On hospital Day 5, barium esophagogram reveals extrinsic compression of the esophagus. To identify the precise cause of compression, chest magnetic resonance angiogram reveals that Ms. B has a right-sided aortic arch with an aberrant left subclavian artery at T4 that, with the ligamentum arteriosum and left pulmonary artery, form a vascular ring impinging around the esophagus.
The authors’ observations
Dysphagia lusoria, coined in 1789 from the root for “natural abnormality,”5 is caused by an aberrant right subclavian artery, which persists because of abnormal involution of the right fourth aortic arch during embryogenesis6 (Figure 1). The condition is diagnosed incidentally in most affected adults, who are asymptomatic throughout life and do not require operative management.6
Symptoms of dysphagia lusoria can include dysphagia and recurrent aspiration if significant esophageal impingement is present.7 It is thought that patients tend to show more symptoms as they age because of sclerosis, aneurysm, or atherosclerosis of the impinging vessel.7 Cough and dyspnea caused by impingement of the trachea also have been reported, and might be more common in children because of tracheal flexibility.5
This case illustrates the complex interrelationship of physical and psychiatric conditions. After the treatment team discovered a physical cause of Ms. B’s symptoms, the initial psychiatric diagnosis became problematic, but remained critically important for long-term treatment of her comorbid eating phobia.
TREATMENT Therapy, surgery
The treatment team is faced with the question of whether dysphagia lusoria fully accounts for Ms. B’s status. The anatomic anomaly might explain the initial choking incident if the food particle was lodged at the site of impingement, but does not account for development of a severe acute eating disorder and subsequent malnutrition.
Because of the presence of dysphagia lusoria, the team concludes that Ms. B does not meet diagnostic criteria for ARFID. She is given a diagnosis of unspecified eating disorder.
Ms. B is discharged and referred to a pediatric cardiothoracic surgeon for operative consult of symptomatic dysphagia lusoria. By discharge, she is successfully eating yogurt with fruit chunks, which she had rejected earlier. She also expresses optimism about eating cake at her birthday party, scheduled for 2 weeks after discharge.
The treatment team strongly recommends outpatient psychiatric follow-up to manage Ms. B’s unspecified eating disorder with cognitive-behavioral therapy and exposure and response prevention, which helped to mildly decrease her anxiety during the hospital stay.
Ms. B’s surgeon deems the case severe enough to warrant surgery. Surgery for dysphagia lusoria can be indicated to prevent progression of symptoms into adulthood8; options include division of the ligamentum arteriosum to loosen the vascular ring and re-implantation of the aberrant subclavian artery.9,10 (On the other hand, dietary modification might be therapeutic in patients whose symptoms are mild.5)
The surgeon elects to divide the ligamentum arteriosum to relieve the pressure of the vascular ring on the esophagus. Intraoperatively, he discovers that Ms. B has a mildly patent ductus arteriosus (PDA) (Figure 2), which is more common in premature infants than in full-term births. The PDA is clipped on both sides and divided. This immediately causes the vascular ring created by the aberrant left subclavian artery, right-sided aorta, and PDA to spring open, releasing pressure on the esophagus.
The aberrant left subclavian artery emerges from an abnormal bulging of aorta, known as Kommerell’s diverticulum. After the vascular ring is released, the diverticulum is not observed to impinge on the esophagus; however, as a preventive measure, the surgeon sutures it to the anterior spinous ligament. This will prevent the diverticulum from enlarging and impinging on the esophagus as Ms. B grows to adulthood.
The surgery is completed without complications. Ms. B tolerates the procedure well.
Ten days after surgery, Ms. B is recovering well. She and her mother report satisfaction with the procedure to release the vascular ring. She discontinues her pain medication after 2 days and slowly begins reintroducing solid foods. Her fear of dysphagia and choking rapidly diminish.
Bottom Line
The diagnosis and management of eating disorders, including avoidant/restrictive food intake disorder and choking phobia, can be challenging. Furthermore, if an anatomical or organic anomaly is found, it is important to question how a patient’s eating disorder can be managed best through interdisciplinary collaboration between medical and behavioral specialties.
Related Resources
• Bryant-Waugh R. Feeding and eating disorders in children. Curr Opin Psychiatry. 2013;26(6):537-542.
• Norris ML, Robinson A, Obeid N, et al. Exploring avoidant/restrictive food intake disorder in eating disordered patients: a descriptive study. Int J Eat Disord. 2014;47(5):495-499.
Disclosures
The authors report no financial relationship with any company whose products are mentioned in this article or with manufacturers of competing products.