at the time of vascular ring operation appears to be a distinct mechanism of failure. […] In order to avoid these reoperations, our approach at the initial operation has evolved to include liberal use of tracheobronchopexy under bronchoscopic guidance for patients with tracheobronchomalacia and significant respiratory symptoms.

• #54 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics

  https://tp.amegroups.org/article/view/114864/html

  For these patients, reoperation with subclavian-to-carotid transposition is standard. […] Failure to recognize a circumflex aorta or midline descending thoracic aorta at the initial operation may result in residual symptoms, and aortic reconstruction may be necessary. […] Development of scar tissue where previously a ligamentum arteriosum or accessory aortic arch has been divided may result in reformation of the previously repaired vascular ring, and this often is apparent on esophagram. […] Finally, the infrequent application of tracheobronchopexy at the time of vascular ring operation appears to be a distinct mechanism of failure. […] In order to avoid these reoperations, our approach at the initial operation has evolved to include liberal use of tracheobronchopexy under bronchoscopic guidance for patients with tracheobronchomalacia and significant respiratory symptoms.

• #55 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3

  Vascular rings are congenital abnormalities of the aortic arch-derived vascular and ligamentous structures, which encircle the trachea and oesophagus to varying degrees, resulting in respiratory or feeding difficulties in children. […] A sling is an abnormality of the pulmonary arterial system resulting in airway compression. […] Although several imaging examinations are available for the evaluation of these anomalies, computed tomography (CT) has become the preferred test because of rapid acquisitions, making it feasible to perform the study without sedation or general anaesthesia. […] Imaging plays an important role in the evaluation and management of vascular rings. […] Vascular rings may be associated with congenital abnormalities, particularly conotruncal anomalies (tetralogy, transposition, truncus) and syndromes including 22q11 deletion.

• #56 Vascular Ring Double Aortic Arch | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/31015

  The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist. The ascending aorta and transverse aortic arch compress the trachea, whereas the right aortic arch compresses the esophagus. The right arch is dominant in three-fourths of patients with a double aortic arch. Double aortic arch is sometimes associated with other congenital heart defects, including ventricular septal defects in about 10% of patients, atrial septal defects in about 5% of the patients, tetralogy of Fallot in about 4% of the patients, and rare cases with truncus and transposition of the great vessels. […] DAA’s exact cause is unknown (nor for any other VR). There have been associations of RAA and DAA with 22q11 deletion in the literature. There is some association of DAA with trisomy 21 and trisomy 18. A left aortic arch with aberrant (retro-esophageal) right subclavian artery is not a ring but is highly associated with trisomy 21.

• #57 Vascular Ring Double Aortic Arch | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/31015

  The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist. The ascending aorta and transverse aortic arch compress the trachea, whereas the right aortic arch compresses the esophagus. The right arch is dominant in three-fourths of patients with a double aortic arch. Double aortic arch is sometimes associated with other congenital heart defects, including ventricular septal defects in about 10% of patients, atrial septal defects in about 5% of the patients, tetralogy of Fallot in about 4% of the patients, and rare cases with truncus and transposition of the great vessels. […] DAA’s exact cause is unknown (nor for any other VR). There have been associations of RAA and DAA with 22q11 deletion in the literature. There is some association of DAA with trisomy 21 and trisomy 18. A left aortic arch with aberrant (retro-esophageal) right subclavian artery is not a ring but is highly associated with trisomy 21.

• #58 Vascular Ring Double Aortic Arch | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/31015

  The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist. The ascending aorta and transverse aortic arch compress the trachea, whereas the right aortic arch compresses the esophagus. The right arch is dominant in three-fourths of patients with a double aortic arch. Double aortic arch is sometimes associated with other congenital heart defects, including ventricular septal defects in about 10% of patients, atrial septal defects in about 5% of the patients, tetralogy of Fallot in about 4% of the patients, and rare cases with truncus and transposition of the great vessels. […] DAA’s exact cause is unknown (nor for any other VR). There have been associations of RAA and DAA with 22q11 deletion in the literature. There is some association of DAA with trisomy 21 and trisomy 18. A left aortic arch with aberrant (retro-esophageal) right subclavian artery is not a ring but is highly associated with trisomy 21.

• #59 Case Based Pediatrics Chapter

  http://hawaii.edu/medicine/pediatrics/pedtext/s07c07.html

  Vascular rings encompass only 1% to 3% of all congenital heart disease. Some vascular rings are associated with other congenital heart lesions while others are isolated defects. Tracheobronchial anomalies are rarely seen with vascular rings but are common in pulmonary artery slings. […] The most common symptomatic vascular ring is the double aortic arch. This results from persistence of the right dorsal aorta. The right and left aortic arches encircle the trachea and esophagus. The double aortic arch thus forms a ring around the trachea and esophagus compressing both the trachea and esophagus to a variable degree. […] A pulmonary sling is the left pulmonary artery arising from the right pulmonary artery. It is also known as an anomalous pulmonary artery and results from regression/failure of development of the left pulmonary artery.

• #60 Vascular Ring Double Aortic Arch | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/31015

  The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist. The ascending aorta and transverse aortic arch compress the trachea, whereas the right aortic arch compresses the esophagus. The right arch is dominant in three-fourths of patients with a double aortic arch. Double aortic arch is sometimes associated with other congenital heart defects, including ventricular septal defects in about 10% of patients, atrial septal defects in about 5% of the patients, tetralogy of Fallot in about 4% of the patients, and rare cases with truncus and transposition of the great vessels. […] DAA’s exact cause is unknown (nor for any other VR). There have been associations of RAA and DAA with 22q11 deletion in the literature. There is some association of DAA with trisomy 21 and trisomy 18. A left aortic arch with aberrant (retro-esophageal) right subclavian artery is not a ring but is highly associated with trisomy 21.

• #61 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/

  A vascular ring occurs when the aorta and its branching blood vessels are improperly formed, causing compression of the trachea and esophagus. Vascular rings occur in approximately 1 in 2,500 births, with a higher prevalence in boys. They are usually isolated, but can also happen with other congenital heart defects. Some patients may have associated genetic conditions, the most common of which is 22q11 deletion syndrome (also known as DiGeorge syndrome). […] If surgery is recommended, the cardiovascular surgeon will decide the best approach after reviewing all imaging. Approaches may include: A thoracotomy (accessing the chest wall from the side between the ribs) or A sternotomy (dividing the breastbone to access the chest cavity). Techniques to resect the vascular ring can include takedown of the vessels or ligaments, re-implanting an abnormal subclavian artery, or re-positioning the aorta.

• #62 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/

  A vascular ring occurs when the aorta and its branching blood vessels are improperly formed, causing compression of the trachea and esophagus. Vascular rings occur in approximately 1 in 2,500 births, with a higher prevalence in boys. They are usually isolated, but can also happen with other congenital heart defects. Some patients may have associated genetic conditions, the most common of which is 22q11 deletion syndrome (also known as DiGeorge syndrome). […] If surgery is recommended, the cardiovascular surgeon will decide the best approach after reviewing all imaging. Approaches may include: A thoracotomy (accessing the chest wall from the side between the ribs) or A sternotomy (dividing the breastbone to access the chest cavity). Techniques to resect the vascular ring can include takedown of the vessels or ligaments, re-implanting an abnormal subclavian artery, or re-positioning the aorta.

• #63 A neonatal case of HDR syndrome and a vascular ring with a novel GATA3 mutation | Human Genome Variation

  https://www.nature.com/articles/s41439-019-0087-1

  HDR syndrome (OMIM #146255) is caused by haploinsufficiency of the GATA3 gene. A vascular ring has not been reported in patients with GATA3-associated HDR syndrome. We report a neonatal case of HDR syndrome and a vascular ring that were possibly due to a novel frameshift mutation in the GATA3 gene. […] A vascular ring, which consists of abnormal blood vessels, is a congenital malformation of the aortic arch and its branches surrounding the trachea and esophagus. To date, a vascular ring has not been reported in patients with GATA3-associated HDR syndrome. […] This is the first case report of HDR syndrome with a concomitant vascular ring. The proband had a novel frameshift mutation in the GATA3 gene. This frameshift mutation leads to disruption of the zinc-finger domain and probably causes haploinsufficiency of GATA3.

• #64 A neonatal case of HDR syndrome and a vascular ring with a novel GATA3 mutation | Human Genome Variation

  https://www.nature.com/articles/s41439-019-0087-1

  The relation between GATA3 mutations and a vascular ring remains unknown. […] Therefore, Gata3 mutations may contribute to the structural anomalies of the aortic arch. […] Thus, although the possibility of coincidence cannot be denied, we speculate that haploinsufficiency of GATA3 contributes to the development of a vascular ring as well as symptoms of HDR syndrome.

• #65 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  A vascular ring is an unusual congenital condition in which the anomalous configuration of the arch, associated vessels, or both surrounds the trachea and esophagus, forming a complete or incomplete ring around them. […] In spite of the significant variability in this group of structural abnormalities of the aortic arch, they all possess a common feature: They all produce some degree of compression on the major airway structures, the esophagus, or both. The location and severity of compression varies with the configuration of the lesion. Individuals with anomalies producing more severe compression are likely to present earlier in life. […] Surgical division of a vascular ring is indicated in all symptomatic patients. To avoid serious complications, surgery should not be delayed, especially in patients with symptoms of airway compression. Individuals who have no symptoms from a vascular ring may not require surgical intervention. Surgical repairs in most cases can be performed successfully and without long-term morbidity.

• #66

  https://step2.medbullets.com/evidence/31676999

  A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. […] In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. […] For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.

• #67 Vascular Rings | SpringerLink

  https://link.springer.com/10.1007/978-3-642-38482-0_53-1

  Vascular rings are rare congenital anomalies caused by an anomalous configuration of the aortic arch or associated vessels surrounding the trachea and esophagus, to form a complete or incomplete compressing ring around them. […] The goal of surgical therapy in patients with a double aortic arch is to divide the smaller of the two arches at a site that does not compromise the blood flow to the head vessels. […] In patients with right-sided aortic arch, the ligamentum arteriosum is divided. […] Patients with an anomalous left subclavian artery and Kommerells diverticulum may additionally have resection of the diverticulum and reimplantation of the left subclavian artery to the left carotid artery as a primary operation.

• #68 Vascular Rings | SpringerLink

  http://link.springer.com/10.1007/978-3-662-43588-5_53

  Vascular rings are rare congenital anomalies caused by an anomalous configuration of the aortic arch or associated vessels surrounding the trachea and esophagus to form a complete or incomplete compressing ring around them. […] A common finding in all forms of vascular rings is the increased occurrence of respiratory infections. […] The goal of surgical therapy in patients with a double aortic arch is to divide the smaller of the two arches at a site that does not compromise the blood flow to the head vessels. […] Excellent results have been achieved in recent years without operative mortality in high-volume centers.

• #69 Vascular Rings | SpringerLink

  https://link.springer.com/10.1007/978-3-642-38482-0_53-1

  Vascular rings are rare congenital anomalies caused by an anomalous configuration of the aortic arch or associated vessels surrounding the trachea and esophagus, to form a complete or incomplete compressing ring around them. […] The goal of surgical therapy in patients with a double aortic arch is to divide the smaller of the two arches at a site that does not compromise the blood flow to the head vessels. […] In patients with right-sided aortic arch, the ligamentum arteriosum is divided. […] Patients with an anomalous left subclavian artery and Kommerells diverticulum may additionally have resection of the diverticulum and reimplantation of the left subclavian artery to the left carotid artery as a primary operation.

• #70 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/

  A vascular ring occurs when the aorta and its branching blood vessels are improperly formed, causing compression of the trachea and esophagus. Vascular rings occur in approximately 1 in 2,500 births, with a higher prevalence in boys. They are usually isolated, but can also happen with other congenital heart defects. Some patients may have associated genetic conditions, the most common of which is 22q11 deletion syndrome (also known as DiGeorge syndrome). […] If surgery is recommended, the cardiovascular surgeon will decide the best approach after reviewing all imaging. Approaches may include: A thoracotomy (accessing the chest wall from the side between the ribs) or A sternotomy (dividing the breastbone to access the chest cavity). Techniques to resect the vascular ring can include takedown of the vessels or ligaments, re-implanting an abnormal subclavian artery, or re-positioning the aorta.

• #71 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/

  A vascular ring occurs when the aorta and its branching blood vessels are improperly formed, causing compression of the trachea and esophagus. Vascular rings occur in approximately 1 in 2,500 births, with a higher prevalence in boys. They are usually isolated, but can also happen with other congenital heart defects. Some patients may have associated genetic conditions, the most common of which is 22q11 deletion syndrome (also known as DiGeorge syndrome). […] If surgery is recommended, the cardiovascular surgeon will decide the best approach after reviewing all imaging. Approaches may include: A thoracotomy (accessing the chest wall from the side between the ribs) or A sternotomy (dividing the breastbone to access the chest cavity). Techniques to resect the vascular ring can include takedown of the vessels or ligaments, re-implanting an abnormal subclavian artery, or re-positioning the aorta.

• #72 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/

  A vascular ring occurs when the aorta and its branching blood vessels are improperly formed, causing compression of the trachea and esophagus. Vascular rings occur in approximately 1 in 2,500 births, with a higher prevalence in boys. They are usually isolated, but can also happen with other congenital heart defects. Some patients may have associated genetic conditions, the most common of which is 22q11 deletion syndrome (also known as DiGeorge syndrome). […] If surgery is recommended, the cardiovascular surgeon will decide the best approach after reviewing all imaging. Approaches may include: A thoracotomy (accessing the chest wall from the side between the ribs) or A sternotomy (dividing the breastbone to access the chest cavity). Techniques to resect the vascular ring can include takedown of the vessels or ligaments, re-implanting an abnormal subclavian artery, or re-positioning the aorta.

• #73 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/

  A vascular ring occurs when the aorta and its branching blood vessels are improperly formed, causing compression of the trachea and esophagus. Vascular rings occur in approximately 1 in 2,500 births, with a higher prevalence in boys. They are usually isolated, but can also happen with other congenital heart defects. Some patients may have associated genetic conditions, the most common of which is 22q11 deletion syndrome (also known as DiGeorge syndrome). […] If surgery is recommended, the cardiovascular surgeon will decide the best approach after reviewing all imaging. Approaches may include: A thoracotomy (accessing the chest wall from the side between the ribs) or A sternotomy (dividing the breastbone to access the chest cavity). Techniques to resect the vascular ring can include takedown of the vessels or ligaments, re-implanting an abnormal subclavian artery, or re-positioning the aorta.

• #74 Expertise in the nuances of diagnosis, management and surgery for vascular rings – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/pediatrics/news/expertise-in-the-nuances-of-diagnosis-management-and-surgery-for-vascular-rings/mac-20530282

  Vascular rings are a group of diagnoses present at birth caused by abnormal development of the brachial arch system, which results in a ring of vessels encircling and constricting the airway and esophagus. […] When there are abnormalities in this process, various types of vascular rings occur. […] There is no known cause of or genetic link for vascular rings. […] Surgical intervention is nearly always recommended to release vascular rings. […] Mayo Clinic pediatric and congenital heart surgeons have conducted retrospective studies on pediatric and adult patients who have undergone surgical correction of vascular rings. „We have found that, in general, surgical repairs have excellent outcomes and minimize complications related to chronic airway and esophageal compression,” says Dr. Stephens.

• #75 Vascular Aortic Arch Ring – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560919/

  Aortic arch anomalies can give rise to vascular rings, a rare vascular anomaly with significant health implications. […] The above embryological knowledge is essential in understanding the formation of vascular rings that can arise from various aortic arch anomalies. […] Since all symptoms result from mechanical compression, there is no role for medical treatment, and surgical correction is the only definitive treatment available with excellent clinical outcomes. […] In children with vascular rings, surgical correction is not associated with any mortality. Rare reported cases of mortality in the vascular ring have been associated with other congenital heart disease or underlying pulmonary pathology. […] More than 70% of patients become asymptomatic after one year after surgical correction. However, respiratory symptoms can persist up to four to six months after surgery. […] The need for reoperation is very low (8%), and the most common reasons for it include persistence of diverticulum, circumflex aortic arch, scarring, and tracheobronchomalacia needing aortopexy.

• #76 Vascular Aortic Arch Ring – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560919/

  Aortic arch anomalies can give rise to vascular rings, a rare vascular anomaly with significant health implications. […] The above embryological knowledge is essential in understanding the formation of vascular rings that can arise from various aortic arch anomalies. […] Since all symptoms result from mechanical compression, there is no role for medical treatment, and surgical correction is the only definitive treatment available with excellent clinical outcomes. […] In children with vascular rings, surgical correction is not associated with any mortality. Rare reported cases of mortality in the vascular ring have been associated with other congenital heart disease or underlying pulmonary pathology. […] More than 70% of patients become asymptomatic after one year after surgical correction. However, respiratory symptoms can persist up to four to six months after surgery. […] The need for reoperation is very low (8%), and the most common reasons for it include persistence of diverticulum, circumflex aortic arch, scarring, and tracheobronchomalacia needing aortopexy.

• #77 Vascular Aortic Arch Ring – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560919/

  Aortic arch anomalies can give rise to vascular rings, a rare vascular anomaly with significant health implications. […] The above embryological knowledge is essential in understanding the formation of vascular rings that can arise from various aortic arch anomalies. […] Since all symptoms result from mechanical compression, there is no role for medical treatment, and surgical correction is the only definitive treatment available with excellent clinical outcomes. […] In children with vascular rings, surgical correction is not associated with any mortality. Rare reported cases of mortality in the vascular ring have been associated with other congenital heart disease or underlying pulmonary pathology. […] More than 70% of patients become asymptomatic after one year after surgical correction. However, respiratory symptoms can persist up to four to six months after surgery. […] The need for reoperation is very low (8%), and the most common reasons for it include persistence of diverticulum, circumflex aortic arch, scarring, and tracheobronchomalacia needing aortopexy.

• #78 Vascular Ring Surgical Repair: Re-Implantation of the Left Subclavian Artery into the Left Carotid Artery in a Pediatric Patient

  https://www.mdpi.com/2075-4418/14/16/1736

  Early surgical repair of symptomatic vascular rings is crucial, aiming to relieve the compression while reducing the associated potential complications. […] In the type of vascular ring described (i.e., RAA with aberrant LSA and LLA), the two primary factors causing tracheoesophageal compression are the space-occupying effect of the KD and the sling-like effect of the aberrant LSA. Therefore, to relieve the compression, the surgical repair should aim to divide the arterial ligament, obliterate the KD, as it can be an indication for reoperation, and transfer the LSA to the LCA. […] The surgical repair of vascular rings has a very low risk of morbidity and mortality, with favorable mid- and long-term outcomes. The obliteration of the KD and reimplantation of the LSA achieve good outcomes in eliminating respiratory and esophageal symptoms after the vascular ring repair. […] This case underscores the significance of managing symptomatic vascular rings via early surgical repair and introduces an alternative method for the objective intraoperative assessment of esophageal compression relief in patients with vascular rings.

• #79 Assessment of Surgical Correction at Vascular Ring Anomalies – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/assessment-of-surgical-correction-at-vascular-ring-anomalies/doi/ejcm.18.00389

  Vascular rings involve an abnormal development of aortic arch and branches during embryological development in neonatal period which causes compression on trachea and/or esophagus; resulting in symptoms such as cough, recurrent respiratory infections, wheezing, dysphagia or respiratory distress. […] Once the patient was diagnosed, surgical treatment is required for decompression as a standard therapy. Despite surgical techniques often differ depending on the anatomical features of the disease, several reports indicate satisfactory results with low mortality and event-free long-term outcomes. […] Vascular rings are classified under six main subgroups, which are: double aortic arch occurring due to the persistence of the fourth right aortic arch, right-sided aortic arch + aberrant left subclavian artery developing as a result of persistence of the aortic arch and regression of the fourth left arch between left subclavian artery and the left carotid artery and left aortic arch + aberrant right subclavian artery (ARSA) occurs following regression of the fourth right arch between the carotid and subclavian arteries. […] The goal of the surgery is to relieve the compression over tracheal and/or esophagus by dividing the dislocated vessel while providing sufficient blood flow towards the head. The outcomes after the surgery are satisfactory and lead to uneventful long-term survival.

• #80 Vascular ring | Iowa Head and Neck Protocols

  https://medicine.uiowa.edu/iowaprotocols/vascular-ring

  All congenital vascular anomalies that cause compression of the trachea and/or esophagus. […] Severity of symptoms and time of presentation are directly proportional to the tightness of the ring. […] The diverticulum is an important factor in causing compressive symptoms. If there is no diverticulum there is possibly a different etiology for breathing or swallowing issues. […] There are many in cardiothoracic literature who do not see this as a compressive entity and are reluctant to operate and treat an aberrant right subclavian artery. […] The decision for surgery is a joint decision between multiple actors. It will often not be clear that dysphagia or airway difficulty are a direct result of vascular compression. Ultimately, cardiothoracic surgery is responsible to the decision to proceed with treatment but it often involves discussions with pulmonology, cardiology, otolaryngology and the family. […] Surgical outcomes are generally very good (70-90% resolution). Dysphagia almost always resolves and persistent problems after initial repair are often related to tracheal anomalies.

Zobacz więcej