From the Guidelines
Vascular rings in a 5-month-old infant presenting with wheezing are diagnosed through imaging studies and treated with surgical correction, as indicated by the most recent guidelines from the American Heart Association 1. The diagnosis typically begins with a chest X-ray, followed by more definitive imaging such as CT angiography, MRI, or echocardiography to visualize the abnormal vascular structures compressing the trachea and esophagus.
- Key diagnostic features include:
- Characteristic posterior indentation of the esophagus on barium esophagram
- Tracheal compression confirmed by bronchoscopy
- Once diagnosed, the definitive treatment is surgical division of the vascular ring through a thoracotomy or thoracoscopic approach, as recommended by recent studies 1. Surgery should be performed promptly after diagnosis to prevent ongoing respiratory symptoms and potential complications like tracheomalacia. While awaiting surgery, supportive care includes:
- Positioning the infant upright after feeding
- Using smaller and more frequent feedings
- Treating respiratory symptoms with bronchodilators if needed
- Administering antibiotics if respiratory infections develop Post-surgical follow-up is essential as some children may have persistent respiratory symptoms due to tracheomalacia that can take months to years to resolve, as noted in recent clinical practice guidelines 1. Parents should be educated about potential warning signs requiring medical attention, such as increased respiratory distress or feeding difficulties.
From the Research
Diagnosis of Vascular Rings
- Vascular rings are congenital aortic arch anomalies that can cause compression of the trachea or esophagus, leading to respiratory symptoms such as wheezing, stridor, and cough 2, 3, 4.
- In a 5-month-old infant presenting with wheezing, vascular rings should be considered as a potential diagnosis, especially if other common causes of wheezing have been ruled out 3, 4.
- Diagnostic imaging modalities such as barium esophagogram, echocardiography, computed tomography (CT) with 3-dimensional (3D) reconstructions, and magnetic resonance imaging (MRI) can be used to confirm the diagnosis and clarify the anatomy of the vascular ring 5, 3, 4.
Treatment of Vascular Rings
- Surgical division of the vascular ring is the treatment of choice for symptomatic patients, and early surgical management is effective in relieving symptoms and preventing long-term complications 3, 4, 6.
- The type of surgical approach used may vary depending on the type of vascular ring and the surgeon's preference, with options including open thoracotomy and video-assisted thoracoscopic surgery (VATS) 6.
- VATS has been shown to be a safe and effective approach for certain types of vascular rings, with benefits including shorter operating time and length of stay 6.
Clinical Presentation and Outcomes
- Vascular rings can present with a range of symptoms, including respiratory distress, stridor, wheezing, and dysphagia, and may be associated with other congenital heart defects 2, 3, 4.
- Surgical outcomes are generally good, with low rates of complications and mortality, although some patients may experience persistent symptoms or require long-term follow-up 4, 6.
- Early diagnosis and treatment are important to prevent long-term complications and improve outcomes for patients with vascular rings 3, 4.