Dysphagia in an 8-Year-Old Child

Significance Statement

Dysphagia lusoria describes difficulty swallowing from vascular compression of the esophagus. The most common cause is an aberrant right subclavian artery (ARSA) branching from the aortic arch and coursing behind the esophagus. It is most commonly seen in adults. This case describes an 8-year-old boy with dysphagia lusoria from an ARSA. Although rarely seen in pediatrics, clinicians should recognize the unique presentation of this condition and include it in the differential for dysphagia in children.

Report

An 8-year-old boy presented with nasal congestion, snoring, and daytime somnolence. He also had a history of asthma and dysphagia with a preference for soft foods. Physical examination showed tonsillar hypertrophy (grade 3+) but an otherwise normal head and neck evaluation. The child was evaluated by pulmonology, gastroenterology, otolaryngology, speech pathology, and nutrition services. Polysomnography was consistent with mild OSA (obstructive apnea–hypopnea index of 2.3/hr). The child had an esophagram that showed a persistent extrinsic compression on the esophagus at the level of the left-sided aortic arch (Figure 1). MR angiography showed an ARSA (Figure 2).

Figure 1.

XR esophagram demonstrating persistent extrinsic compression on the esophagus at and just above the level of the left-sided aortic arch.

Figure 2.

MR angiography 5 years prior to presentation demonstrating aberrant right subclavian artery in transverse>coronal>sagittal view (top) and sagittal view (bottom).

The child underwent a direct laryngoscopy with bronchoscopy (DLB), esophagogastroduodenoscopy (EGD), and tonsillectomy and adenoidectomy (T&A). DLB showed marked pulsatile posterior compression, and EGD findings were consistent with a vascular ring 19 cm from the incisors with external compression (Figure 3). These clinical, radiological, and endoscopic findings were consistent with dysphagia lusoria due to an ARSA. The patient underwent a T&A with an unremarkable recovery. The plan is to keep the child on a soft diet and undergo a swallow study in 2 months.

Figure 3.

Direct laryngoscopy with bronchoscopy identifying a posterior, pulsatile compression on the tracheal wall (top). Esophagogastroduodenoscopy revealing pulsatile compression of esophagus 19 cm from incisors (bottom).

Anomalies of the aortic arch are found in 3% of autopsies. ¹ ARSA is the most common congenital anomaly of the aortic arch with an incidence of .5% to 2.0%.^1-3 The aortic arch normally has 3 main branches: the brachiocephalic trunk, left common carotid, and left subclavian arteries. The right common carotid and the right subclavian artery branch from the brachiocephalic trunk. Patients with an ARSA have 4 branches from the aortic arch: right common carotid, left common carotid, left subclavian, and ARSA. Despite arising from the left side of the aortic arch, the ARSA supplies blood to the right arm and loops behind the esophagus. This can cause compressive symptoms which lead to dysphagia lusoria, a swallowing disorder resulting from extrinsic compression of the esophagus.^4-6 Dysphagia lusoria can be caused by compression from any vascular structure but is most commonly due to an ARSA. ⁶ This condition was first defined by Bayford in 1787 in a woman with a long history of dysphagia and an autopsy finding of ARSA. ⁴

It is estimated that 60–80% of patients with an ARSA remain symptom-free throughout their life. ¹ When symptomatic, an ARSA typically presents differently in children than adults. Respiratory symptoms such as stridor or recurrent lung infections predominate in children, while dysphagia is more common in adults.^6-8 This dichotomy is thought to be related to the absence of tracheal rigidity in children, leading to more respiratory than esophageal symptoms.^9,10 Adults may be more prone to compressive symptoms on the esophagus from increased rigidity in the esophagus or vessel wall, elongation of the aorta, or formation of an aneurysm.^5,11,12

Children with dysphagia lusoria can present with projectile vomiting and choking, silent aspiration, or reduced interest in eating as seen in the patient described in this report. ¹³ Clinicians should have a higher index of suspicion for dysphagia lusoria in children with known heart or vascular anomalies, even if they present without obvious signs. ¹⁴ The presence of dysphagia in children can result in nutritional deficiencies and requires investigation, ¹³ preferably by a multidisciplinary team. ¹⁵ Dysphagia lusoria is typically diagnosed by an esophagram and confirmed by noninvasive angiography.^1,16 Although upper endoscopy is usually normal, ¹ in this case, diagnosis was confirmed by identifying pulsating masses on EGD and DLB.

Management of dysphagia lusoria in children includes observation with lifestyle changes or surgical management. Surgical repair of an ARSA was traditionally performed by an open approach but now can also be done with endovascular and hybrid techniques.^17,18 Operative management is reserved for severely symptomatic patients.^6,12,19 In this child, a decision was made to proceed conservatively with lifestyle modification and further analysis with a videofluoroscopic swallow study. This will allow for additional examination of swallow function as this patient continues to present with significant food refusals.

Children with dysphagia and congenital heart or vascular abnormalities may benefit from an esophagram to exclude dysphagia lusoria. Evaluation and management require a multidisciplinary approach including otolaryngologists, pulmonologists, gastroenterologists, speech pathologists, and nutritionists. The management of dysphagia lusoria is best decided on a case-by-case basis and can be managed with lifestyle modifications or surgery.