Double Aortic Arch in an Infant With Persistent Stridor

Double aortic arch-a vascular ring anomaly in which both embryonic aortic arches persist and encircle the trachea and esophagus-is rare, presenting in just 1 of 1000 pregnancies.¹ The presentation of vascular rings often is nonspecific, including respiratory and GI features, which complicates the diagnosis.^2,3

In this case report, we present an infant who has a double aortic arch vascular ring anomaly. We emphasize the importance of maintaining a high level of clinical suspicion for vascular ring when evaluating an infant with the symptom of persistent stridor. Recognizing persistent stridor early and treating the underlying cause can, indeed, optimize outcomes.

Case

A 3-day-old boy presented to the primary care office for evaluation of what the child’s mother described as “strange breathing.” He was full-term, born by vaginal delivery without complications. His mother had no significant past medical history and had not taken any medication during the pregnancy.

On examination, he was noted to have persistent stridor. His mother noted that the noise was present during the hospital stay and had not changed in character. She also noted that the noise occurs on inspiration and amplifies when the infant is feeding or crying.

The infant is breast-fed and has no difficulty with choking, swallowing, or reflux during feeds. The findings of the physical examination were unremarkable.

We recommended that the patient be evaluated at a local pediatric hospital for symptoms of persistent stridor at rest.

Discussion

The most common causes of chronic stridor in infants are laryngeal and tracheal diseases.^4,5 More than 90% of infants with stridor have laryngomalacia, a benign, congenital condition typified by a “floppy” supraglottis that collapses inward on inspiration.⁶

Infants with laryngomalacia do not need to be hospitalized. Identifying those who have more serious presentations requires locating the obstruction in the airway, determining the onset of stridor, and assessing for the presence of key associated symptoms.⁷

Airway obstruction above the glottis, as with laryngomalacia, presents as inspiratory stridor; obstruction of the trachea, as with a vascular ring anomaly, presents as expiratory stridor. Biphasic stridor may be attributed to a glottic or subglottic lesion.⁷

The onset of stridor symptoms also is telling. Congenital lesions, such as vascular ring, typically cause stridor that is present from birth. However, laryngomalacia presents later in postnatal life, at about age 4 to 6 weeks.⁷

Vascular ring anomalies that compress the lower trachea are associated with respiratory symptoms, including wheezing, dyspnea, cough, chest retractions, recurrent respiratory infection, and apnea.^4,8 Vascular rings also can compress the esophagus, causing dysphagia, vomiting, feeding intolerance, and other symptoms.^3,8 The severity of symptoms varies with the degree of tracheoesophageal compression.⁹

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.⁷ Stridor caused by vascular ring anomalies worsens with feeding, similar to tracheomalacia and tracheoesophageal fistula.⁷

Figure 1.

The double aortic arch impinges on neighboring structures to produce a symptom profile marked by respiratory and GI complaints. Esophageal indentation resulting from double aortic arch anomaly may be recognized in plain, lateral radiographs (left). Bronchoscopy may demonstrate indentation of the trachea (right).

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