B. Vascular Ring
Discussion
Vascular rings are a result of congenital anomalies of the aortic
arch or other large vascular structures which result in the compression
of the tracheobronchial tree and/or of the esophagus. Vascular rings are
classified as complete (involving both the trachea and esophagus) or
incomplete (involving either the trachea or esophagus). The most common
types of complete vascular rings are the double aortic arch and the
right aortic arch. The child in this case was diagnosed with a right
aortic arch, with posterior indentation of the esophagus by the aberrant
left subclavian artery clearly visualized in the figure.
Children with vascular rings may present with variable symptoms such
as wheezing outside of respiratory infections, noisy breathing, stridor,
cough, or dyspnea. Physical examination may show signs of tachypnea,
intercostal retractions, nasal flaring, or stridor. The diagnostic
work-up typically begins with a chest radiograph to identify anatomic
abnormalities, but compression of the trachea and/or esophagus may be
difficult to identify. Further imaging may include a barium swallow or
esophagram (can help identify both types of complete vascular rings), in
addition to computed tomography (CT) with angiography for further
evaluation of vascular structures. Treatment involves surgical
correction. The surgical approach depends on the specific type of
vascular ring. For this particular patient, the surgical approach was
via a left thoracotomy and the ductus or ligamentum was identified and
divided.
Bronchomalacia and croup do not present with dysphagia; while croup
can present with nocturnal stridor, our child did not have the sudden
onset and infectious symptoms typically occurring with this diagnosis
(choices A and D are incorrect). Our patient did not respond to
bronchodilator therapy, making asthma an unlikely the diagnosis (choice C
is incorrect). Obstructive sleep apnea can be a comorbidity with
asthma, airway malacia, croup, and vascular rings; while OSA may
contribute to noisy breathing during sleep, it would not explain the
dysphagia reported.
References
- Davies M, et al. Developmental abnormalities of the great vessels of
the thorax and their embryological basis. Br J Radiol 2003; 76: 491.
- Licari A, et al. Congenital Vascular Rings: a clinical challenge for the pediatrician. Pediatr Pulmonol 2015; 50: 511.
- Fulton et al. Vascular Rings and Slings.
