Disease Information for Vascular ring congenital syndrome

Clinical Manifestations
Signs & Symptoms
Dysphagia Swallowing Difficulties
Feeding/swallowing difficulties/infant
Allergic Barking Cough
Bronchospasm signs
Cough Chronic
Cough Dry Non-productive
Cough like seal bark
Cough Whooping Quality
Cyanotic spells
Localized wheeze/unilateral
Nocturnal choking/SOB episodes
Stridor (Inspiratory noise)
Stridor infant Child
Clinical Presentation & Variations
Presentation/Recurrent Bronchitis LRI
Presentation/Recurrent pneumonia Child
Disease Progression
Course/Episodic clusters of attacks
Demographics & Risk Factors
Population Group
Sex & Age Groups
Diagnostic Test Results
Other Tests & Procedures
Bronchoscopy/Abnormal findings
CT Scan
CT Scan/Chest abnormal
Xray/Chest abnormal
X-RAY With contrast
Esophagogram/barium swallow abnormal
Associated Diseases & Rule outs
Rule Outs
Asthma Children
Associated Disease & Complications
Airway obstruction/Children
Apnea, episodic
Breath holding spells/childhood
Lower respiratory infection
Pneumonia, bacterial
Vascular ring congenital syndrome
Asthma Children
Disease Mechanism & Classification
CLASS/Great vessels disorder (ex)
CLASS/Esophagus involvement/disorder (ex)
CLASS/Pulmonic (category)
CLASS/Trachea disorder/lesion (ex)
Pathophysiology/Anomalous vessels block trachea-esophagus
PROCESS/Congenital/developmental (category)
PROCESS/Two/multiple subsets/disease pattern
PROCESS/Anomalies/Deformities/Malformations (EX)
Vascular ring congenital, Synonym/Double aortic arch variant, Synonym/Innominate artery compression synd variant, Synonym/Pulmonary artery sling variant, Synonym/Right aortic arch variant
Drug Therapy - Indication
SX/ENT Surgery

The phrase "vascular ring" was first used by Dr; Robert Gross (Chief of Surgery, Boston Children"s Hospital, 1947 to 1967) in his report describing the first successful division of a double aortic arch in 1945[1] In that manuscript, Gross recalled his observations at the time of an autopsy he performed in 1931; A ring of blood vessels was found encircling the intrathoracic portion of the esophagus and trachea in such a way that the esophagus was indented from behind, whereas the trachea was compressed on its anterior surface; The pathologic findings at once suggested that a division of some part of the so-called vascular ring during life would probably have relieved some of the pressure on the constricted esophagus and trachea;

Since Gross" original description, the phrase "vascular ring" has been used to refer to a collection of congenital vascular anomalies that encircle and compress the esophagus and trachea; In that original report Gross described the two classic vascular rings: right aortic arch with left ligamentum and divided aortic arch (now called double aortic arch); In 1948, Gross reported successful suspension of the innominate artery to the sternum for innominate artery compression syndrome in a 4-month-old infant with wheezing and respiratory distress; In 1954, Willis J; Potts and Paul Holinger (Chief of Bronchology, CMH, 1937 to 1972) coined the term "pulmonary artery sling" when they reported the first successful repair of this anomaly in a 5-month old with wheezing and intermittent attacks of dyspnea and cyanosis;[2] Although innominate artery compression syndrome and pulmonary artery sling are not complete anatomic "rings," they are classified with the classic vascular rings because of the similarities in patient presentation, diagnosis, and surgical therapy; Complete tracheal rings occur in 50% of patients with pulmonary artery sling and hence their inclusion with vascular rings;

Anomaly # Children frequency: Double aortic arch 89,

Right aortic arch, left ligamentum 81, Right aortic arch, right ligamentum 1, Innominate artery compression syndrome 82, Pulmonary artery sling 10, Complete tracheal rings 26,

Ring-sling complex 12


Clinical presentation and diagnosis

The classic symptom of a child with a vascular ring is the "seal-bark" cough; Other symptoms include stridor (noisy breathing), asthma, recurrent pneumonia, and cyanotic spells; Apnea is a common symptom in children with the innominate artery compression syndrome; Children with a pulmonary artery sling and/or complete tracheal rings often have severe respiratory distress requiring emergent intubation and ventilation; Dysphagia tends to occur as a symptom only in older children taking solid foods; Vascular rings are rare in the overall spectrum of diseases that cause respiratory symptoms in children, and their diagnosis requires a heightened index of suspicion; Once the diagnosis of a vascular ring has been made, it is important to resist the temptation to continue obtaining different studies that simply reconfirm the diagnosis; Examinations that may lead to the correct diagnosis include chest radiograph, barium esophagram, bronchoscopy, echocardiogram, CT/MRI scans, and angiography; The chest radiograph can usually establish the location of the aortic arch—normal left, right, or indeterminate (double aortic arch); The trachea should be closely inspected on both the AP and lateral views for tracheal compression; Hyperinflation of the right lung is common in patients with pulmonary artery sling; The barium esophagram is the single most important and reliable technique for making the diagnosis of a vascular ring; It is also relatively inexpensive in the current era of heightened cost awareness; If the child has classic symptoms and a barium swallow that shows typical compression of the esophagus, we can proceed to surgical intervention without further studies; Pulmonary artery sling is the only vascular ring which causes anterior compression of the esophagus with no posterior component; Barium esophagram is normal in patients with innominate artery compression syndrome;

Bronchoscopy is a very important diagnostic tool for infants and young children with stridor; Bronchoscopic examination in a child with a vascular ring demonstrates extrinsic (often pulsatile) compression of the trachea; Bronchoscopy is the diagnostic procedure of choice for infants with complete tracheal rings and innominate artery compression syndrome;Echocardiography is very useful for making the diagnosis of pulmonary artery sling; In our current practice, the patient with complete tracheal rings diagnosed by bronchoscopy is evaluated by echocardiography as the diagnostic procedure of choice to rule out pulmonary artery sling; Computed tomography (CT) and magnetic resonance imaging (MRI) are useful in that they identify both the vascular structures and the tracheo-bronchial anatomy; In patients with either a double aortic arch or a right aortic arch with a left ligamentum, there are four separate bracheocephalic vessels (instead of the normal three) in the superior mediastinum grouped around the trachea; This is called the "four vessel" sign;

Angiography is rarely needed for the diagnosis of a vascular ring, but in unusual cases it can offer information not available from any other studies


External Links Related to Vascular ring congenital syndrome
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)