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Background: Reimplantation of the left pulmonary artery (LPA) and slide tracheoplasty has been our standard approach of care for patients with pulmonary artery sling (PAS) and tracheal stenosis. We present our experience, with emphasis on tracheal arborization and hypoplastic lungs; and their impact on long-term outcome of children with PAS and tracheal stenosis. Methods: It is a retrospective comparative study. Data were classified and analyzed based on the type of tracheobronchial arborization and normal versus hypoplastic lungs. Results: Seventy-five children operated between January 1994 and December 2019 (67 with normal lungs and 8 with lung hypoplasia/agenesis) were included. Patients with hypoplastic lungs had higher rates of preoperative ventilation (87.5%), postoperative ventilation (10 vs 8 days, P = .621), and mortality (50% vs 9%, P = .009) compared with those with normal lungs. Nineteen patients had tracheal bronchus (TB) variety and 30 patients had congenital long-segment tracheal stenosis (CLSTS) variety of tracheobronchial arborization. Endoscopic intervention was needed in 47.4% of patients with TB type and 60% with CLSTS type. CLSTS patients had higher rates of preoperative ventilation (60% vs 47.4%, P = .386), longer periods of postoperative ventilation (13 vs 6.5 days, P = .006), and ICU stay (15 vs 11 days, P = .714) compared with TB type. Conclusion: Surgical repair of PAS with tracheal stenosis has good long-term outcomes. All variations of tracheal anatomy can be managed with slide tracheoplasty. Persistence of airway problems requires intervention during follow-up as tracheal stenosis continues to be the Achilles heel.

Background Postpneumopnectomy syndrome (PPS) is an extreme rotation and malposition of mediastinum causing dynamic and symptomatic central airway compression, arising after pneumonectomy or more uncommonly, in congenital single-lung physiology. Affected patients present with severe respiratory compromise. Intrathoracic prosthesis placement is an evolving technique in children that mitigate the effects of thoracic dead space. Research Question Assessment of clinical recovery and functional benefit in children undergoing placement of intrathoracic prosthesis following pneumonectomy or in congenital single lung situations. Study Design and Methods Retrospective chart review of patients at Great Ormond Street Hospital from 2010 and 2020 was performed of all patients who underwent intrathoracic tissue expander placement. We summarize the outcomes of twenty-four children, including those with both congenital and postpneumonectomy PPS etiology. Results 24 children who underwent placement of intrathoracic prosthesis for PPS in the study period with median age of 3.5 months and weight of 5 kg. Single lung etiology was congenital in 15 children (6 agenesis, 9 hypoplasia), and postpneumonectomy in 9 children. In seven patients, there was associated long segment tracheal stenosis. Pre-operative ECMO was required in 2 patients, and pre-operative ventilation was required in 12 patients – all of whom had congenital single lung. Intrathoracic prosthesis placement was concurrent with intracardiac repair in 5 patients. There were no operative deaths, but one early postoperative death related to septicaemia. Median follow up was 75 months with 10 patients on continued respiratory support and 3 on nocturnal support with good quality of life. Two children needed reoperations for replacement of prosthesis. Conclusion The use of tissue expanders is within the armamentarium of most plastic surgeons’ practice. We also therefore advocate for a collaborative team approach involving Plastic and Cardiothoracic Surgery for surgical treatment of these patients. This multidisciplinary strategy has improved management of this rare and debilitating condition of PPS, thereby offering significant improvements in general progress of these sick children having single lung physiology. Evidence is still lacking on functional outcomes in these children and further work is necessary to prove that this is indeed achievable.

Background Pulmonary artery sling (PAS) is usually associated with long-segment congenital tracheal stenosis (LSCTS). This combination of abnormalities can also be associated with lung hypoplasia abnormalities (hypoplasia, aplasia, or agenesis). This study analyzed the association of lung hypoplasia abnormalities with combined PAS and LSCTS and its influence on its surgical outcomes. Methods All patients (0 to 18 years) who underwent surgical procedures for both PAS and LSCTS from 1995 to 2019 were analyzed retrospectively for mortality, ventilation days, and intensive care unit days by dividing them into those with normal lungs (group 1) and hypoplastic lungs (group 2). Results Included were 75 patients (30 girls [40%]), who were a median age of 5.7 months (interquartile range [IQR], 2.9-13.3 months), median weight of 5.5 kg (IQR, 4.1-7.9 kg), and had a median follow-up of 99.8 months (IQR, 54.5-152.0 months); of these, 8 patients (10.7%) had hypoplastic right lung, comprising hypoplasia in 7 (87.5%), aplasia in 1 (12.5%), and agenesis in 0 (0%). There was a significant difference in mortality (group 1, 9.0%; group 2, 50%; P = .007) but no significant difference in median ventilation days (group 1, 9.0; group 2, 9.0; P = .89) or in median intensive care unit days (group 1, 14.0; group 2, 11.5; P = .44). Conclusions Lung hypoplasia associated with PAS and LSCTS is usually right-sided. As a result of severe airway obstruction and single-lung physiology, there is a high requirement of preoperative cardiorespiratory support and a significant association with adverse surgical outcomes.

Slide tracheoplasty (STP) is the procedure of choice for treatment of long segmental congenital tracheal stenosis (LSCTS). Few studies predict factors leading to reintervention or mortality after STP. We analyzed a pediatric population to identify such factors and compared the outcome between 2 eras (1995-2012 and 2013-2017).We analyzed 150 consecutive children who underwent STP from February 1995 to December 2017 in our hospital.Median age and weight were 6.9 months and 6.1 kg. Average tracheal diameter of LSCTS was 2.3 mm. Tracheal stenosis extended into bronchus in 36 patients and distal malacia in 38. Median follow-up was 67 months; mortality was 12.7%. Balloon dilatation was required in 81 patients (54%), stents in 29 (19%), and reoperation in 4 (3%). The presence of malacia, preoperative extracorporeal membrane oxygenation, congenital anomalies, and single lung anatomy increased the risk for reintervention. Cox regression analysis revealed preoperative ventilation to be an independent factor predicting reintervention and single lung tracheal anatomy for mortality. In the current era (after 2013), survival improved from 88% to 97% and stent requirement was reduced from 25% to 11%.Slide tracheoplasty can be applied to various airway configurations seen in LSCTS. The requirement for reintervention such as balloon dilatation and stenting is high in the group requiring preoperative ventilation. Mortality is highest in the single lung anatomy group. Centralization of care allowed us to develop the multidisciplinary team expertise to manage this and other rare airway conditions with acceptable outcomes.

Tracheal pathology in children are primarily congenital. They can be considered primary or de novo, when this is seen as an inherent defect within the cartilages of the tracheal segment. While segmental cartilage defects are very rare, there are often occasions when one or more cartilages can be considered missing from the length of trachea, contributing to airway abnormality. Secondary tracheal pathologies can often be seen in relation to disorders affecting nearby vascular elements or thoracic cage in general. In general, the pathological entity of tracheal disorders can be classified into either tracheomalacia or tracheal stenosis.

Aortopexy has been demonstrated to be an effective procedure for the treatment of tracheo(broncho)malacia in children. Several operative approaches have been proposed in the literature, including left anterior thoracotomy, right thoracotomy, full median sternotomy, or a thoracoscopic approach. In this video tutorial we present our technique for anterior aortopexy using a limited upper sternotomy.

OBJECTIVE Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. METHODS Patients

An acquired posterior tracheal wall defect, most commonly an acquired tracheoesophageal fistula, is a challenging clinical scenario. Autologous pedicled pericardial patch repair is a versatile and sustainable technique for the repair of a large tracheal defect, from primary management to airway salvage. This video tutorial demonstrates the technical aspects of this technique using step-by-step video.

Aim Symptomatic tracheobronchomalacia can be fatal. Successful treatment includes aortopexy. We report outcomes of the thoracoscopic approach in a single centre. Methods All patients undergoing thoracoscopic aortopexies from 2009 to 2018 were retrospectively reviewed. Data was reported as median (interquartile range). Risk factors for subsequent tracheostomy were analyzed with logistics regression model, p Results Twenty-one patients with mid to distal tracheomalacia (n = 17) and bronchial involvement (n = 4) were determined on bronchoscopy, tracheobronchogram, or CT thorax. Preoperative patient demographics and comorbidities, e.g., gastro-oesophageal reflux disease, prematurity, and cardiac anomalies were recorded. Indications for thoracoscopic aortopexy were apparent life-threatening event(s) (n = 14), recurrent chest infections (n = 5), and failure to wean invasive ventilation (n = 2). Thoracoscopic aortopexies (n = 20) with conversion to open (n = 1) were performed. Intraoperative bleeding (n = 2) occurred, and chest tube (n = 1) was inserted for monitoring. Intraoperative bronchoscopy (n = 17) confirmed improvement of tracheomalacia. Anesthetic time was 140 (90–160) minutes. Postoperatively, 2 patients had dehiscence of the aorta from the sternum. They underwent redo open aortopexy with posterior tracheopexy, and 1 required subsequent tracheostomy. Another 2 patients required tracheostomies. Potential risk factors for subsequent tracheostomy were investigated, and only the association of tracheobronchomalacia was close to significance (OR 16 (95% CI 0.95–267.03), p = 0.05). Follow up duration was 365 (72–854) days. Symptoms resolution occurred in n = 17 (81%) of patients. Conclusion Different modalities were used to delineate the site of tracheobronchomalacia and its etiology. Tracheomalacia with bronchial involvement may be a risk factor for subsequent tracheostomy. Level of Evidence Level 3 (Case Series).

Mesenchymal hamartomas of the chest wall are unusual tumours diagnosed in neonates. They mostly resolve spontaneously hence conservative management has been advocated. Some compress vital structures in the thoracic cavity or bleed warranting surgical intervention. We present a neonate with mesenchymal hamartoma of the chest wall presenting as unilateral multifocal lesions with life threatening complications. He responded well to surgical intervention and was successfully discharged.

Objectives Pediatric patients born with single functional lung can be associated with symptomatic airway abnormalities. Management of such patients is not only technically demanding but is also ethically challenging. This study reports our experience of managing symptomatic airway abnormalities in pediatric patients with congenital single functional lung. Methods Observational retrospective cohort study performed at a tertiary children’s hospital from January 2001 to September 2017. All children (0 to 18 years old) with congenital single functional lung (agenesis and hypoplasia) presenting with symptomatic airway abnormalities (long segment congenital tracheal stenosis and tracheomalacia) and requiring surgical interventions were included in the study. Children with single functional lung secondary to non-congenital causes were excluded. Results A total of 16 patients with single functional lung (agenesis = 8 and hypoplasia = 8) and airway abnormalities (long segment congenital tracheal stenosis = 12 and tracheomalacia = 4) were eligible for the study. Lung abnormalities were common on the right side (n = 10, 62.5%). Associated abnormalities (cardiac and non-cardiac) were seen in 11 patients (68.8%). Surgical interventions for airway abnormalities, alone or in combination, included slide tracheoplasty (n = 12), aortopexy with or without pericardiopexy (n = 7), excision of rudimentary lung (n = 4) and placement of intrathoracic tissue expanders to reposition the mediastinum (n = 3). Nine patients (56.3%) underwent a one-stage repair while staged repairs (airway and cardiac) were performed in 7 (43.7%). Fourteen patients (87.5%) survived to hospital discharge. Of the survivors, 9 (64.2%) had stable airways not requiring respiratory support at home. Conclusion Management of pediatric patients with airway abnormalities in the setting of congenital single functional lung is feasible with acceptable surgical outcomes. This is facilitated by staged repairs and repositioning of mediastinum before a definitive airway repair in patients with significant comorbidities. Treatment should not be deferred to these patients if there are no obvious contraindications. Type of Study Retrospective Case Control Study Level of Evidence Level III.

Purpose Although mechanical circulatory support devices in children are primarily utilised as a bridge to transplant, explantation to recovery is possible in selected cases. The purpose of this study is to report our national data of explantation of mechanical circulatory support devices to clinical recovery in children. Methods Retrospective study performed in two institutions from January 2007 to September 2018 (12 years). All children who had explanation of mechanical circulatory support devices were included. Relevant variables analysed were gender, age, weight at implantation, primary diagnosis, extracorporeal membrane oxygenation (ECMO) pre & post implantation, type of device used, need for bi-ventricular support, cardiopulmonary bypass time (CPB), arrhythmia before explantation and duration of mechanical device support. Primary outcome was survival after explantation. Secondary outcomes were arrhythmia after explantation, renal replacement therapy (RRT) during mechanical circulatory support, neurological complications and eventual need for heart transplantation. Results 19 children were eligible (females = 42%, mean age = 38 months, mean weight = 15.4 kilograms). Primary diagnosis included cardiomyopathy (n = 6, 31.6%), myocarditis (n = 12, 63.1%) and congenital heart disease (n = 1, 5.3%). 9 children pre-implant (47.4%) and 2 children post-implant (10.5%) required ECMO. Devices used were Berlin Heart (n = 16, 84.2%) and Levitronix (n = 3, 15.8%). Bi-ventricular support was needed in 4 children (21%). Mean CPB was 108.3 minutes. 5 children (26.3%) had arrhythmia pre-explantation. Mean duration of mechanical circulatory support was 43.9 days (range, 6 to 120 days). 17 children survived explantation (89.4%), of which 1 (5.3%) eventually required heart transplantation. RRT was required in 7 children (36.8%). 1 child had arrhythmia post-explantation (5.3%). 5 children had neurological complications (26.3%). Conclusion Explantation of mechanical circulatory support devices to clinical recovery is possible in selected cases in children. The option of device explantation or using them as a bridge to recovery should be considered in children especially if the primary diagnosis is myocarditis. This can mitigate the high risk of morbidities associated with their long-term usage as a bridge to transplant.

Background: In children, significant complications can be encountered after airway interventions (balloon dilatation and stent deployment). Hence following the procedures after midday, our usual practice is to observe them overnight as an in-patient. This impacts patient management as treatment is often delayed if an overnight bed is not secured. Aims and objectives: 1. To analyse the complications of elective airway assessment and intervention by flexible bronchoscopy in children with tracheal abnormalities (stenosis and malacia) 2. To explore the possibility of an early discharge (same day) if major complications were infrequent Methods: All children with tracheal abnormalities who had elective airway assessment +/- intervention by flexible bronchoscopy in 2015 in our tertiary paediatric airway centre were included. They were divided into two groups, intervention and non-intervention. Retrospective data collection included procedure time (morning/ afternoon), intervention type, balloon size, duration of stay and complications. Major complications are air leak, bleeding, stridor and respiratory failure. Minor complications are oxygen need, fever and mild respiratory difficulty. Results: 57 elective procedures (intervention = 30, non-intervention = 24, missing data = 3) were performed for 39 children. Interventions included 29 balloon dilatations and 1 stent deployment. Complications in the intervention group were oxygen need (3/30) and fever (1/30). Complications in the non-intervention group were fever (2/24) and stridor (1/24). Conclusions: Early discharge is possible after elective flexible bronchoscopy in children with tracheal abnormalities as significant complications were infrequent.

Foreign body ingestion is common in infants and young children and can cause numerous complications, mainly if ingested and left in place for prolonged periods. In recent years, particular dangers, specifically from ingested button batteries, have become increasingly recognized as a public health issue. Of particular note is the rapid onset of full thickness burns and oesophageal perforation which may occur within as little as 2 h following the ingestion of button batteries. The aim of this review is twofold: (1) to increase awareness of the need for rapid action from radiologists, emergency care physicians and paediatricians on identifying a button battery impacted within the oesophagus, and (2) to review the imaging appearances that can distinguish button batteries from other similar appearing foreign bodies, most notably coins.