Management of bronchomalacia in infants post-cardiac surgery using synchronized nasal DuoPAP: A novel technology.

Background: Tracheo-bronchomalacia (TBM) is the weakness in the structural integrity of the cartilaginous ring and arch. It may occur in isolation with prematurity or secondarily in association with various congenital anomalies. Bronchomalacia is more commonly associated with congenital heart diseases. The conventional treatment options include positive pressure ventilation with or without tracheostomy, surgical correction of external compression and airway stenting. Aim: To use "synchronized" nasal Dual positive airway pressure (DuoPAP), a non-invasive mode of ventilation as an alternative treatment option for bronchomalacia to avoid complications associated with conventional treatment modalities. Study Design: Prospective observational study conducted in Army Hospital Research and Referral from Jul 2019 to Dec 2020. Material and Methods: We diagnosed seven cases of TBM post-cardiac surgery at our institute, incidence of 4.2%. Four infants were diagnosed with left sided bronchomalacia, 2 were diagnosed with right sided bronchomalacia and one with tracheomalacia. Those infants were managed by "synchronized" nasal DuoPAP, a first in ventilation technology by Fabian Therapy Evolution ventilator (Acutronic, Switzerland). Results: All seven infants showed significant improvement with synchronized nasal DuoPAP both clinically as well as radiologically. None of the infant required tracheostomy and discharged to home successfully. Conclusion: The synchronized nasal DuoPAP is a low cost and effective treatment option for infants with TBM. It could be attributed to synchronization of the breaths leading to better tolerance and compliance in paediatric age group.

External Stenting for Bronchomalacia Involving the Bronchus Intermedius.

In recent years, external stenting has been used as a reliable method to relieve airway compression of the trachea and main bronchi in young children with acceptable age-proportional airway growth. However, to our knowledge, little literature supporting this approach for the distal airway has been published. A 1-year-old girl with absent pulmonary valve syndrome who had recurrent respiratory infections was diagnosed with bronchomalacia. She underwent external stenting; the infections disappeared postoperatively. This case describes a successful external stenting for malacia of the bronchus intermedius, which exhibited short- and mid-term safety and effectiveness.

Bronchomalacia in Right Aortic Arch Treated With Descending Aortic Translocation and Airway Splint.

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.

Bronchial stenting in infants with severe bronchomalacia: Technique and outcomes.

OBJECTIVES: To report the first use of a balloon expandable bare metal stent for treating infant bronchomalacia. BACKGROUND: Infant bronchomalacia often requires prolonged mechanical ventilation and can be life-threatening. Effective treatment for severe infant bronchomalacia continues to be elusive. We present three cases of bronchial stenting for no-option or treatment refractory infant bronchomalacia. METHODS: Three consecutive cases of stenting to relieve conservative treatment refractory severe infant bronchomalacia were performed between February 2019 and December 2020. Initial diagnosis was confirmed with Computed Tomography (CT) angiography. Patients underwent rigid micro laryngoscopy, bronchoscopy, and flexible bronchoscopy to evaluate the airway. Initial conservative management strategies were pursued. Patients failing initial conservative management strategies were considered for rescue bronchial stenting. RESULTS: Our initial clinical experience with a coronary bare metal stent for these procedures has been favorable. The stent was easy to deploy with precision. We did not encounter stent embolization or migration. There was sufficient stent radial strength to relieve bronchomalacia without causing restenosis or erosion. There was no significant granulation tissue formation. In one patient, the stent was removed after 12 months of somatic growth; this was uneventful and bronchial patency was maintained. There were no complications in any of our patients regarding stent placement and reliability. CONCLUSION: In cases of three infants with severe bronchomalacia, we found that bronchial stenting with the bare metal coronary stent was effective in relieving bronchial stenosis.

Hybrid reconstruction of bronchial dehiscence following lung transplant.

Surgical repair of right-sided bronchial dehiscence post lung transplant is challenging. We report a hybrid reconstruction of the bronchus using an aortic homograft patch with stenting as a novel technique of management of ischemic airway injury following lung transplantation.

Successful repair of critical bronchial stenosis with coexisting bronchomalacia and congenital heart defects in infants by slide bronchoplasty.

Isolated bronchial stenosis in infancy is rare, difficult to manage and becomes more complex when associated with congenital heart defects. Given the small luminal diameter and the proximity of the lung parenchyma to the bronchial lesion in infants, reconstruction of the bronchial stenosis is surgically challenging. We present 2 infant cases having isolated critical bronchial obstruction with congenital heart defects, which were successfully managed by primary one-stage slide bronchoplasty with concomitant heart repair. Both cases had excellent results after the surgery and did well at home without requiring respiratory support.

Absorbable Microplate Externally Suspending Bronchomalacia in Congenital Heart Disease Infant.

To evaluate the feasibility and efficacy of external suspension with absorbable poly-l-lactic acid material shaping microplates for infants with severe bronchomalacia and congenital heart disease. From November 2017 to January 2019, 11 continual patients with severe bronchomalacia and congenital heart disease underwent bronchial membrane external suspension together with cardiovascular surgery. An absorbable plate made with poly-l-lactic acid material was used as the shaping fixation material in all patients. Data included the details of the operation, and clinical results were collected. The mean age was 1.2 ± 1.0 years, and the mean weight was 7.7 ± 2.9 kg. The patients with cardiac malformations were operated on under low-temperature cardiopulmonary bypass (CPB) through median sternotomy. There were no in-hospital deaths. The CPB time, mechanical ventilation time, and length of intensive care unit stay were 123.9 ± 36.9 min, 20.7 ± 19.4 h, and 71.6 ± 54.9 h, respectively. Two patients underwent surgery through a left posterolateral incision without CPB. One was a double aortic arch repair, and the other was only bronchial membrane external suspension with prior IAA repair. No patients needed ECMO support. The mean follow-up time was 12.1 ± 5.6 months, and no patients were lost to follow-up. No cases of late death were noted, and no patients needed reoperation. According to the CT scans, no patients had bronchial restenosis. External bronchial membrane suspension with an absorbable poly-l-lactic acid material shaping plate, which had better histocompatibility, for infants with severe bronchomalacia and congenital heart disease was a safe and feasible procedure.

Transposition of the Great Arteries Associated With Non-Kommerell Diverticulum: Lessons From Two Cases.

Vascular ring malformations usually occur as an isolated lesion. d-Transposition of the great arteries (d-TGA) associated with vascular ring malformations has seldom been reported in the literature. In this report, we describe two unusual cases of d-TGA associated with non-Kommerell diverticulum resulting in vascular ring. Our approach for the diagnosis and surgical management of this unusual combination of congenital heart lesions is described.

A Successful Case of External Stenting for Bronchomalacia Lasting Over 20 Years.

We report on a patient with perimembranous ventricular septal defect and pulmonary hypertension. He underwent pulmonary artery banding followed by external stenting of the left bronchus because of secondary bronchomalacia. The external stent applied was a ringed expanded polytetrafluoroethylene graft, 10 mm in diameter and fixed at 8 points. Intracardiac repair was performed at the age of 2 years and the external stent was left in place. Since then, the patient has been healthy and computed tomography reveals that the external stent graft has retained function for over 20 years.

Descending Aortopexy and Posterior Tracheopexy for Severe Tracheomalacia and Left Mainstem Bronchomalacia.

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.

Successful Infant Pneumonectomy with Unilateral Pulmonary Artery Occlusion Test.

The use of unilateral pulmonary artery occlusion (UPAO) test for the preoperative evaluation of pneumonectomy was reported in adult patients. On the contrary, in infants, no strategies have yet been recommended to predict hemodynamics after pneumonectomy, nor has use of the UPAO test been reported. We describe the first case of infant with abnormal pulmonary circulation in whom successful pneumonectomy was performed after preoperative evaluation using UPAO test. Right pneumonectomy was planned for an 8-month-old girl, because of decreased right pulmonary function, high risk of pneumothorax, and impaired left lung expansion due to overexpansion caused by severe left bronchial stenosis and bronchomalacia. However, she had also prolonged pulmonary hypertension and there was difficulty in accurate echocardiographic evaluation of its severity due to concomitant left pulmonary artery stenosis. Furthermore, contrast-enhanced computer tomography suggested a certain degree of right pulmonary venous flow, discordant with the result showing scarce right pulmonary flow in perfusion scintigraphy. Predicting postoperative hemodynamic changes was therefore considered difficult. To evaluate these concerns, we performed cardiac catheterization and UPAO test to simulate postoperative hemodynamics. Pulmonary arteriography showed decreased but significant right pulmonary arterial and venous flows. Measurements including pulmonary artery pressure and cardiac index showed no marked changes after occlusion. Based on UPAO test results, the operation was successfully performed and hemodynamics remained stable postoperatively. The UPAO test may be useful for infants with cardiopulmonary impairment to evaluate the tolerability of pneumonectomy.

Bronchoscopic assessments and clinical outcomes in pediatric patients with tracheomalacia and bronchomalacia.

BACKGROUND: Tracheomalacia and bronchomalacia (TM/BM) are one of the serious causes of airway obstruction in infants and children. This study reviewed our bronchoscopic assessments and clinical outcomes in pediatric patients with TM/BM, and investigated risk factors of surgical intervention for TM/BM. METHODS: Fifty-seven consecutive patients who were diagnosed as TM/BM by bronchoscopy between 2009 and 2013 were reviewed retrospectively. They were divided into two groups according to the presence (group E, n = 26) or absence (group N, n = 31) of acute life-threatening events and extubation failure (ALTE/EF). The severity of TM/BM was evaluated by Oblateness Index which was obtained from bronchoscopic images. RESULTS: Oblateness Index was significantly higher in Group E than in Group N. Patients in Group E underwent surgical intervention for TM/BM more frequently, and had significantly longer intubation period and hospital stay. Clinical symptoms of ALTE/EF, Oblateness Index ≥ 0.70, and multiple malacic lesions were significant risk factors indicating surgical events in patients with TM/BM. CONCLUSIONS: Patients with TM/BM who had ALTE/EF had more severe malacic lesions indicating surgical intervention, and worse clinical outcomes. Oblateness Index is a simple and semi-quantitative index for bronchoscopic assessment of TM/BM, and can be one of the prognostic tools to predict clinical severity of pediatric TM/BM.

Combined use of Neurally Adjusted Ventilatory Assist (NAVA) and Vertical Expandable Prostethic Titanium Rib (VEPTR) in a patient with Spondylocostal dysostosis and associated bronchomalacia.

Jarcho-Levin syndrome is a rare disorder characterised by defects in vertebral and costal segmentation of varying severity. Respiratory complications are the main cause of death or severe comorbidity due to a restrictive rib cage. A 3 months old infant with Spondylocostal dysostosis and associated bronchomalacia experiencing severe asynchrony during the weaning process is reported. The Neurally Adjusted Ventilatory Assist (NAVA) mode was used to improve adaptation to mechanical ventilation after Vertical Expandable Prosthetic Titanium Ribs (VEPTRs) were implanted. The synchrony achieved with the NAVA mode allowed a decrease of the sedoanalgesia he received. A follow-up CT scan showed a reduction in the volume of the posterobasal atelectasis. The evolution of this patient suggests that the combined use of VEPTR for thoracic expansion and ventilation using NAVA can favour the global improvement. This mode could be an option to consider in selected patients with difficult weaning from mechanical ventilation in paediatric intensive care units.

Direct Tracheobronchopexy and Posterior Descending Aortopexy for Severe Left Mainstem Bronchomalacia Associated With Congenital Pulmonary Airway Malformation and Left Circumflex Aortic Arch.

Complex tracheobronchial obstruction and malacia can be associated with conotruncal and aortic arch anomalies. A circumflex aortic arch composed of a left aortic arch and right descending aorta is an extremely rare anomaly that can severely affect the distal trachea and mainstem bronchi, resulting in severe respiratory symptoms. We report the case of a patient with circumflex aortic arch and severe left mainstem bronchial obstruction and malacia in which the external aortic compression and residual bronchomalacia were addressed with direct bronchial and tracheal intervention.

Condroma endobronquial: un caso atípico de obstrucción bronquial / Endobronchial chondroma: An unusual case of bronchial obstruction

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Oki stenting for anastomotic bronchomalacia in lung transplantation.

Anastomotic airway complications are a frequent cause of disease in lung transplantation. However, there is no consensus on the type of treatment to be performed with prosthetic devices. While some recent gadgets such as the Oki stent have been proposed for main right bronchus stenosis, there are no reports of stenting using this prosthesis in cases where the main complication is malacia rather than stenosis. We present 2 patients diagnosed with main right bronchus bronchomalacia, also involving bronchius intermedius. After several attempts to bypass the anastomosis employing different types of stent, including a T-tube Montgomery device, normal sputum drainage was not possible. Oki stenting was performed without complications, with a remarkable reduction in endoscopic procedures as well as important functional improvement. For both stenosis and bronchomalacia in lung transplantation, we propose Oki stenting as the first choice of treatment.

Tratamiento de la estenosis de bronquio intermediario en el trasplante pulmonar con endoprótesis de Montgomery. Una técnica novedosa / Treatment of Bronchus Intermedius Stenosis in Lung Transplantation With Montgomery T-tube Stent. A Novel Technique

Las complicaciones de la vía aérea en pacientes con trasplante pulmonar son una entidad relativamente frecuente, aunque su incidencia es variable según los diferentes estudios. Su patogenia es variada, siendo el principal mecanismo responsable la isquemia bronquial asociada al periodo postrasplante inmediato. La principal complicación suele ser la estenosis bronquial, y en el trasplante pulmonar derecho la afectación del bronquio intermediario se produce con relativa frecuencia. Se han propuesto distintos tratamientos para la estenosis del bronquio intermediario, como dilatación con balón endobronquial, láser, criocirugía y endoprótesis bronquiales. Presentamos 2 casos clínicos con trasplante pulmonar y estenosis del bronquio intermediario que han sido tratados con una endoprótesis de Montgomery o en T, que se emplea habitualmente para estenosis traqueales, con una respuesta clínica y funcional muy favorable

Airway complications after lung transplant are relatively common although the rates vary according to the different studies. Pathogenesis is diverse but the principal mechanism is usually bronchus intermedius ischemia in the post-transplant period. One major complication is bronchial stenosis, with relatively frequentinvolvement ofthe bronchus intermedius in the case of rightlung transplantation. Various treatments have been proposed for bronchus intermedius stenosis, such as endobronchial balloon dilation, laser, cryosurgery and bronchial stents. We presenttwo cases of lung transplant recipients with bronchus intermedius stenosis treated with a Montgomery stent or T-stent, commonly used for tracheal stenosis, who showed positive clinical and functional response