Vascular rings are a rare cause of obstructive lung disease. We report a case of a double aortic arch that was mistaken for asthma and did not manifest itself until adulthood.
A 35-year-old female with history of refractory asthma treated with systemic corticosteroids for a decade presented with worsening dyspnea. She did not have childhood asthma but had multiple hospitalizations for bronchitis and pneumonia. She was diagnosed with asthma in her early twenties with spirometry showing severe obstruction with a bronchodilator response of 19% and 420cc in FEV1. Her prolonged treatment with steroids led to significant weight gain, sleep apnea, and pregnancy induced diabetes. She remained dyspnic despite bonchodilators so a spiral CT was done to evaluate for pulmonary embolus. The CT showed a double aortic arch resulting in compression of the trachea and the esophagus. Subsequent spirometry showed blunting of both the inspiratory and expiratory limb of the flow volume loop. The patient was referred to cardiothoracic surgery for definitive treatment.DISCUSSION: This case is unique for a vascular ring because of the delayed onset of symptoms in adulthood. This case of a vascular ring in an adult presented with wheezing rather than dysphagia. Delayed diagnosis of the vascular ring caused significant morbidity in the form of recurrent dyspnea and side effects from systemic corticosteroids.CONCLUSIONS: Upper airway obstruction is one of the differential diagnoses of patients presenting with obstructive airway disease. Vascular rings frequently manifest during childhood although some people are incidentally diagnosed on autopsy. The primary presenting symptom of older children and adults with a vascular ring is dysphagia . Barium has been the primary method of diagnosis of vascular rings in the past, but newer imaging modalities such as CT and MRI are also effective. Definitive treatment is surgical repair.
T.P. Stern, None.