OBJECTIVE: The objective was to measure health-related quality of life (HRQoL) of children following treatment of all-cause tracheomalacia with aortopexy.
METHODS: Children ≥5 years and parents of children <18 years who had undergone aortopexy completed the Paediatric Quality of Life Inventory (PedsQL4.0). Scores were compared to published norms.
RESULTS: Completed questionnaires were received from 35 parents (65%) and 10 children (38%). Median age at aortopexy was 9.8 months (1 month-12.7 years) and median years of follow-up was 2.6 (4 months-6.9 years). Children who completed questionnaires had a median age of 8.4 (5.7-13.4) years. Parent and child-reported total PedsQL scores were 69.61 (SD : 19.74), and 63.15 (SD : 20.40) respectively. Half of parents and 80% of children reported scores suggesting poor HRQoL outcomes. Parent-reported total, physical and psycho-social scores were lower than those of healthy children and those with acute illness but comparable to children with chronic health conditions and cardiovascular disease. Similarly, children themselves reported comparable total scores to children with chronic illness but child-reported psycho-social scores were lower in the aortopexy group than any other group. There was no association between PedsQL scores and cause of malacia, age or time since aortopexy. The presence of complex congenital comorbidities had a significant (p < 0.05) impact on HRQoL scores.
CONCLUSIONS: Following aortopexy children remain at risk of poor HRQoL, especially those with complex comorbidities. HRQoL reported by both parent and child provides important insight into the lives of children following this procedure. Further longitudinal and qualitative study are required to better understand this complex group.

UNASSIGNED: Innominate artery compression syndrome (IAS) is caused by an abnormally originating innominate artery compressing the trachea anteriorly. One option to relieve such compression is an anterior aortopexy (AA). We describe our technique of an AA via a partial upper median sternotomy.
UNASSIGNED: Nine consecutive patients underwent AA for IAS via a partial upper median sternotomy from July 2017 to November 2020 at two US teaching hospitals. The median age was 9 months [interquartile range (IQR), 3-16.5 months]. The male to female ratio was 1.25. All patients had >70% compression by flexible bronchoscopy. Two patients had previous surgeries. The median follow-up was 6 months (IQR, 4-8.5 months). The indications for the operation were: acute life-threatening events (ALTEs) (4/9 patients), recurrent intubation (4/9), and severe stridor with >70% luminal reduction (1/9). Technical success (defined as ≤20% residual stenosis) was achieved in 78% (7/9) of the patients. The two patients with unsuccessful AAs required either a tracheal resection or an innominate artery reimplantation. Both achieved full symptom resolution. Overall, 78% (7/9) of patients experienced full symptom resolution. Of the two patients without full symptom resolution, one had mild stridor at 6 months post-operation. The other patient without full resolution is awaiting further vocal cord surgery for an associated glottic pathology.
UNASSIGNED: A partial upper sternotomy provides a very versatile approach to an AA for IAS. In addition to facilitating an adequate AA, a partial upper sternotomy provides options for direct tracheal surgery or an innominate artery reimplantation in case an optimal result is not obtained by an AA.

OBJECTIVE: The objective was to report and analyse the characteristics and results of open aortopexy and thoracoscopic aortopexy for the treatment of airway malacia in a paediatric population.
METHODS: We report a retrospective consecutive case series of paediatric patients undergoing aortopexy for the treatment of airway malacia at a quaternary referral centre between December 2006 and January 2021. Outcome measures included days to extubation, continued need for non-invasive ventilation, further intervention in the form of tracheostomy and death.
RESULTS: 169 patients underwent aortopexy: 147 had open procedures (135 via median/limited median sternotomy and 12 thoracotomy) and 22 thoracoscopic. Mean follow up was 8.46 yrs (range 1-20 yrs). Most common site of airway malacia was the trachea (n = 106, 62.7 %), and 48 (28.4 %) had additional involvement at the bronchi with tracheobronchomalacia (TBM). 15 (8.9 %) had bronchomalacia (BM) only. Incidence of bronchial disease was lower in the thoracoscopic than open group (13.6 % vs 40.82 %; p < 0.0001). Mean time to extubation was 1.45 days, 2.59 days, 5.23 days in tracheomalacia, TBM and BM groups, respectively (p = 0.0047). Mean time to extubation was 1.35 days, 2 days, 3.67 days, and 5 days in patients with external vascular compression, TOF/OA, primary airway malacia, and laryngeal reconstruction, respectively (p = 0.0002). There were 21 deaths across the cohort, and all were in the open group. 71.4 % (n = 15) had bronchial involvement of their airway malacia.
CONCLUSIONS: Open and thoracoscopic aortopexy are effective treatments for airway malacia in children. We have identified that involvement of the bronchi is a risk factor for adverse outcomes, and the optimum treatment for this patient cohort is still debatable.
METHODS: IV.
METHODS: Retrospective Study.

BACKGROUND: Following surgical correction, many patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) present to the emergency department (ED) with acute airway complications. We sought to determine the incidence and risk factors for severe acute life-threatening events (ALTEs) in pediatric patients with repaired congenital EA/TEF and the outcomes of operative interventions.
METHODS: A retrospective cohort chart review was performed on patients with EA/TEF with surgical repair and follow-up at a single centre from 2000 to 2018. Primary outcomes included 5-year ED visits and/or hospitalizations for ALTEs. Demographic, operative, and outcome data were collected. Chi-square tests and univariate analyses were performed.
RESULTS: In total, 266 EA/TEF patients met inclusion criteria. Of these, 59 (22.2%) had experienced ALTEs. Patients with low birth weight, low gestational age, documented tracheomalacia, and clinically significant esophageal strictures were more likely to experience ALTEs (p < 0.05). ALTEs occurred prior to 1 year of age in 76.3% (45/59) of patients with a median age at presentation of 8 months (range 0-51 months). Recurrence of ALTEs after esophageal dilatation was 45.5% (10/22), mostly due to stricture recurrence. Patients experiencing ALTEs received anti-reflux procedures (8/59, 13.6%), airway pexy procedures (7/59, 11.9%), or both (5/59, 8.5%) within a median age of 6 months of life. The resolution and recurrence of ALTEs after operative interventions are described.
CONCLUSIONS: Significant respiratory morbidity is common among patients with EA/TEF. Understanding the multifactorial etiology and operative management of ALTEs have an important role in their resolution.
METHODS: Original Research, Clinical Research.
METHODS: Level III Retrospective Comparative Study.

Despite improving the survival after repair of esophageal atresia (EA), the morbidity of EA repair remains high. Specifically, tracheomalacia (TM) is one of the most frequent complications of EA repair. Continuous positive airway pressure is generally applied for the treatment of TM. However, surgical intervention is required against an apparent life-threatening event or inability to perform extubation for a long period. According to our review, most cases of TM showed symptom improvement after aortopexy. The ratio of the trachea\'s lateral and anterior-posterior diameter at the brachiocephalic artery crossing the trachea, which reflects the compression of the trachea by the brachiocephalic artery, is a good indicator of aortopexy. Our finding suggests that most TM cases associated with EA may not be caused by tracheal fragility alone, but may involve blood vessel compression. Posterior tracheopexy (PT) is also an effective treatment for TM. Recently, open or thoracoscopic PT was able to be performed simultaneously with EA repair. In many cases, aortopexy or PT is a safe and effective surgical treatment for TM with EA. Other surgical procedures, such as external stenting, should be considered for patients with diffuse-type TM for whom aortopexy and PT appear relatively ineffective.

Patients with Marfan syndrome present anatomic variations that may increase the risk of a difficult airway. Moreover, they can present large aortic aneurysms, which may cause extrinsic airway compression. Therefore, difficult ventilation during general anesthesia poses a challenge in that the anesthesiologist has to promptly make a crucial differential diagnosis. Multidisciplinary preoperative assessment and planning of the airway and ventilation management are of utmost importance in such uncommon and highly complex clinical cases. Fiberoptic bronchoscopy is probably a really useful tool in order to assess the severity and extent of the airway compression, both preoperatively and intraoperatively. We present a clinical case where difficult ventilation occurred immediately after the induction of general anesthesia.

Circumflex aortic arch is a rare vascular ring anomaly, which is often associated with tracheobronchomalacia. Aortic uncrossing is the definitive repair of this condition, which is a major high-risk operation; therefore, it is not suitable for small patients such as newborn infants or individuals who have severe comorbidities. We present a case of the circumflex aortic arch with tracheobronchomalacia in a 2.7-kg infant who underwent successful innovative combined surgery of the airway and the aorta.

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OBJECTIVE: The aim of this study was to assess the surgical and follow-up outcomes in children who operated for aberrant innominate artery.
METHODS: A total of 15 consecutive patients (12 males, 3 females; mean age 16.3 ± 19.0 months; range 3 months to 6 years) who underwent aortopexy between February 2018 and December 2021 were evaluated. Demographic data, preoperative and postoperative clinical status and postoperative outcomes were retrospectively analyzed.
RESULTS: The mean age at operation was 16.3 ± 19.0 months. The median weight was 8.3 kg (range, 7-14.5 kg).There was no complications at intraoperative period. The mean percent degree of tracheal stenosis was 0.68 ± 0.12. The median (range) MV duration, PICU stay, and ward stay of the patients were 2 h (0-3 h), 2.5 days (1-4 days), and 5 days (3-8 days), respectively. The mean patients\' number of emergency service applications and hospitalization at the preoperative period was 6.2 ± 3.9/2.3 ± 1.6 and, at the postoperative period was 3.3 ± 2.2/0.9 ± 0.8. In comparison of the preoperative and postoperative service application number and hospitalization number, there was significant difference (p < 0.005 and 0.006, respectively). No reoperation was required. There was no mortality.
CONCLUSIONS: Aberrant innominate artery is rarely seen. These pathologies misdiagnosis with different reactive airways. Following the diagnosis, treatment can be achieved by surgery successfully.

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