Tracheobronchomalacia (TBM) refers to airway collapse due to typically excessive posterior membrane intrusion and often associated with anterior cartilage compression. TBM occurs either in isolation ...or in association with other congenital or acquired conditions. Patients with TM typically present non-specific respiratory symptoms, ranging from noisy breathing with a typical barking cough to respiratory distress episodes to acute life-threatening events and recurrent and/or prolonged respiratory infections. There are no definitive standardized guidelines for the evaluation, diagnosis, and treatment of TBM; therefore, patients may be initially misdiagnosed and incorrectly treated. Although milder cases of TBM may become asymptomatic as the diameter of the airway enlarges with the child, in cases of severe TBM, more aggressive management is warranted. This article is an overview of the clinical presentation, evaluation, diagnosis, medical management, and surgical treatment options in pediatric tracheomalacia.
Aortic uncrossing is an effective procedure for relieving the external airway compression from a circumflex aortic arch by transferring the aortic arch to the same side as the descending aorta. ...However, patients frequently have residual tracheobronchomalacia (TBM), which may result in persistent postoperative symptoms. We review a series of patients who underwent an aortic uncrossing and concomitant tracheobronchopexy to correct the airway compression and residual TBM.
Retrospective review of all patients who underwent aortic uncrossing and concomitant tracheobronchopexy at a single institution between September 2016 and March 2019. Preoperative evaluation included computed tomography angiography and rigid 3-phase dynamic bronchoscopy.
Eight patients who ranged in age from 4 months to 15 years with significant respiratory symptoms underwent an aortic uncrossing procedure with concomitant tracheobronchial procedures. Mild hypothermic cardiopulmonary bypass (mean time, 105.6 ± 39.4 min) and regional perfusion (mean time, 44 ± 10 min) were used without circulatory arrest. Intraoperative bronchoscopy demonstrated no patients had residual TBM. There were no postoperative mortalities, neurologic complications, chylothoraces, coarctations, or obstructed aortic arches. Two patients required tracheostomy and gastrostomy for bilateral recurrent laryngeal nerve paresis (patients 2 and 3). One patient with bronchial stenosis after concomitant slide bronchoplasty required stenting. At a median follow-up of 22 months (range, 5-34 months), all patients were alive without evidence of significant respiratory symptoms.
The aortic uncrossing procedure can be performed safely in pediatric patients of all ages without circulatory arrest. Concomitant procedures addressing associated TBM can significantly improve respiratory symptoms.
Although most children do well after operations to relieve vascular compression of the esophagus and airway, many will have persistent/recurrent symptoms. We review our surgical experience using a ...customized approach to correct various etiologies of failure after vascular ring/decompression surgery.
Our institutional database identified children who underwent reoperation for persistent/recurrent symptoms after vascular ring or aberrant arterial decompression surgery between January 2014 and December 2019. Charts were reviewed for operative approaches and clinical data. Findings were analyzed by Fisher exact test for comparison between groups.
Twenty-seven children required reoperative surgery. Detailed preoperative workup identified 5 etiologies of failure for a customized approach. Residual scarring was corrected by lysis and rotational esophagoplasty (n = 23/27); fibrotic bands re-creating a ring were divided (n = 11); ongoing vascular compression was addressed by descending aortopexy (n = 19), aberrant subclavian division (n = 7), aortic uncrossing procedure (n = 4), and Kommerell resection (n = 8); anterior aortopexy (n = 6) and anterior tracheopexy (n = 9) corrected cartilage malformation; and tracheobronchomalacia was addressed with posterior airway pexy (n = 26). At available short-term follow-up (median 1 year), 21 of 22 patients (95%) had symptom improvement, and on bronchoscopy, the average number of airway sections with severe tracheobronchomalacia decreased from 2.8 ± 1.7 to 0.5 ± 0.9 (P < .001).
Persistent/recurrent symptoms after release of vascular compression are frequently caused by 5 different etiologies. A multidisciplinary strategy for workup and a customized operative approach can effectively treat these cases and may suggest opportunity at the index surgery to prevent reoperation and achieve optimal outcomes.
The methods (top left), the operative strategy based on etiology of failure (upper right), and the clinical results of this approach (bottom right). The future implications of this research are suggested (bottom left). Display omitted
Airway collapse from dynamic tracheobronchomalacia (TBM), static compression from vascular compression, and/or tracheobronchial deformation are challenging conditions. Patients are best assessed and ...managed by a multidisciplinary team in centers specializing in complex pediatric airway disorders. Suspicion is made through clinical history and physical examination, diagnosis of location and severity by dynamic 3-phase bronchoscopy, and surgical treatment planning by MDCT and other studies as necessary to completely understand the problems. The treatment plan should be patient-based with a thorough approach to the underlying pathology, clinical concerns, and combined abnormalities. Patients should undergo maximum medical therapy prior to committing to other interventions. For those children considered candidates for surgical intervention, all other associated conditions, including vascular anomalies, chest wall deformities, mediastinal lesions, or other airway pathologies, should also be considered. Our preference is to correct the airway lesions at the same operation as other comorbidities, if possible, to prevent multiple reoperations with their attendant increased risks. We also strongly advocate for the use of recurrent laryngeal nerve monitoring in all cases of cervical or thoracic surgery to minimize the risks to vocal cord function and laryngeal sensation. Studies that evaluate the effect of these interventions on the patient and caregiver's quality of life are needed to fully grasp the impact of TBM on this challenging patient population.
Pediatric Tracheomalacia Fraga, Jose Carlos, MD, PhD; Jennings, Russell W., MD; Kim, Peter C.W., MD. CM, PhD
Seminars in pediatric surgery,
06/2016, Letnik:
25, Številka:
3
Journal Article
Recenzirano
Tracheomalacia (TM) is defined as increased collapsibility of the trachea due to structural anomalies of the tracheal cartilage and/or posterior membrane. Tracheomalacia has a wide range of ...etiologies, but is most commonly present in children born with esophageal atresia and tracheal esophageal fistula. Clinical symptoms can range from minor expiratory stridor with typical barking cough to severe respiratory distress episodes to acute life-threatening events (ALTE). Although the majority of children have mild to moderate symptoms and will not need surgical intervention, some will need life changing surgical treatment. This chapter examines the published pediatric literature on TM, discusses the details of clinical presentation, evaluation, diagnosis and variety of treatments.
Abstract Purpose In severe tracheomalacia, aortopexy addresses anterior vascular compression, but does not directly address posterior membranous tracheal intrusion. We review patient outcomes of ...posterior tracheopexy for tracheomalacia with posterior intrusion to determine if there were resolution of clinical symptoms and bronchoscopic evidence of improvement in airway collapse. Methods All patients who underwent posterior tracheopexy from October 2012 to March 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores based on standardized dynamic airway evaluation by anatomical region, and persistent airway intrusion were collected. Data were analyzed by Wald and Wilcoxon signed-ranks tests. Results 98 patients (51% male) underwent posterior tracheopexy at a median age of 15 months (IQR 6–33 months). Median follow-up was 5 months (range 0.25–36 months). There were statistically significant improvements in clinical symptoms postoperatively, including cough, noisy breathing, prolonged and recurrent respiratory infections, transient respiratory distress requiring positive pressure, oxygen dependence, blue spells, and apparent life-threatening events ( p < 0.001), as well as ventilator dependence ( p = 0.04). Tracheomalacia scores on bronchoscopy improved significantly in all regions of the trachea and bronchi ( p < 0.001). 9.2% had persistent airway intrusion requiring reoperation, usually with aortopexy. Conclusions Posterior tracheopexy is effective in treating severe tracheomalacia with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy. Level of evidence Level III, treatment study.
Long Gap Esophageal Atresia Shieh, Hester F., MD; Jennings, Russell W., MD
Seminars in pediatric surgery,
04/2017, Letnik:
26, Številka:
2
Journal Article
Recenzirano
Abstract The management of long gap esophageal atresia remains challenging with limited consensus on the definition, evaluation, and surgical approach to treatment. Efforts to preserve the native ...esophagus have been successful with delayed primary anastomosis and tension-based esophageal growth induction processes. Esophageal replacement is necessary in a minority of cases, with the conduit of choice and patient outcomes largely dependent on institutional expertise. Given the complexity of this patient population with significant morbidity, treatment and long-term follow up is best done in multidisciplinary esophageal and airway treatment centers.
Management of recurrent symptomatic tracheobronchomalacia and tracheobronchial compression after prior aortopexy and tracheobronchopexy is a surgical challenge. In patients with right aortic arch, ...the course of the aortic arch over the right mainstem bronchus can result in superior and posterior compression of the airway. This report presents 2 cases of recurrent bronchomalacia and bronchial compression treated with descending aortic translocation. The addition of bronchial splinting to aortic translocation effectively relieved airway symptoms.
•Infants with long-gap esophageal atresia are at risk for brain findings.•Brain volumes are smaller in infants following repair of long-gap esophageal atresia.•In comparison to norm, infant head ...circumference is not changed following surgical repair (novel finding).•Head circumference is not the best indirect measure of brain size in these infants.•Future studies should focus on the etiology of described head/brain size discrepancy.
We extended our pilot study in infants following long-gap esophageal atresia (LGEA) repair to report head circumference, an easily obtainable indirect measure of brain size. Data are presented in the context of previously reported body weight and T2-weighted MRI measures of intracranial and brain volumes.
Clinical information and head circumference were obtained for term-born (n = 13) and premature (n = 13) infants following LGEA repair with Foker process, as well as healthy term-born controls (n = 20) <1-year corrected age who underwent non-sedated research MRI. General Linear Model univariate analysis with corrected age at scan as a covariate and Bonferroni adjusted p values assessed group differences.
We report no difference in head circumference between the three groups. Such findings paralleled trends in body weight and total intracranial volume but not in brain volume as previously reported for the same pilot cohort.
Results suggest uncompromised somatic and head growth after repair of LGEA. In contrast, a novel finding of discrepancy between head circumference (novel data) and brain size (previously published data) in the same cohort suggests that head circumference might not be the best indirect measure of brain size in selected group of patients.
Posterior descending aortopexy can relieve posterior intrusion of the left mainstem bronchus that may limit the effectiveness of posterior tracheobronchopexy. We review outcomes of patients ...undergoing both descending aortopexy and posterior tracheopexy for severe tracheobronchomalacia with posterior intrusion and left mainstem compression to determine if there were resolution of clinical symptoms and bronchoscopic evidence of improvement in airway collapse. All patients who underwent both descending aortopexy and posterior tracheopexy from October 2012 to October 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores based on standardized dynamic airway evaluation by anatomical region, and persistent airway intrusion requiring reoperation were collected. Data were analyzed by Wald and Wilcoxon signed-rank tests. Thirty-two patients underwent descending aortopexy and posterior tracheopexy at median age of 18 months (interquartile range 6-40 months). Median follow-up was 3 months (interquartile range 1-7 months). There were statistically significant improvements in clinical symptoms postoperatively, including cough, noisy breathing, prolonged and recurrent respiratory infections, ventilator dependence, blue spells, and brief resolved unexplained events (all P < 0.001), as well as exercise intolerance ( P = 0.033), transient respiratory distress requiring positive pressure ( P = 0.003), and oxygen dependence ( P = 0.007). Total tracheomalacia scores improved significantly ( P < 0.001), with significant segmental improvements in the middle ( P = 0.003) and lower ( P < 0.001) trachea, and right ( P = 0.011) and left ( P < 0.001) mainstem bronchi. Two patients (6%) had persistent airway intrusion requiring reoperation with anterior aortopexy or tracheopexy. Descending aortopexy and posterior tracheopexy are effective in treating severe tracheobronchomalacia and left mainstem intrusion with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy.
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