An Uncommon Cause for a Preschool Child's Chronic Cough

Beverly P. Giordano, MS, RN, CPNP, PMHS; Sanjeev Y. Tuli, MD; Sonal S. Tuli, MD; Karin Reuter-Rice, PhD, CPNP-AC, FCCM; Terea Giannetta, DNP, RN, CPNP


J Pediatr Health Care. 2014;28(3):267-271. 

In This Article


Vascular rings that compress the trachea were first recognized in the 1930s (Berdon, 2000). The term "vascular ring" refers to any vascular anomaly that partially or completely encircles the trachea and esophagus, causing compression (Shanmugam, Macarthur, & Pollock, 2005). Isolated vascular rings are rare, with a prevalence of approximately 1 of 1000 pregnancies (Bronshtein, Lober, Auslander, & Zimmer, 2005). However, they represent about 1% to 2% of all congenital malformations of the heart and great vessels. DAA is the most common form of vascular ring and results from the persistence of the fourth pharyngeal arches during embryologic development.

Six pairs of pharyngeal arch arteries develop in conjunction with the brachial pouches. The fourth pharyngeal arches form the right and left dorsal aortas. The right dorsal aorta usually involutes, and the left dorsal aorta gives rise to the aortic arch. However, if the right dorsal aorta also persists, the pair of arches meets behind the trachea and esophagus, resulting in a vascular ring that encircles these structures. DAA are grouped into three categories: right dominant, which is the case in more than 75% of patients with DAA; left dominant; or balanced (Backer and Mavroudis, 2000, Bernstein, 2011).

Vascular rings are the most important vascular cause of tracheal obstruction (Berdon, 2000). The symptoms associated with vascular rings involve constriction of the trachea and/or esophagus by the surrounding aortic branches. Children with vascular rings caused by aortic arch anomalies generally present with respiratory distress, dyspnea, stridor, and swallowing difficulties shortly after birth. Respiration may be difficult if the trachea is compressed, resulting in wheezing or other respiratory distress symptoms. With esophageal compression, the DAA causes the esophagus to bulge forward, which indents the trachea and exacerbates respiratory distress. Esophageal compression can also cause regurgitation and aspiration pneumonia. Solid food may not be swallowed easily, and vomiting or choking may occur. Tracheal compression can obstruct air flow and decreased mucociliary clearance of secretions, which can cause recurrent bronchopulmonary infections (Shanmugam et al., 2005). Some children, especially those with loose vascular rings, may be completely asymptomatic or have such mild symptoms that their vascular ring diagnosis is delayed until they are noted to have recurrent respiratory tract difficulties or dysphagia later in life (Bernstein, 2011,Shanmugam et al., 2005).

DAA usually occurs without associated cardiovascular anomalies, but it may occur with ventricular septal defect and tetralogy of Fallot. DAA also is associated with various genetic syndromes (e.g., chromosome band 22q11 deletion in DiGeorge sequence, velocardiofacial or Shprintzen-Goldberg syndrome, and conotruncal anomaly face syndrome;Cury et al., 2013,Jones and Smith, 2005). The patient in this case report did not have facial abnormalities or other cardiac defects.

Management of DAA includes a thorough evaluation of the heart to rule out additional congenital anomalies. Either a CT scan or a barium upper gastrointestinal series is indicated to determine the effect on the trachea and esophagus. Surgical intervention is indicated in all cases with respiratory symptoms or in cases with cardiac or tracheal abnormalities detected on imaging. Surgical intervention involves access to the aorta, usually via a posterolateral approach, although an anterior approach is often needed if additional cardiac anomalies need to be repaired concomitantly (Ruzmetov, Vijay, Rodefeld, Turrentine, & Brown, 2009). The nondominant portion of the vascular ring is ligated and cut. In addition, any additional fibrous bands are divided to completely mobilize the trachea and esophagus. Care must be taken to ensure that the perfusion of the carotid arteries and all limbs are checked after clamping the vessels that are to be divided before they are ligated and cut.

Surgical complications can include bleeding, pleural effusion, and chylothorax. One group of thoracic surgeons who described a 22-year experience of managing patients with vascular rings reported a 10.3% incidence of chylothorax among their patients (Adams & Cipolla, 2011). Thoracic duct leaks close spontaneously in 50% of patients. Management of chylothorax is based on treating the underlying cause, decreasing chyle production, draining and obliterating the pleural space, providing appropriate fluid and nutritional replacement, and providing respiratory care. Chyle production is reduced by either instituting total parental nutrition or having children follow a fat-restricted oral diet supplemented with medium-chain triglycerides. The child in this case study required chest tube drainage, dietary management, and diuretic therapy to resolve her postoperative episodes of chylothorax and pleural effusion.