A 24-year-old woman was referred to pulmonologist with worsening breathlessness and wheeze. During childhood, she was diagnosed with asthma and subsequent exacerbations were treated with bronchodilators for many years. The chest X-ray and a spirometry testing raised a doubt of extrinsic tracheal compression and a subsequent enhanced chest CT (computerized tomogram) scan confirmed a right-sided aortic arch and a vascular ring anomaly compressing the trachea. Standard surgical division of ligamentum arteriosum was able to relieve the trachea and so the symptoms.
Vascular rings are congenital malformations that result from abnormal development of the aortic arch complex and can cause encirclement of the trachea and oesophagus [
We report on an interested case of a young woman who had been treated several years as an asthmatic on clinical background, but, in fact the underlying pathology has been a tracheal compression caused by congenital vascular ring.
A 24-year-old woman came first time to the pulmonary clinic complaining of progressive breathlessness on exertion, chest tightness, audible wheezing, and dry cough for the past few months. Since her late childhood, she was diagnosed as being asthmatic with intermittent exacerbations. She had received inhalational bronchodilators, antibiotics, and several courses of corticosteroids over the past few years but, never subjected to any spirometry testing. The worsening symptoms, in spite of aggressive medical treatment, compelled her to consult a pulmonologist.
Her physical examination revealed mild inspiratory and expiratory wheezes over the upper anterior chest with stridor, though her pulse oximetry showed 100% oxygen saturation on room air. Otherwise, the rest of the physical examination was normal. A routine chest roentgenogram showed absent aortic knuckle on the left side with a right-sided aortic arch and an abnormal distal tracheal air shadow (Figure
A posteroanterior view of chest X-ray showing an abnormal distal tracheal air shadow (arrow) and the absent left aortic knuckle.
(a) Preoperative flow-volume loop demonstrating flattening of the inspiratory and expiratory segments (b) Flow-volume loop, after 8 weeks of surgery, showing slightly improved inspiratory and expiratory phases.
Subsequent, spiral CT of chest with 3-dimensional reconstruction (Figure
A collated picture of chest CT scan with an image of axial view and a reconstructed image with posterior facing showing a right-sided aortic arch, an aberrant origin of left Subclavian artery (Kommerell’s diverticulum) behind the oesophagus which was obscured due to compression.
She underwent a standard left posterolateral thoracotomy, and through the bed of the 4th rib, chest was entered. The ligamentum arteriosum, connecting the origin of the left subclavian artery and the left pulmonary artery, constituted the majority of compression by culprit vascular ring. Its division, over sewn and with subsequent mobilization of the surrounding structures, successfully relieved the constriction. The patient made a remarkable improvement after surgery and continued to improve with gradual disappearance of her exertional breathlessness, wheeze, and stridor. At 8 weeks, she underwent a repeat Respiratory Function Test which has revealed slight improvement as of increase in FVC and FEV1/FVC% and reshaping of flow-volume loop (Figure
Vascular rings compressing the trachea and the esophagus are congenital malformations that result from abnormal development of the embryonic aortic arch complex. The usual presentation is that of respiratory distress in the neonatal period. However, there have been few cases diagnosed in adulthood with varying modes of presentations, such as asthma [
In this case, asthma had been diagnosed since childhood and she received several courses of steroids treatment and bronchodilators. One suspect that she had a number of chest X-ray performed, but it is possible that due to the lack of prominence of the right aortic arch and the low index of suspicion, this anomaly could not be suspected. Unfortunately, she did not undergo any pulmonary function testing prior to pulmonologist consultation which could have given a strong suspicion.
It is always imperative to have anatomical details of aortic anomalies as these could determine the surgical approach for correction. As mentioned earlier, in this woman, the anomalous origin and course of left subclavian artery and the ligamentum arteriosum were the main reason for the extrinsic compression of trachea and esophagus. A left thoracotomy was the approach for correction of this anomaly with immediate improvement in symptoms after surgery.
Computerized tomogram
Forced expiratory volume in first second
Forced vital capacity.
The authors have reported to “Case reports in Vascular Medicine” that there are no financial interests or connections, direct or indirect, or other situations that might raise the question of bias in the work reported or the conclusions, implications, or opinions stated—including pertinent commercial or other sources of funding for the individual author(s) or for the associated department(s) or organization(s), personal relationships, or direct academic competition.
This work did not require any financial funding from any agency or individual.
Thanks to the Department of Pulmonary medicine and Radiology at Al Ahli Hospital, who provided diagnostic modalities to confirm the diagnosis.