The term vascular ring refers to a spectrum of congenital aortic arch anomalies that can cause compressive symptoms of the aerodigestive tract. The estimated prevalence of true vascular rings is 0.05–0.1% of the general population1. The resulting “vascular rings or slings” may represent an uncommon but potentially serious cause of compression of variable degree of the trachea, bronchi, and oesophagus2. In these disorders, the most prevalent type is the aberrant innominate artery (AIA)2–4.
Among respiratory illnesses, which are particularly challenging to diagnose, AIA is important reason for presentation to pediatric emergency departments. But, aberrant IA may misdiagnosis the other respiratory diseases including reactive airway disease due to cause a variety of respiratory symptoms associated with innominate artery tracheal compression. In a clinical situation detected during infancy and which cannot be explained by persistent or recurrent respiratory diseases, this anomaly should be considered.
Most of these anomalies are treated like any other respiratory problem. Therefore, the diagnosis is delayed. Although AIA is common among vascular ring anomalies, it is a little-known and treatable respiratory disease among pediatricians. Especially, late diagnosis of the AIA may cause an increase in mortality and morbidity due to persistence of respiratuar distress after medical therapy, inability to extubate, ventilator and prolonged intensive care unit. Therefore, providing the correct diagnosis with an effective preoperative evaluation is very important for the preoperative and postoperative course of the patient.
When we evaluate our work, our patient’s mean age at operation was 16.3 ± 15.0 months. There was a male dominant among the patients as the rate of 80%. In the study of Wine et al5., patients who needed aortopexy were evaluated and similarly, male predominance was observed with a rate of 65%, and the mean age at operation was found to be 16.8 ± 17.6 months. Again, male dominance was observed in the study by Gardella et al.6 frankly, with these data, it is not clear whether male gender has an effect in patients with aberrant inominate artery disease or only in severely symptomatic patients.
The median body weight of the patients was 8.3 kg (range, 7–14.5 kg) at the time of operation. Unfortunately, there are no studies examining the relationship between body weight and disease in AIA patients. Further studies are needed on this subject in the future, because although respiratory symptoms are prominent in AIA, recurrent infections are likely to affect body weight since it coincides with the rapid growth period in pediatric patients. It can be thought that the decline in body weight may also affect the prognosis of the disease and the success of the surgery.
The most prevalent symptom was a noisy recurrent or chronic dry cough, not well responsive to therapy, present in 92% of the individuals6. There is general agreement that those with respiratory distress, persistent stridor, acute life-threatening events, obstructive apnea, or exercise intolerance may be necessary a more aggressive diagnostic approach and, in case of emergency, followed by surgical correction by means of aortopexy 7,8. Clinical presentation can vary but the severity of respiratory disorders doesn’t correlate with the degree of the anatomical obstruction. The mean percent degree of tracheal stenosis was 0.68 ± 0.22 in our study. Although the degree of TC could not be shown to be related to the clinical situation, the mean percent degree of tracheal stenosis of the patients who underwent aortopexy was found to be 0.7 ± 0.12 in a study and it is high, similar to our result.5,6
Although, AIA can occur as an isolated disorder, it may appear with many other congenital cardiac anomalies. Cardiovascular computed tomography (CT) has emerged as the standard of reference for identification and characterization of AIA. Increased use of MDCT in cardiac imaging highlights detection of such anomalies. Therefore, every patient diagnosed with AIA should be examined in detail whether there is an additional cardiac malformation.
There are several different approaches for aortopexy, including ,lateral thoracotomy, partial sternotomy, and thoracoscopic approach. Nowadays, thoracoscopic approach has become an alternative treatment modality in appropriately selected patients. However, more experiences are mandatory. We use upper ministernotomy, which provide the same exposure of the aorta and its branches as full sternotomy. Surgical treatment is generally the approach of choice for aberrant innominate artery However, the impact of surgical approach on the survival of patients is still uncertain.
Other than aortopexy, the most commonly performed procedure for innominate artery tracheal compression is innominate artery reimplantation, which was first described in 1992 by Hawkins et al.9 Grimmer et al10 reported a shorter hospital stay after innominate artery reimplantation (mean, 6.5 days; median, 3 days) in 2009. On that date, the only description of length of hospitalization after aortopexy in the literature was 16 days.11 Wine et al.5 found a mean length of hospitalization of 2.2 days following aortopexy. This finding emphasized the short hospital stay required by aortopexy, and with these results gave more confidence to physicians who opted for aortopexy. In our study, such a significant short-term hospital stay was not observed. The median PICU and hospital stay is found 2.5 days (1– 4 days), and 5 days (3–8 days). The median (range) MV duration was 2 hours (0–3 hours). Although there is no data on this subject after aortopexy applied for isolated AIA, in a study evaluating children who underwent aortopexy for tracheobronchomalacia, the mean MV duration was found to be 2 days.12 The reason for the short duration of mechanical ventilation may be the effective use of non-invasive ventilation in our center. Because it was determined that half of our patients used non-invasive ventilation in the post-extubation period.
Various data were used in studies to assess whether patients benefited from aortopexy, such as improvement in tracheal stenosis, spirometry results, or questionnaires assessing quality of life affected by respiratory status. In general, these studies report complete response rates ranging from 57–100%. Nonresponders were limited from 0–25%. These data showed that aortopexy was highly beneficial.5,6,13,14 In our study, instead of these, we evaluated pre- and postoperative emergency department admission and hospitalization number per month due to respiratory problems. We thought that in this way, we could better understand the reflection of aortopexy on the clinical situation. The mean number of emergency service application and hospitalization per month at the postoperative period were significantly lower than the mean number at the preoperative period. In addition, the higher the preoperative emergency admission, the higher the postoperative PIM score was. Furthermore, it was observed that there were more frequent preoperative emergency admission at a younger age.
The limitations of our study are that it had a retrospective design and was conducted in a single center. The other limitations of the study are the small number of patients.
AIA may lead to a variety of respiratory manifestations, being observed between infants and children. It is known that these recurrent respiratory problems experienced at a young age can leave permanent effects. When choosing treatment, it should be considered that aortopexy is a safe and effective treatment method, and it significantly reduces recurrent respiratory problems independent of the degree of tracheal compression.