Figure 7 : Partial sternotomy incision after closure with a
Blake drain draining the mediastinal and pericardial space and exiting
inferior to the xiphoid process.
Results: A total of 9 patients underwent AA for IAS from 2017
to 2020 (Table 1). The median age was 9 months (IQR 3- 16.5). The male
to female ratio was 1.25 (5/4 patients). The aortic arch was left sided
in 89% (8/9) patients. 2 patients had chromosomal abnormalities (one
each with Trisomy 21 and 18). All patients had > 70%
compression by flexible bronchoscopy. 2 patients had previous surgeries.
One patient had a double aortic arch with an atretic left arch which was
divided previously via left thoracotomy and the other patient had a
trachea-esophageal fistula which was previously repaired via right
thoracotomy. The follow-up was a median of 6 (IQR 4- 8.5) months. The
indications for the operation were: reflex apnea 44% (4/9 patients),
recurrent intubation 44% (4/9 patients), and severe stridor 11% (1/9).
IAS was a technical success (defined ≤ 20% residual stenosis) in 78 %
of the patients (7/9 patients). Complete symptom resolution after an AA
was seen 71% of the patients (5/7 patients) with technical success. Of
the two patients who had incomplete symptom resolution despite a
technical success, one patient had a 20% residual stenosis with mild
stridor but has not had any further episodes of reflex apnea. The other
patient with 15% residual stenosis had mild residual stridor but also
had an associated glottic pathology. AA was unsuccessful in 2 patients
with an initial attempted procedure producing 50% residual stenosis.
One patient had an associated intrinsic tracheal stenosis from isolated
tracheal cartilage deficiency which was not diagnosed preoperatively and
was treated with a tracheal resection and anastomosis using
cardiopulmonary bypass. The other patient had an innominate artery
arising very leftward along the aortic arch and needed a re-implantation
of the innominate artery rightward of the trachea in a more normal
position. Interestingly, this patient also had a right sided upper
partial anomalous pulmonary venous return needing a Warden repair. Both
had an excellent result after the above described procedures. There were
no wound infections, phrenic nerve palsy, pericardial or pleural
effusion in the patients. One patient needed a repeat full sternotomy 6
months after the AA to repair a complete atrioventricular canal defect
at 8 months of age.