Vascular ring (patient information)

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 * Associate Editors-In-Chief: Jinhui Wu, MD; Priyamvada Singh, M.B.B.S. [mailto:psingh@perfuse.org]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [mailto:kfeeney@perfuse.org]

Overview
Vascular rings are unusual congenital anomalies that occur early in the development of the aortic arch and great vessels. The abnormal development of the aorta may cause compression or obstruction of the esophagus or airway. Pressure on the trachea and esophagus can lead to breathing and digestive problems. Usual signs and symptoms include high-pitched cough, repeated pneumonia and respiratory infections, respiratory distress, wheezing, choking, difficulty eating solid foods, and vomiting. Tests such as barium esophagram, chest x-ray, chest CT and MRI can show abnormal compression of the middle part of the esophagus characteristic for a vascular ring. Treatments include medicines and surgery. The prognosis of vascular ring depends on the pressure of the vascular ring putting on the esophagus and trachea, how quickly the infant is diagnosed and treated when symptoms appear.

What are the symptoms of vascular ring?
Some children with vascular ring never develop symptoms. However, other cases may show breathing and digestive problems caused by the pressure on the windpipe and esophagus.


 * High-pitched cough
 * Wheezing
 * Respiratory infections
 * Dysphagia
 * Gastroesophageal reflux (GERD)
 * Slow breast or bottle feeding
 * Vomiting

What causes vascular ring?
Vascular ring is rare. It accounts for less than 1% of all congenital heart problems. The condition occurs as often in males as females. Some infants with vascular ring also have another congenital heart problem. Vascular ring occurs very early in the baby's development in the womb. Normally, the aorta develops from one of several curved pieces of tissue (arches). The body breaks down some of the remaining arches, while others form into arteries. Some arteries that should break down do not; this forms vascular rings.

With vascular ring, some of the arches and vessels that should have changed into arteries or disappeared are still present when the baby is born. These arches form a ring of blood vessels, which encircles and presses down on the windpipe (trachea) and esophagus.

Several different types of vascular ring exist. In some types, the vascular ring only partially encircles the trachea and esophagus, but it still can cause symptoms.

Who is at highest risk?
Although the cause of vascular ring is not clear, studies have demonstrated that it is associated with chromosome deletion.

When to seek urgent medical care?
Call your health care provider if your baby has symptoms of vascular ring. Getting diagnosed and treated quickly can prevent serious complications such as damage to the trachea and even death. If one emerges the following symptoms, seeking urgent medical care as soon as possible:
 * Persisting wheezing
 * Dyspnea

Diagnosis

 * Barium esophagram: Barium esophagram may be the most important study in patients with a suspected vascular ring. During the test, the patient drinks a milk shake-like substance called barium. X-ray method called fluroscopy tracks how the barium moves through your esophagus, stomach, and small intestine. Pictures are taken in a variety of positions. Patients may show abnormal compression of the middle part of the esophagus characteristic for a vascular ring.
 * Chest x-ray: Children with vascular ring usually present respiratory difficulty, so chest x-ray is always the first and most commonly performed test. If a child with airway difficulties, respiratory distress or dysphagia appears a right aortic arch on chest radiograph, a vascular ring should be suspected.
 * Chest ct or MRI: A chest CT or MRI can demonstrate the details of the arch anatomy extremely well, such as the positions of vascular, tracheobronchial, and esophageal structures and their relationships to one another.
 * Echocardiography: Echocardiographic studies have been increasingly used for the diagnosis of a vascular ring. It may show an anomalous left pulmonary artery and associated congenital cardiac defects.
 * Cardiac catheterization: This is not a common diagnostic measure for vascular ring. In a catheter room, the doctor threads a thin tube through a blood vessel in the patient's arm or groin to an artery in the heart and injects dye to see the heart and the arteries on an x-ray. Cardiac catheterization can reveal associated cardiac abnormalities are known or suspected.

Treatment options
Treatments for patients with vascular ring are medicines and surgery. Children without symptoms may not need treatment, but need to be carefully observed to make sure the condition doesn't become worse. The goal of medicines, such as antibiotics, is to treat any respiratory tract infections. Surgery is the main treatment for patients with vascular ring. Before treatment starts, talk to your child's doctor about treatment options and your family's preferences on treatment decisions.


 * Diets: Fluid or soft diets may help relieve the digestive symptoms of vascular ring.
 * Medicines: Medicines such as antibiotics are used to treat respiratory tract infections. There are some other drugs used to supply nutrition for the baby and prepare for the surgery.
 * Surgery: The goal of surgery is to convert a restrictive and relieve pressure on the surrounding structures. Surgery needs to perform as soon as possible on children with symptoms. During the surgery, the anesthetist gives medicine to make the child sleepy and comfortable. Then the surgeons make a small cut in the left side of the chest between the ribs, then split the vascular ring.

Diseases with similar symptoms

 * Aortic coarctation
 * Interrupted aortic arch

Where to find medical care for vascular ring?
Directions to Hospitals Treating Vascular ring

What to expect (Outlook/Prognosis)?
Prognosis of vascular ring depends on:
 * The pressure of the vascular ring putting on the esophagus and trachea.
 * How quickly the infant is diagnosed and treated when symptoms appear.
 * Whether the surgery has been performed or not.

Prevention
Because the cause is not clear, the preventive measure is unknown.

Copyleft Sources
http://www.nlm.nih.gov/medlineplus/ency/article/007318.htm http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/rings.htm