Materiały źródłowe

• #1 Vascular Ring | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/vascular-ring

  The following symptoms may be present in children with vascular rings: […] Noisy or labored breathing, especially with eating […] Difficulty eating and swallowing: the child regurgitates milk or other food […] Dysphagia: the child feels like something is stuck in the throat; the child keeps choking or feels like he or she is choking on food […] Persistent cough […] Acid reflux. […] In most cases, the symptoms of a vascular ring are mild. That means the condition may go undiagnosed until your child is older, or even an adult. If the condition is severe and your child has trouble breathing, a vascular ring may be diagnosed at several weeks or months of age. […] Vascular rings require surgery (not open heart) if there are symptoms. In most cases, the surgeon makes an incision on the left side of the chest and goes between the ribs to access the area.

• #2 Vascular Rings: Causes and Symptoms

  https://my.clevelandclinic.org/health/diseases/23947-vascular-rings

  A vascular ring is a congenital heart defect that can cause problems with breathing or eating. […] The condition can cause breathing or eating problems. […] Depending on the type and severity, vascular ring symptoms may appear during infancy, later in life or not at all. […] If the vascular ring affects your (or your baby’s) trachea, you may experience symptoms such as: Cough. Frequent respiratory infections. Nasal flaring (nostrils widen during breathing). Noisy breathing (wheezing or stridor). Respiratory cyanosis (bluish skin). […] If the vascular ring affects your (or your baby’s) esophagus, you may experience symptoms including: Acid reflux. Failure to thrive (failure to gain weight at a normal pace). Feeling like something is stuck in your throat. Trouble eating or frequent choking when eating. Trouble swallowing (dysphagia), especially solid foods. Vomiting. […] Surgery to correct a vascular ring is quite safe and often successful. Although you or your child may have symptoms for a few months afterward, most people are symptom-free within a year of surgery.

• #3 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

  Patients with a true vascular ring would typically present with symptoms of tracheal and/or esophageal compression. […] As a whole, respiratory symptoms, e.g., noisy breathing, stridor, cough, or recurrent upper respiratory infections, are present in 88-94% of patients. Additionally, 43-50% of patients will have esophageal symptoms including dysphagia and choking. […] However, up to 47% to 66% of adult patients with aberrant right subclavian will present primarily with esophageal symptoms. […] A challenge that the clinician may face with respect to eliciting symptoms is that patients may have been labeled as picky or slow eaters or carry a diagnosis of reflux, and so the exact symptomatology may be underappreciated. […] 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.

• #4 Vascular Rings Clinical Presentation: History and Physical Examination

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

  Symptoms and physical findings produced by vascular rings are primarily those of airway or esophageal compression. Individuals with a narrow or tight ring have a significant degree of constriction of one or both of these structures and present very early in life. […] The vast majority of patients with a vascular ring present with symptoms in infancy or very early in childhood. However, a small number of patients do not manifest symptoms until later in life, and others remain entirely asymptomatic. […] Common symptoms include the following: Stridor, Cyanosis, Wheezing, Respiratory distress, Apnea, Characteristic high-pitched, brassy cough. […] Symptoms of airway obstruction predominate in patients who present in infancy or the first few years of life. Dysphagia and symptoms related to the esophagus are the more likely presenting findings in older children and adults with vascular rings.

• #5 Vascular Rings – Seattle Children’s

  https://www.seattlechildrens.org/conditions/vascular-rings/

  Most babies with vascular rings have symptoms because the rings put pressure on their trachea and esophagus. Some children have no symptoms as babies. They may develop symptoms as they get older. […] Pressure on the trachea can cause breathing problems, such as these: Loud breathing (stridor), Working hard to breathe, Wheezing or high-pitched cough, Infections in the lungs (respiratory infections) or repeated pneumonia. […] Breathing problems may get worse for babies when they try to feed or for older children when they eat. […] Pressure on the esophagus can cause feeding problems, such as these: Slow feeding, Trouble swallowing, Trouble eating solid food, Choking, Gastroesophageal reflux, Vomiting.

• #6 8.6 Vascular Rings | Ento Key

  https://entokey.com/8-6-vascular-rings/

  The term vascular ring refers to an aortic arch abnormality in which the trachea and esophagus are surrounded by vascular structures. It may be complete or incomplete. The greater the degree of compression the vascular ring causes, the more severe the symptoms are and the earlier they present. For symptomatic patients, treatment is generally surgical. […] Symptoms depend on the location and degree of vascular compression. Wheezing, stridor, aspiration, cyanotic or apneic attacks, and dysphagia are characteristic. Feeding may exacerbate stridor. Dysphagia is worsened by solid foods. Recurrent respiratory infections such as aspiration pneumonia may also be present. […] Physical findings vary, often in accordance with the patients history. Stridor is characteristically expiratory. It is often associated with cough, tachypnea, and rhonchi. Expiratory, high-pitched wheezes and intercostal retractions can also be appreciated. Patients may hold their neck in hyper-extension to alleviate respiratory distress. Respiratory findings typically do not improve with nebulized bronchodilator treatment and are worsened by exertion. Pulmonary infection may be the presenting symptom, especially in older children.

• #7 Chapter 22 – Vascular Rings | Thoracic Key

  https://thoracickey.com/chapter-22-vascular-rings/

  Vascular rings are a group of rare congenital abnormalities in which the great vessels form a complete ring of vascular structure around the trachea and oesophagus. […] They do not cause cardiovascular symptoms. Their clinical significance and management are entirely related to the compression they cause to the trachea and oesophagus. If they cause no compression, then they do not require treatment. […] Between 50 and 67 per cent of cases present in the first year of life and are most commonly related to tracheal compression causing a stridor or noisy breathing. There may be a history of recurrent chest infections, or the stridor may be picked up on examination during one of these episodes. Younger children may have a characteristic seal-bark cough, and older children may have a chronic non-productive cough due to tracheal irritation or have been mis-diagnosed as asthmatic. Severity of symptoms is variable, but generally, the more severe the compression, the earlier is the presentation. The stridor is not only due to the compression but also due to a degree of tracheo-broncho-malacia of the affected segment of the airway. […] Oesophageal symptoms are less severe, but there may be a history of dysphagia. Infants may have been noticed to have frequent vomiting or drooling of saliva. However, the dysphagia is not usually severe enough to prevent normal nutrition or cause failure to thrive.

• #8 Vascular rings – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vascular-rings/cdc-20389579

  A vascular ring can affect breathing and swallowing. Surgery is usually needed to treat a vascular ring. […] Some people with a vascular ring don’t have symptoms. Sometimes symptoms aren’t noticed until later in life. […] Symptoms of a vascular ring may include: Wheezing. Coughing, especially coughing that is long-term, also called chronic, or doesn’t go away after a cold. Noisy breathing. Shortness of breath with exercise. Heartburn. Frequent respiratory infections. Trouble swallowing. Spitting up. Choking. Difficulty eating. The person may prefer soft foods, eat slowly and chew thoroughly. […] Some people with a vascular ring may be born with other heart conditions. Specific symptoms depend on the types of heart conditions present. […] Congenital heart conditions such as vascular rings often are diagnosed before or soon after a child is born. If your baby has symptoms of a heart condition, call your child’s healthcare professional. […] Call your baby’s healthcare professional if your child has symptoms of a vascular ring, including: Coughing. Wheezing. Noisy breathing. Difficulty feeding and swallowing.

• #9 Vascular ring: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/007318.htm

  Vascular ring is an abnormal formation of the aorta, the large artery that carries blood from the heart to the rest of the body. It is a congenital problem, which means it is present at birth. […] Some children with a vascular ring never develop symptoms. However, in most cases, symptoms are seen during infancy. Pressure on the windpipe (trachea) and esophagus can lead to breathing and digestive problems. The more the ring presses down, the more severe the symptoms will be. […] Breathing problems may include: High-pitched cough, Loud breathing (stridor), Repeated pneumonias or respiratory infections, Respiratory distress, Wheezing. […] Eating may make breathing symptoms worse. […] How well the infant does depends on how much pressure the vascular ring is putting on the esophagus and trachea and how quickly the infant is diagnosed and treated.

• #9 Vascular ring: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/007318.htm

  Surgery works well in most cases and often relieves symptoms right away. Severe breathing problems may take months to go away. Some children may continue to have loud breathing, especially when they are very active or have respiratory infections. […] Delaying surgery in serious cases can lead to serious complications, such as damage to the trachea and death. […] Contact your provider if your baby has symptoms of vascular ring. Getting diagnosed and treated quickly can prevent serious complications.

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

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

  Vascular ring is a congenital aortic arch anomaly where the aorta and surrounding blood vessels develop abnormally, forming a ring-like structure that encircles the trachea and esophagus. A vascular ring can lead to breathing and swallowing difficulties as well as heart problems. […] A child may or may not have symptoms depending on how tight the vascular ring is around the trachea and esophagus. Some children and adults may have no symptoms at all. Others may have symptoms due to airway or esophageal compression. […] Some common symptoms include noisy breathing (stridor), difficulty breathing, frequent respiratory infections with prolonged recovery, chronic cough, difficulty swallowing foods, and poor feeding and/or frequent vomiting. […] At Rady Childrens Hospital, we believe that not all vascular rings need surgical repair, especially if a child is asymptomatic. However, for children experiencing symptoms, the primary treatment is surgery. Surgery is typically successful, and most children recover fully. The goal is to remove the abnormal ring of blood vessels that is compressing the trachea and esophagus.

• #11 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. […] Infants with a double aortic arch often present with stridor, dyspnea, and a barking cough, all which are due to tracheal compression. These symptoms worsen during feeding which may also trigger apnea that lasts seconds or minutes. In cases where a diagnosis is not made during infancy, this defect may be misdiagnosed as asthma and present in older children as a history of chronic cough or wheezing. Effects of esophageal compression are less common but include vomiting, choking, and difficulties in feeding. […] The symptoms of a right aortic arch and aberrant left subclavian artery depend on whether the vascular ring is loose or tight. In cases where the ring is loose, patients may be asymptomatic and a diagnosis may be made only when imaging is performed for unrelated symptoms. In contrast, when the ring is tight, symptoms are identical to those in patients with a double aortic arch. Clinical manifestations of vascular rings can be traced to tracheal and esophageal compression.

• #12 Vascular ring – Wikipedia

  https://en.wikipedia.org/wiki/Vascular_ring

  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.

• #13 What Is A Double Aortic Arch?

  https://my.clevelandclinic.org/health/diseases/23432-double-aortic-arch

  Symptoms usually appear early in infancy (especially in severe cases). But sometimes they can occur by age three or even in the teenage years. […] Respiratory symptoms affect 91% of babies and children with a double aortic arch. These include noisy breathing with stridor (a high-pitched sound) or wheezing. […] Gastrointestinal symptoms affect 40% of babies and children with a double aortic arch. These include choking while being fed and trouble swallowing (dysphagia). […] Heart symptoms affect 30% of babies and children with a double aortic arch. These include chest pain and heart murmur. […] The double aortic arch is the type of vascular ring most likely to cause symptoms. So, your babys healthcare provider will diagnose the issue sooner. And your baby may need surgery sooner after birth compared with babies who have other types of vascular rings.

• #14 Vascular ring – UF Health

  https://ufhealth.org/conditions-and-treatments/vascular-ring

  Some children with a vascular ring never develop symptoms. However, in most cases, symptoms are seen during infancy. Pressure on the windpipe (trachea) and esophagus can lead to breathing and digestive problems. The more the ring presses down, the more severe the symptoms will be. […] Breathing problems may include: High-pitched cough, Loud breathing (stridor), Repeated pneumonias or respiratory infections, Respiratory distress, Wheezing. […] Eating may make breathing symptoms worse. […] Digestive symptoms are rare, but may include: Choking, Difficulty eating solid foods, Difficulty swallowing (dysphagia), Gastroesophageal reflux disease (GERD), Slow breast or bottle feeding, Vomiting. […] How well the infant does depends on how much pressure the vascular ring is putting on the esophagus and trachea and how quickly the infant is diagnosed and treated. […] Surgery works well in most cases and often relieves symptoms right away. Severe breathing problems may take months to go away. Some children may continue to have loud breathing, especially when they are very active or have respiratory infections.

• #15 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. […] Symptoms occur when a vascular ring puts pressure on a childs esophagus, trachea, or both. Symptoms range widely depending on the severity of the compression and can include: Stridor (noisy breathing), Wheezing or cough, Respiratory distress, Difficulty eating when introduced to solid foods, Swallowing difficulties (dysphagia), Gastroesophageal reflux (GERD), Frequent or prolonged respiratory infections. […] Children with vascular rings often also have an airway disorder called tracheomalacia. With this condition, the trachea narrows or collapses when a child exhales, which makes it feel hard to breathe and may lead to a vibrating noise or cough. Tracheomalacia can be present at birth, or it can develop in response to vascular ring or other anomalies.

• #16 HIE Multimedia – Vascular ring

  https://ssl.adam.com/content.aspx?productid=117&isarticlelink=false&pid=1&gid=007318&site=vmfh.adam.com&login=VIRG4865

  Digestive symptoms are rare, but may include: Choking, Difficulty eating solid foods, Difficulty swallowing (dysphagia), Gastroesophageal reflux disease (GERD), Slow breast or bottle feeding, Vomiting. […] How well the infant does depends on how much pressure the vascular ring is putting on the esophagus and trachea and how quickly the infant is diagnosed and treated. […] Surgery works well in most cases and often relieves symptoms right away. Severe breathing problems may take months to go away. Some children may continue to have loud breathing, especially when they are very active or have respiratory infections. […] Delaying surgery in serious cases can lead to serious complications, such as damage to the trachea and death. […] Contact your provider if your baby has symptoms of vascular ring. Getting diagnosed and treated quickly can prevent serious complications.

• #17 Vascular ring | Iowa Head and Neck Protocols

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

  Common initial presenting symptoms variable: Stridor (biphasic), cyanotic episodes, persistent cough, recurrent pneumonia/URI, recurrent vomiting and dysphagia in the first year of life. […] Severity of symptoms and time of presentation are directly proportional to the tightness of the ring. […] Children generally present with symptoms consistent with vascular ring cough and apnea. […] Almost all resolve by age 2 without treatment. […] There are case reports of repair leading to resolution of dysphagia. […] Dysphagia lusoria is a relatively rare condition involving a vascular ring (usually an aberrant right subclavian as this is the most common aortic anomaly). As children these patients are asymptomatic but develop worsening dysphagia later in adulthood. Presumably this is secondary to increasing calcification and blood pressure.

• #18 Vascular Rings Clinical Presentation: History and Physical Examination

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

  Symptoms that are present soon after birth may include slow breast or bottle feeding, fatigue with feeding, frequent regurgitation, and aspiration pneumonias. […] The double aortic arch is the anomaly that usually produces the most severe airway compression in the youngest patients. […] Although patients with associated cardiac lesions may have additional symptoms secondary to that abnormality, symptoms of airway compression related to the constricting vascular ring are usually most prominent. […] Finally, it should be kept in mind that some patients with a complete vascular ring have minimal symptoms or remain asymptomatic.

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

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

  Murmurs, heart failure. […] Completely depend upon the severity of tracheal and esophageal compression from the vascular ring. Infants can develop tracheobronchomalacia secondary to compression that, in turn, leads to „noisy breathing,” which on examination presents as inspiratory stridor and occasionally rhonchi, which worsens with agitation. […] As the severity of compression worsens, respiratory distress/failure signs become more apparent: nasal flaring, intercostal and subcostal retractions, tachypnea, hypoxia, and cyanosis. […] 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. Surgery is indicated only in symptomatic individuals. […] 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.

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

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

  A vascular ring is a condition in which both the trachea and the esophagus are encircled by vascular structures or its remnants (ligamentous remnant, atretic chord like remnant). […] A high index of suspicion is needed to get to a prompt diagnosis of an aortic arch anomaly. A clue to the diagnosis is an infant or a child presenting with a history of multiple visits to the pulmonologist or a gastroenterologist with no definitive diagnosis for the underlying long-standing symptoms. […] Wheezing, stridor, pneumonia, upper respiratory tract infection, and respiratory distress are observed. Cough and respiratory cyanosis can also be seen. Respiratory arrest, choking, and bronchiolitis are rare. Apnea, tracheomalacia, and aspiration are extremely rare. […] Vomiting, failure to thrive, dysphagia, and choking with feeds.

• #20 Case Based Pediatrics Chapter

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

  Double aortic arch, right aortic arch with left ligamentum arteriosum and anomalous pulmonary artery usually present early in infancy whereas left aortic arch/aberrant right subclavian artery may be clinically silent or present in adolescence/adulthood with dysphagia (known as „dysphagia lusoria”). […] Dysphagia is the most common GI symptom, but this typically only occurs after solid foods are introduced and dysphagia symptoms are more pronounced. […] 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. […] Postoperatively, many patients will have respiratory symptoms related to tracheomalacia and airway obstruction. Reoperation is required in 5% to 10% of patients due to recurrent or persistent symptoms. In 95% of patients, surgical correction of the vascular ring is curative.

• #21 Vascular Ring | Thoracic Key

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

  Aberrant right subclavian artery is the most common arch anomaly. Most cases are asymptomatic. When the right subclavian artery arises independently from the descending aorta, it courses behind the esophagus, compressing the posterior aspect of the esophagus and producing mild feeding problems. […] Anomalous left PA (also called vascular sling) is a rare anomaly in which the left PA arises from the right PA. Therefore, both respiratory symptoms and feeding problems (e.g., coughing; wheezing; stridor; episodes of choking, cyanosis, or apnea) may occur.

• #22

  https://link.springer.com/article/10.1007/s11886-022-01764-8

  On the other hand, some patients with a complete vascular ring are oligosymptomatic or asymptomatic and the diagnosis is made incidentally. […] LPA sling is usually diagnosed during infancy, since these children often present with significant respiratory insufficiency. It carries a poor prognosis, and it is often associated with intrinsic congenital tracheobronchial malformations. […] Respiratory symptoms are caused by either external tracheal compression due to the PA sling itself or, more importantly, by intrinsic tracheal stenosis with complete cartilaginous rings. […] The natural history of unrepaired vascular rings was described by Lodeweges et al. They found that the preoperative values for peak expiratory flow (PEF), forced expiratory flow after 25% of forced vital capacity (FVC) expired, and forced expiratory flow after 50% of FVC expired were significantly lower and below normal in patients with onset of symptoms during childhood.

• #23 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. […] Symptoms such as difficulty swallowing, difficulty feeding, or wheezing and coughing can appear early in infancy, especially in those with double aortic arch. In other types of vascular rings, symptoms often don’t appear until a baby starts to eat solid food, and symptoms may not appear until even later in life. […] Respiratory symptoms such as frequent colds or inability to clear a cough after a cold are often viewed to be a normal variant, especially for children in child care, delaying diagnosis. […] Surgical intervention is nearly always recommended to release vascular rings.

• #23 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

  The subgroup of left arch with aberrant right subclavian, technically a sling, rarely causes significant symptoms. These patients usually do not need intervention, but there is a select group in which the anatomy causes significant dysphagia. […] „We have found that, in general, surgical repairs have excellent outcomes and minimize complications related to chronic airway and esophageal compression,” says Dr. Stephens.

• #24 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/v/vascular-ring

  A vascular ring is a type of congenital (present at birth) defect. This happens when normal vessels are in the wrong place. This may cause pressure on the esophagus or airway. […] There are many symptoms seen when vascular rings are present. Some vascular rings are not found before birth. […] Many patients do not have symptoms or have mild symptoms. Vascular rings diagnosed in patients less than 6 months of age are usually a result of a patient having noisy breathing (stridor). Worsening of breathing, difficulties with feeding, or upper respiratory infections may be seen. […] Children with double aortic arch defects show up earlier than those with right aortic arch defects. Swallowing problems are uncommon in the first months of life. But choking or swallowing difficulties are more common in older children as the main symptoms. A vascular ring can be discovered by chance during evaluation of an unrelated problem.

• #25 Treatment of Symptomatic Vascular Rings in the Elderly

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1336722/

  A 76-year-old woman presented with respiratory failure that was later determined to be a result of a right aortic arch with an aberrant left brachiocephalic artery. This vascular ring compressed the trachea, requiring operative intervention. […] Vascular rings are developmental abnormalities involving partial or complete encirclement of the trachea or esophagus by the aortic arch, which can cause pressure symptoms of respiratory distress or dysphagia. Common presenting symptoms include dyspnea on exertion, recurrent pneumonia, bronchitis, stridor, and dysphagia in the event of concomitant esophageal compression. Symptomatic vascular rings require operative intervention. Relief of symptoms follows surgical correction, in most cases. […] To our knowledge, this is the oldest and heaviest patient ever reported with symptomatic presentation and one of only 4 patients over the age of 50.

• #26 Treatment of Symptomatic Vascular Rings in the Elderly

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1336722/

  Dysphagia, rather than respiratory symptoms, is thought to be the most common presenting symptom of a vascular ring in adults. […] The acute presenting respiratory symptoms in our patient demonstrate the crucial need in such cases for airway control, which often necessitates intubation to prevent airway collapse. Furthermore, symptomatic vascular rings require operative intervention, which should not be unduly deferred due to comorbidities or advanced age, or pending extensive radiographic evaluation. […] This rare congenital lesion should be included in the differential diagnosis when a patient presents with respiratory distress or dysphagia. Awareness of vascular rings as a potential cause of these symptoms in the elderly avoids misdiagnosis and treatment delay.

• #27 Vascular Ring | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/vascular-ring

  In some cases, a child has no symptoms of vascular ring and the condition is discovered during an unrelated medical test, procedure or evaluation. Instead of surgery, a Cardiac Center team will monitor the child to make sure symptoms dont appear and the condition isnt harming the child’s health. […] The procedure to correct vascular ring is considered low risk. In cases where there are symptoms as outlined above, surgery is recommended. […] In most cases, children with vascular rings recover completely after surgery and wont need additional procedures. It is not uncommon for noisy breathing to continue for several weeks or months after surgery as the trachea slowly takes on a more normal shape. Sometimes, symptoms such as coughing or labored breathing continue after surgery because the airway is soft (called malacia). We will monitor your child to make sure recovery is as complete and rapid as possible.

• #28 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/v/vascular-ring

  Complete relief of symptoms may show up right away after surgery. […] In infants, there may be some degree of tracheomalacia (floppiness of the trachea). This may be seen during active times or during upper respiratory infections. Improvement of swallowing symptoms in such cases may be seen gradually.

• #29 Vascular Ring Symptoms, Doctors, Treatments, Advances & More | MediFind

  https://www.medifind.com/conditions/vascular-ring/6261

  Some children with a vascular ring never develop symptoms. However, in most cases, symptoms are seen during infancy. Pressure on the windpipe (trachea) and esophagus can lead to breathing and digestive problems. The more the ring presses down, the more severe the symptoms will be. […] Breathing problems may include: High-pitched cough, Loud breathing (stridor), Repeated pneumonias or respiratory infections, Respiratory distress, Wheezing. […] Eating may make breathing symptoms worse. […] Digestive symptoms are rare, but may include: Choking, Difficulty eating solid foods, Difficulty swallowing (dysphagia), Gastroesophageal reflux disease (GERD), Slow breast or bottle feeding, Vomiting. […] How well the infant does depends on how much pressure the vascular ring is putting on the esophagus and trachea and how quickly the infant is diagnosed and treated. Surgery works well in most cases and often relieves symptoms right away. Severe breathing problems may take months to go away. Some children may continue to have loud breathing, especially when they are very active or have respiratory infections. […] Delaying surgery in serious cases can lead to serious complications, such as damage to the trachea and death. […] Contact your provider if your baby has symptoms of vascular ring. Getting diagnosed and treated quickly can prevent serious complications.

• #30 Five things parents should know about vascular rings – Boston Children’s Answers

  https://answers.childrenshospital.org/five-things-parents-should-know-about-vascular-rings/

  While some vascular rings may never cause problems, others can trigger symptoms ranging from mild to life threatening. […] If your child has a high-pitched cough, breathes loudly, wheezes, has difficulty eating or swallowing, or always seems to be coming down with pneumonia or respiratory infections, the problem could be more complex than you realize. […] Not all patients with vascular rings need surgery. In fact, some people without symptoms may live their entire lives not even realizing that they have a vascular ring. More often, however, the condition causes a variety of respiratory and digestive woes. […] For some kids, vascular rings seem to go hand in hand with an airway disorder called tracheomalacia. […] In the long term, it can lead to progressive lung injury. […] Children who have symptoms from vascular rings usually need to surgery to relieve pressure on the airway and esophagus. […] Surgeons may also treat tracheomalacia at this time.

• #31 Double aortic arch: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/007316.htm

  Because symptoms of double aortic arch are often mild, the problem may not be discovered until the child is a few years old. […] The double aortic arch may press on the trachea and esophagus, leading to trouble breathing and swallowing. The severity of the symptoms depends on how much the aortic arch is pressing on these structures. […] Breathing symptoms include: High-pitched sound during breathing (stridor), Noisy breathing, Repeated pneumonias, Wheezing. […] Digestive symptoms may include: Choking, Difficulty eating and swallowing, Vomiting. […] Most children feel better right after surgery, although some may continue to have breathing symptoms for some time after surgical repair. This is most often due to weakness of the trachea because of the pressure on it before surgical repair. […] In rare cases, if the arch is pressing down very hard on the airway, the child can have severe breathing difficulty that leads to death.

• #32 What Is A Double Aortic Arch?

  https://my.clevelandclinic.org/health/diseases/23432-double-aortic-arch

  Babies without symptoms should have surgery at 6 to 9 months of age. Babies with symptoms may need surgery sooner than that, especially if the symptoms are severe. […] After surgery, your baby may have persistent noisy breathing for up to one year. That’s because a double aortic arch prevents your babys trachea from developing properly. So, it takes some more time for the trachea to get stronger. […] Rarely, babies and children have respiratory symptoms more than one year after surgery.

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

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

  Among those with less optimal long-term results are patients with an anomalous left pulmonary artery with and without complete tracheal rings and those with severe associated congenital cardiac defects. […] A number of patients continue to show evidence of some pulmonary function abnormalities years after surgery. Several studies report that measured pulmonary function studies show some degree of airway obstruction in as many as 50% of patients 7-8 years postoperatively.

• #33 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. […] The primary symptomatology associated with vascular rings relates to the structures that are encircled by the ring, chiefly the trachea and esophagus. Prompt diagnosis and treatment of these congenital abnormalities can be lifesaving. […] 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. […] About 95% of patients who undergo surgical correction of a vascular ring survive for long periods, and most of these are soon relieved of their symptoms.

• #34 Vascular ring | Iowa Head and Neck Protocols

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

  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. Persistent post-operative aerodigestive concerns range from mild stridor to tracheostomy and positive pressure ventilation.

• #35 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

  In presence of significant tracheal compression for a long time, there may be irreversible damage to the tracheal structure as well as a localized area of tracheomalacia and cause permanent complaint. Approximately, 70-75% of symptoms disappear in early period following surgery. […] We also had no residual symptoms except for one case suffering from asthma. However, in this patient there was no significant pressure measured on trachea.

• #36 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/86251/html

  Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. […] Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. […] At a median follow-up of 54 months, 20 patients (24%) still experienced some form of symptoms. […] The most common persisting or recurrent respiratory symptoms was asthma (9.4%), followed by stridor (3.5%). […] Overall in-hospital morbidity was acceptable (18%) and consisted mostly in pleural effusions and pulmonary atelectasis. […] In this historical series, the prevalence of KD in RAA and ALSA was 32% and it was resected only if its diameter exceeded 150% of the intra-thoracic left SCA.

• #37 Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound | BMC Pregnancy and Childbirth | Full Text

  https://bmcpregnancychildbirth.biomedcentral.com/articles/10.1186/s12884-023-05637-y

  Early diagnosis of congenital vascular rings is intended to improve the symptoms of compression in patients with airway compression. […] The prognosis is relatively good. […] The earlier the diagnosis of vascular ring disease and the timely surgical treatment of patients with possible compression, the better the prognosis.

• #38 Vascular ring – Knowledge and References – Taylor & Francis

  https://taylorandfrancis.com/knowledge/Medicine_and_healthcare/Cardiology/Vascular_ring/

  A vascular ring is a rare congenital condition in which an anomalous configuration of the aortic arch or associated vessels surrounds the trachea and esophagus, forming a complete compressing ring around them. The primary symptoms associated with vascular rings relate to the structures that are encircled by the ring, namely, the trachea and esophagus. […] Patients with vascular rings tend to present earlier in life and with more severe airway symptoms than those with vascular slings. […] Surgical treatment, almost always necessary, is by dividing the lesser component of the ring, but there is invariably a localized area of tracheomalacia produced by the compression which may persist for months or even years. […] This can cause varying degrees of airway compression. This can be subclinical or clinical, manifesting as acute stridor, severe respiratory compromise or symptoms of chronic airway compression. Early surgical repair of a DAA has a favourable long-term prognosis; therefore prompt detection and intervention are required to prevent long-term morbidity.

• #39

  https://link.springer.com/article/10.1007/s11886-022-01764-8

  Although most patients having a surgical repair of a vascular ring will have symptom resolution or improvement, approximately 5% will develop recurrent symptoms that are clinically significant and will require reintervention. […] The presence of KD in adults with previous diagnosis of vascular ring increases the risk of aortic aneurysm and aortic dissection. […] However, a minor proportion of repaired subjects will present with recurrence of clinically significant symptoms and require reintervention.

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