Pedicled pericardial patch in tracheal reconstruction in children - Novel technique to provide vascularized tissue for salvaging difficult airways.

BACKGROUND: Management of large central airway defects are often complex. Children who present with these defects have multiple co-morbidities or have had previous surgeries. Surgical options include various tissue cover for these defects without longer term benefits. Vascularized autologous pericardial patch offers a better solution to these defects by providing vascularity and potential for remodelling in future. METHODS: 41 children (M:F of 24:17) were operated for large trachea-bronchial defects between January 2015 and August 2022. The median age of was 12 months with median weight of 8.9 kg (IQR 3.3 kg-17.7 kg) Causes leading to the central tracheal defect include failed repair of previous trachea-oesophageal fistula (TOF) (n = 21) and acquired fistula due to button battery injury (n = 11). Surgical repair consisted of autologous pedicled pericardial patch repair for the airway defect under cardiopulmonary bypass. RESULTS: There were two operative deaths related to extensive sepsis and necrosis of reconstructed trachea. Four children had further reoperation with additional patch. Bronchoscopy was used as surveillance in all these children, with use of airway stents (biodegradable stent) in 9 children. The median ventilation time was 8 days, with tracheostomy being needed in 5 for long term support. CONCLUSIONS: Autologous pericardial patch is a versatile technique and can be used to salvage large tracheal defects when other method have failed or not feasible. Tracheomalacia at the site of repair could be managed with biodegradable stents. Vascularity and ciliary function of the patch still needs to be evaluated.

Impact of Tracheal Arborization and Lung Hypoplasia in Repair of Pulmonary Artery Sling in Combination With Long-Segment Tracheal Stenosis.

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.

Complex airway reconstruction in children with tracheobronchial injuries: a case series.

Paediatric airway surgery in the setting of complex tracheobronchial defects is challenging. This report describes the surgical management and outcomes of pericardial flap repair in three children. The first patient was a 4-month-old boy with a history of tracheoesophageal fistula repair who presented after out-of-hospital cardiac arrest. He was treated by re-do tracheobronchial reconstruction of the carina using a pedicled pericardial flap. The second patient was an 11-month-old boy who presented following aspiration of a button battery. Bronchoscopy showed erosion of the battery through both main bronchi and the oesophagus. The patient underwent emergency reconstruction of the extensive tracheobronchial defect with pedicled right and left pericardial patches. The third patient was a 5-year-old girl who fell from a swing, resulting in avulsion of the right main bronchus. Pedicled pericardium was used to reconstruct the damaged posterior tracheal wall and the right and left main bronchi. All three patients underwent successful repair of complex tracheobronchial defects with good outcomes in terms of survival and quality of life during 6 to 21 months of follow-up. Pedicled pericardial flap repair may be a viable option for achieving improved results in children with severe tracheobronchial defects.

Risk Stratification of Slide Tracheoplasty for Pediatric Airway Stenosis.

BACKGROUND: 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). METHODS: We analyzed 150 consecutive children who underwent STP from February 1995 to December 2017 in our hospital. RESULTS: 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%. CONCLUSIONS: 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.

Lung Hypoplasia Associated With Ring-Sling Complex Is Usually Right-Sided.

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.

Intrathoracic prosthesis in children in preventing post pneumonectomy syndrome: Its role in congenital single lung and post pneumonectomy situations.

BACKGROUND: Postpneumopnectomy syndrome (PPS) is an extreme rotation and malposition of mediastinum causing dynamic and symptomatic central airway compression, arisingafter 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 to 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.

Importance of Acute Anterior Angulation in Double Aortic Arch Needing Attention at Primary Surgery.

BACKGROUND: Vascular rings are rare congenital abnormalities of the aortic arch. There are many embryological variants including a double aortic arch. In symptomatic children, division of ring and release of airway structures may be sufficient. Persistence of symptoms can be related to an anterior angle formed between the two arches. The aim of this study is to evaluate the clinical efficacy in improving symptoms and on changing this angle at the primary surgery. METHODS: All children who had surgery for double aortic arch between 2005 and 2020, were studied. Relevant factors were analyzed for persistent symptoms including anatomical substrates and surgical details. RESULTS: A total of 87 out of 224 children had surgery for a double aortic arch. At presentation, airway symptoms (n = 74/87) were more common than esophageal symptoms (n = 27/87). Early onset symptoms within 1 year were seen in 49 children. In addition to division of one arch, surgical steps also included realigning the anterior left arch, thereby eliminating the acute angle in 36 children (after 2014). After surgery, symptom relief within 12 months following surgery was seen in 64% of children (56 out of 87) but in 27 out of 36 children (75%) with additional surgical modification, as against 29 out of 51 (57%) in those with division of the arch. Symptoms persisted beyond 1 year needing reintervention in eight children. CONCLUSION: Anterior arch angulation plays an important role in double aortic arch by causing a "nutcracker" phenomenon. Repair in double aortic arch should consider this aspect and include modification of surgical steps by realigning the corresponding aortic arch branches and an anterior pexy in selected cases.

Bronchial Dieulafoy's Disease in Children: A Case Report and Review of Literature.

Dieulafoy's disease is a rare vascular lesion characterized by presence of large aberrant arteries within the submucosa of gastrointestinal tract or respiratory tract with a potential to cause life-threatening hemorrhage. Treatment includes bronchoscopy ablation, angiographic embolization or surgery. We report management of 7-year old girl with Dieulafoy's disease in the airway who presented with recurrent hemoptysis. Bronchial angiography revealed multiple feeding vessels to the lesion. Considering the potential risk of recurrence with embolization, sleeve resection of bronchus offered complete resolution. This case demonstrates the usefulness of bronchial angiography as part of multi-faceted approach before surgery in the management of Dieulafoy's disease.

Management of Tracheal Diseases in Children.

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 for the treatment of tracheo - broncho - malacia in children.

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.

Staging of Surgical Procedures in Comorbid Congenital Tracheal Stenosis and Congenital Cardiovascular Disease.

BACKGROUND: Comorbid long segment congenital tracheal stenosis and congenital cardiovascular abnormalities in children pose significant challenges with regard to repairing these abnormalities simultaneously or in stages. The aim of this study was to explore whether this combination of abnormalities needs a staged approach for surgical repairs. METHODS: All children who underwent both tracheal and cardiac surgical procedures at a tertiary hospital from 1995 to 2018 were analyzed retrospectively for mortality, ventilation days, postoperative intensive care unit days, mediastinitis, and unplanned reoperation by dividing them into simultaneous repairs (group 1), staged repairs within the same admission (group 2), and staged repairs during different admissions (group 3). RESULTS: Of 110 patients included in the study (group 1, 74; group 2. 10; and group 3, 26 patients), there was no significant difference in mortality (P = .85), median ventilation days (P = .99), median intensive care unit days (P = .23), unplanned airway reoperation (P = .36), and unplanned cardiac reoperation (P = .77). There was a significant difference in the rate of mediastinitis (group 1, 3%; group 2, 10%; and group 3, 19%; P = .02). There was no significant difference in 5-year survival (group 1, 86.2%; group 2, 77.8%; and group 3, 85.1%; P = .86). A higher STAT category was identified to be a risk factor for mortality in multivariate Cox regression analysis (relative risk, 5.45). CONCLUSIONS: Combined tracheal and cardiac abnormalities need a stratified approach to facilitate better clinical outcomes. Although the trajectory of care is often based on the clinical presentation, establishing a management protocol will be helpful, for which setting an international database will be useful.

Autologous pedicled pericardial patch repair of tracheobronchial defect.

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.

Thoracoscopic aortopexy for symptomatic tracheobronchomalacia.

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 < 0.05 as significant. 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).

Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT).

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 <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered. RESULTS: Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029). CONCLUSION: The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments. LEVEL OF EVIDENCE: NA Laryngoscope, 130:E243-E251, 2020.

Life-threatening mesenchymal hamartoma of the chest wall in a neonate.

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.

Tailored Management of Airway Abnormalities in Children with Congenital Single Functional Lung.

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.

Button battery ingestion in children-a potentially catastrophic event of which all radiologists must be aware.

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.