A strident girl.Arch Dis Child Educ Pract Ed. 2019 Oct 25 [Online ahead of print]AD
A 12-year-old girl was referred with a 7-month history of episodes of dyspnoea, stridor and a sense of chest constriction during physical exercise. These were self-limiting and never occurred at night. Physical examination was normal. An initial diagnosis of vocal cord dysfunction was made.Spirometry showed a plateau of forced expiratory flow, with a truncated aspect of the expiratory phase (figure 1). Six weeks later she described occasional dysphagia with solid foods. The barium swallow, performed only in anteroposterior projection, did not show oesophageal dilation. A bronchoscopy showed extrinsic compression of the middle lower third of the trachea and the right main bronchus. A chest CT scan was performed (figures 2 and 3).edpract;archdischild-2019-317625v1/F1F1F1Figure 1The spirometry showed a truncated expiratory phase with a substantially decreased PEF, compared with FEV1: indicating central intrathoracic airway obstruction. FEF, forced expiratory flow; FEV1, forced expiratory volume in 1 s; FIF, forced inspiratory flow; FIV1, forced inspiratory volume in 1 s; FIVC, forced inspiratory vital capacity; FVC, forced vital capacity; PEF, peak expiratory flow; PIF, peak inspiratory flow.edpract;archdischild-2019-317625v1/F2F2F2Figure 2Contrast enhanced CT axial section image showing right aortic arch (white arrow on the left) with aberrant subclavian artery (red arrow on the right) encircling the trachea and the oesophagus.edpract;archdischild-2019-317625v1/F3F3F3Figure 3CT three-dimensional reconstruction arteriography posterior view showing right aortic arch (white arrow), diverticulum of Kommerell (white star) from which the left subclavian artery (red arrow) arose. D Ao, descending aorta. QUESTIONS: What is your diagnosis?Persistent vocal cord dysfunctionAchalasiaVascular ringAsthmaWhat is the gold standard for diagnosis of VR?ECGChest radiographCT and/or MRIBronchoscopyHow should this patient be treated?Surgical correctionVideo-assisted thoracoscopyDecompression of the oesophagus with a nasogastric tubeInspiratory muscle training and ipratropium bromide inhalerWhat signs in the history pointed away from vocal cord dysfunction?Dysphagia with solid food was present.The episodes of dyspnoea and stridor never occurred at night.The episodes arose mainly on exertion.The episodes of dyspnoea and stridor were self-limiting. Answers can be found on page 2.