Plastic bronchitis during childhood: Diversity of presentation, etiology, treatment, and outcomes.

OBJECTIVE: Plastic bronchitis (PB) is a rare disease in children, and reliable data are scarce. Here, we aimed to analyze the clinical features, management, and outcomes in children with PB. METHODS: The medical data of patients who were followed up with a diagnosis of PB between January 2010 and March 2022 were retrospectively analyzed. RESULTS: The median age of 15 patients was 9 (interquartile range: 4-10) years with a male/female ratio of 12/3. Initial symptoms included recurrent pneumonia (33.3%), persistent atelectasis (33.3%), cast expectoration (26.6%), and intense, persistent cough (6.6%). The most common underlying diagnosis was asthma (n = 12, 80%), and six of the patients were newly diagnosed. The most common radiological findings were atelectasis as a consequence of major airway obstruction on chest X-ray or computed tomography. Five patients, all diagnosed as having asthma, had recurrent PB and required multiple airway procedures for treatment and diagnosis. During a median 7-year follow-up of five patients, occasionally cast expectoration was observed in one patient with asthma who had poor compliance with inhaled corticosteroids. CONCLUSION: PB is a common reflection of the different underlying etiologies in the pediatric age group, and treatment and outcomes are closely related to these. It should be kept in mind that asthma can be a predisposing factor for the development of PB.

Transvenous retrograde thoracic duct embolization for effective treatment of recurrent plastic bronchitis after fontan palliation.

We report the case of a 5.5-year-old patient (16 kg/105 cm) who presented with plastic bronchitis (PB) refractory to conservative treatment 3 months after completion of Fontan palliation. Bi-inguinal transnodal fluoroscopy-guided lymphangiogram confirmed the chylous leak originating from the thoracic duct (TD) into the chest and did not opacify any central lymphatic vessel for direct transabdominal puncture. Retrograde transfemoral approach was adopted to catheterize the TD and selectively embolize its caudal portion using microcoils and liquid embolic adhesive. Recurrence of symptoms after 2 months indicated a redo catheterization to occlude the TD entirely using the same technique. The procedure was successful and the patient was discharged after 2 days with sustained clinical improvement at 24 months postoperative. In the context of refractory PB, end-to-end transvenous retrograde embolization of the TD appears to be an interesting alternative to more complex interventions such as transabdominal puncture, decompression, or surgical ligation of the TD.

[Plastic bronchitis].

Plastic bronchitis (PB) is a rare disease caused by abnormal lymphatic vessels in the thorax. These vessels drain into the bronchi creating solid casts and potential life-threatening airway obstruction. This is a case report of a 30-year-old man diagnosed with PB after several years of extensive examinations due to symptoms misconceived as non-allergic asthma. We describe the first interventional treatment in Denmark using special T2 weighed MR imaging and dynamic contrast MR lymphangiography with subsequent embolisation of abnormal lymphatic vessels in the thorax.

Plastic bronchitis: A rare complication following a motor vehicle collision.

Plastic bronchitis, more appropriately termed chyloptysis, is a rare and potentially fatal condition caused by chylous coating of the airways. These cast coating can dislodge and become an obstructive mass in the patient's airway, necessitating rapid intervention. PB is well described to occur following single ventricle physiology heart disease corrective procedures, particularly following Fontan procedures. It is less commonly seen in traumatic settings. We present the youngest known case of a traumatic injury induced plastic bronchitis. A 19-year-old man was involved in a motor vehicle accident with airbag deployment. The airbags struck him in the chest; however, the patient felt well at the time and did not seek medical attention. Several months later the patient began coughing up milky white masses identified as casts. He was initially diagnosed with asthma but did not respond to therapy. He ultimately was found to have evidence of thoracic duct injury. Options for therapy were discussed, including possible thoracic duct ligation. The patient opted to continue a lowfat diet and has remained cast free. This case highlights the importance of considering plastic bronchitis in patients with cast production and a history of trauma to the chest.

Transcatheter Thoracic Duct Decompression for Multicompartment Lymphatic Failure After Fontan Palliation.

BACKGROUND: Lymphatic embolization therapy has proven effective for Fontan failure from plastic bronchitis or protein-losing enteropathy but not when multiple lymphatic compartments are involved; furthermore, embolization does not alter the underlying pathophysiology of lymphatic dysfunction. A technique for transcatheter thoracic duct decompression (TDD), rerouting the thoracic duct to the pulmonary venous atrium to treat multicompartment lymphatic failure is described and early outcomes presented. METHODS: Initially covered stents were used to channel the innominate vein flow inside of the cavopulmonary pathway into the pulmonary venous atrium. A modified approach was developed where covered stents redirected innominate vein directly to the left atrium via an extravascular course. Baseline and follow-up data on all patients undergoing TDD were reviewed. RESULTS: Twelve patients underwent TDD between March 2018 and February 2021 at a median age of 12 (range: 2-22) years. Lymphatic failure occurred in median of 3 compartments per patient (protein-losing enteropathy, ascites, pleural effusions, plastic bronchitis); 10 patients had lymphatic embolizations before TDD. TDD method was intra-Fontan tunnel in 4, direct approach in 7, and other in 1. There were no major procedural complications; 6 patients underwent subsequent procedures, most commonly to treat endoleaks. Lymphatic failure resolved in 6 patients, improved in 2, and was unchanged in 4 at 6 (range: 1-20) months follow-up. One patient died after TDD from Fontan failure. CONCLUSIONS: TDD is a promising new treatment for the failing Fontan physiology from multicompartment lymphatic failure. Additional work is needed to refine the technique and define optimal candidates.

Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies.

Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially devastating complications of the Fontan circulation. PB occurs in ∼4% of Fontan patients, typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing, fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and elevated fecal alpha-1 antitrypsin 1. These disorders have similar pathophysiology involving disruption of the lymphatic system resulting from elevated central venous pressure combined with elevated lymphatic production and inflammation, resulting in lymphatic drainage into low-pressure circuits such as the airways (PB) and duodenum (PLE). Our understanding of these disorders has greatly improved over the past decade as a result of advances in imaging of the lymphatic system through magnetic resonance lymphangiography and early success with lymphatic interventions including lymphatic embolisation, thoracic duct embolisation, and percutaneous thoracic duct decompression. Both PB and PLE require a multidisciplinary approach that addresses and optimises residual hemodynamic lesions through catheter-based intervention, lowers central venous pressure through medical therapy, minimises symptoms, and targets abnormal lymphatic perfusion when symptoms persist. This review summarises the pathophysiology of these disorders and the current evidence base regarding management, proposes treatment algorithms, and identifies future research opportunities. Key considerations regarding the development of a lymphatic intervention program are also highlighted.

Nursing Progress of Hypertonic Saline Inhalation in the Treatment of Infantile Bronchitis Based on Image Enhancement.

The onset of bronchiolitis is closely related to the anatomical characteristics of the bronchi in children of this age. This kind of injury is caused by epithelial necrosis, nasal mucosa, and mucosal edema caused by narrowing and blockage of the trachea. Children with this serious phenomenon will have respiratory and heart failure, which threatens the life of children to a large extent. In this paper, based on image enhancement technology, hypertonic saline aerosol inhalation treatment of pediatric bronchiolitis nursing care, through related cases, the application of image enhancement technology in hypertonic saline aerosol inhalation therapy and pediatric bronchiolitis is analyzed, and the tone mapping function is used. Tone mapping functions, hereditary arithmetics, and slope regimes for experimental field capture and detection were used for the objective of therapeutic approaches for the treatment of pediatric capillary pneumonia by hypertonic inhalation. Experimental results show that imaging technology hypertonic inhalation can control the main symptoms of bronchiolitis in infants and young children. Inhalation of 3% saline can shorten the course of moderately chronic children to half a year and can reduce the length of hospital stay by a quarter of the original requires hospitalization time, and the cure rate of pediatric bronchiolitis is increased to 93.7%.

Tracheobronchitis in children with tracheostomy tubes: Overview of a challenging problem.

Tracheobronchitis is common in children with tracheostomy tubes. These children are predisposed to respiratory infections due to the bypassing of normal upper airway defense mechanisms by the tracheostomy, bacterial colonization of the tracheostomy tube itself, and underlying medical conditions. Diagnosis of bacterial tracheobronchitis is challenging due to the difficulty in differentiating between bacterial colonization and infection, as well as between viral and bacterial etiologies. Difficulty in diagnosis complicates management decisions, and there are currently no consensus guidelines to assist clinicians in the treatment of these patients. Frequent administration of systemic antibiotics causes adverse effects and leads to the emergence of resistant organisms. Topical administration of antibiotics via nebulization or direct instillation may lead to a significantly higher concentration of drug in the upper and lower airways without causing systemic side effects, although therapeutic trials in children with tracheostomy tubes are lacking. Several preventative measures such as regular airway clearance and the use of a speaking valve may mitigate the risk of developing respiratory infections.

Current Treatment Options for the Failing Fontan Circulation.

The Fontan operation was introduced in 1968. For congenital malformations, where biventricular repair is unsuitable, the Fontan procedure has provided a long-term palliation strategy with improved outcomes compared to the initially developed procedures. Despite these improvements, several complications merely due to a failing Fontan circulation, including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein-losing enteropathy, hepatic dysfunction, plastic bronchitis, and thrombo-embolism, may occur, thereby limiting the life-expectancy in this patient cohort. This review provides an overview of the most common complications of Fontan circulation and the currently available treatment options.

Fibrin airway cast obstruction: Experience, classification, and treatment guideline from Denver.

BACKGROUND AND OBJECTIVES: Plastic bronchitis (PB) is a condition characterized by the formation of thick airway casts leading to acute and often life-threatening airway obstruction. PB occurs mainly in pediatric patients with congenital heart disease (CHO) who have undergone staged surgical palliation (Glenn, Fontan), but can also occur after chemical inhalation, H1N1, severe COVID-19, sickle cell disease, severe asthma, and other diseases. Mortality risk from PB can be up to 40%-60%, and no treatment guideline exist. The objectives herein are to develop a standardized evaluation, classification, and treatment guideline for PB patients presenting with tracheobronchial casts, based on our experience with PB at the Children's Hospital of Colorado in Denver. METHODS: We describe 11 patients with CHO-associated PB (post-Fontan [n = 9], pre-Fontan [n = 2]) who presented with their initial episodes. We utilized histopathological analysis of tracheobronchial casts to guide treatment in these patients, utilizing our hospital-wide guideline document and classification system. RESULTS: We found that 100% of post-Fontan PB patients had fibrinous airway casts, while pre-Fontan PB casts were fibrinous only in one of two patients (50%). Utilizing histopathology as a guide to therapy, PB patients with fibrin airway casts were treated with airway-delivered fibrinolytics and anticoagulants, as well as aggressive airway clearance and other supportive care measures. These therapies resulted in successful cast resolution and improved survival in post-Fontan PB patients. CONCLUSION: We have shown an improved outcome in PB patients whose treatment plan was based on Denver's PB classification schema and standardized treatment guideline based on tracheobronchial cast histopathology.

Acute plastic bronchitis after Ross procedure treated with veno-venous extracorporeal membrane oxygenation.

PURPOSE: Plastic bronchitis is a rare condition characterized by the formation of airway casts occluding the lower respiratory tract. It is described more commonly in children, especially following correction of congenital heart disease. It involves lymphatic abnormalities leading to endobronchial lymph precipitating airway cast formation. When it presents acutely, it can lead to acute airway obstruction, which can be life-threatening. Plastic bronchitis has been rarely described in adults and is potentially underdiagnosed. The purpose of this case report is to emphasize, for the adult anesthesiologist and adult critical care physician, the importance of prompt diagnosis and respiratory support in a case of plastic bronchitis. CLINICAL FEATURES: A 40-yr-old female with severe aortic stenosis underwent a Ross procedure. The surgery was uneventful, but within two hours of arrival in the intensive care unit, the patient developed severe hypoxemia. Despite attempts to optimize her respiratory status, the patient remained severely hypoxemic, and veno-venous extracorporeal membrane oxygenation (ECMO) was initiated using a percutaneous femoro-femoral cannulation. A bronchoscopy showed bronchial secretions casting the proximal bronchus, suggestive of plastic bronchitis. After numerous bronchoscopies, we were able to clean the airways and wean the ECMO support on postoperative day 3. CONCLUSION: Plastic bronchitis can present in adult patients and be life-threatening when associated with acute respiratory failure. We report an unusual case of an adult patient treated with veno-venous ECMO for plastic bronchitis following cardiac surgery. Use of ECMO support while providing airway cleaning can be lifesaving in patients with respiratory failure secondary to plastic bronchitis.

RéSUMé: OBJECTIF: La bronchite plastique est une affection rare caractérisée par la formation de bouchons muqueux qui moulent et obstruent les voies aériennes inférieures. Elle est plus fréquemment décrite chez les enfants, en particulier après la correction d'une cardiopathie congénitale. Elle découle d'anomalies lymphatiques conduisant à l'accumulation de lymphe endobronchique, précipitant la formation de bouchons muqueux dans les voies aériennes. Lorsqu'elle se présente de manière aiguë, la bronchite plastique peut entraîner une obstruction aiguë des voies aériennes, une complication potentiellement fatale. La bronchite plastique a rarement été décrite chez l'adulte et est potentiellement sous-diagnostiquée. L'objectif de cette présentation de cas est de souligner, pour l'anesthésiologiste et l'intensiviste s'occupant d'une population adulte, l'importance d'un diagnostic rapide et d'un support respiratoire en cas de bronchite plastique. CARACTéRISTIQUES CLINIQUES: Une femme de 40 ans souffrant d'une sténose aortique sévère a bénéficié d'une procédure de Ross. La chirurgie s'est déroulée sans incident, mais dans les deux heures suivant son arrivée à l'unité de soins intensifs, la patiente a présenté une hypoxémie sévère. Malgré les tentatives d'optimisation de son état respiratoire, la patiente est restée gravement hypoxémique et une oxygénation par membrane extracorporelle (ECMO) veino-veineuse a été amorcée à l'aide d'une canulation fémoro-fémorale percutanée. Une bronchoscopie a montré des sécrétions bronchiques moulant les bronches proximales, évoquant une bronchite plastique. Après de nombreuses bronchoscopies, nous avons pu nettoyer les voies aériennes et sevrer la patiente du soutien ECMO au 3ème jour postopératoire. CONCLUSION: La bronchite plastique peut se présenter chez les patients adultes et être potentiellement fatale lorsqu'elle est associée à une insuffisance respiratoire aiguë. Nous rapportons un cas inhabituel d'une patiente adulte traitée par ECMO veino-veineuse pour une bronchite plastique après une chirurgie cardiaque. L'utilisation du soutien par ECMO simultanément au nettoyage des voies aériennes peut être nécessaire chez les patients atteints d'insuffisance respiratoire secondaire à une bronchite plastique.

Plastic Bronchitis in Adult and Pediatric Patients: A Review of its Presentation, Diagnosis, and Treatment.

PURPOSE OF STUDY: Plastic Bronchitis (PB) is a rare pulmonary condition characterized by the presence of casts in the trachea or bronchial tree. While there are many individual cases reported in pediatric and adult populations, no thorough reviews of pediatric and adult cases of PB exist in the literature. The purpose of this article is to conduct a comprehensive review of PB regarding presentation, diagnosis, pathophysiology, and treatments. ETIOLOGY: In the pediatric population, PB can be attributed to pediatric cardiothoracic surgeries such as Fontan procedures, infections, inflammatory processes, acute chest syndrome, or iatrogenic processes. In the adult population, PB can be idiopathic or due to infections, anatomic variations in lymphatic vessels, surgeries, medications, or other comorbidities. PATHOPHYSIOLOGY: The pathophysiology of PB is still widely unknown; however, associations with inflammatory diseases and cardiac surgery have been proposed. There are two types of cast formations found in plastic bronchitis: Type I casts are associated with inflammatory diseases and Type II casts are associated with surgical procedures. TREATMENT: Historically, PB has been treated by a variety of pharmacological methods including the use of corticosteroids and mucolytics. Recently, the treatment paradigm has shifted towards procedures such as lymphatic embolization, duct ligation, and stent grafting. CONCLUSIONS: The information available regarding PB is still sparse, hence future research is necessary for further understanding of the disease. Due to its numerous presentations and disease associations, awareness of plastic bronchitis, and its treatment options is essential for primary care providers and respiratory specialists.

Recycling plastic: diagnosis and management of plastic bronchitis among adults.

Plastic bronchitis is a rare, underdiagnosed and potentially fatal condition. It is characterised by the formation and expectoration of branching gelatinous plugs that assume the shape of the airways. These airway plugs differ from the allergic mucin that characterises allergic bronchopulmonary aspergillosis and mucoid impaction of the bronchi. Plastic bronchitis is most often encountered in the paediatric population following corrective cardiac surgery, such as the Fontan procedure. It also occurs in adults. Plastic bronchitis in adults is rare, heterogeneous in its aetiology, and can lead to respiratory distress or even life-threatening airway obstruction. Plastic bronchitis in adulthood should not be overlooked, particularly in patients with chronic inflammatory lung diseases. This review presents current understanding of the presentation, aetiology, pathogenesis, pathology and management of plastic bronchitis in adults.

Bronchoscopic interventions for plastic bronchitis in children without structural heart disease.

Plastic bronchitis (PB) is a rare and life-threatening complication encountered in several disease states that leads to airway obstruction by branching casts. PB is most often reported in children with cyanotic congenital heart disease where recurrence is common, and mortality is high. There is limited data on optimal management strategies or recurrence of non-structural heart disease-related PB in children. We describe the clinical features, management, and outcomes in our cohort of children with non-structural heart disease-related PB. Among the 12 identified patients, asthma was the most common (67%) diagnosis. Ventilatory requirements ranged from room air to one patient who required extracorporeal membrane oxygenation (ECMO). Most patients (92%) required bronchoscopy, and cryotherapy was successfully utilized in two patients to relieve refractory obstructive airway casts. All patients received chest physiotherapy, and 11 patients were treated with two or more medications. There was one mortality despite ECMO, and one-third had recurrent PB, all of whom had asthma.Conclusion: Asthma is a risk factor for recurrent PB. Bronchoscopic interventions including cryotherapy are safe and effective treatment options in patients with refractory PB. What is Known: • Plastic bronchitis is a rare but life-threatening cause of airway obstruction caused by branching casts that are generally reported in patients with congenital heart disease. What is New: • In children without structural heart disease, asthma is a risk factor for recurrent plastic bronchitis. Cryotherapy via bronchoscopy is a safe and effective intervention in patients with refractory plastic bronchitis.

miR-122 promotes virus-induced lung disease by targeting SOCS1.

Virus-induced respiratory tract infections are a major health burden in childhood, and available treatments are supportive rather than disease modifying. Rhinoviruses (RVs), the cause of approximately 80% of common colds, are detected in nearly half of all infants with bronchiolitis and the majority of children with an asthma exacerbation. Bronchiolitis in early life is a strong risk factor for the development of asthma. Here, we found that RV infection induced the expression of miRNA 122 (miR-122) in mouse lungs and in human airway epithelial cells. In vivo inhibition specifically in the lung reduced neutrophilic inflammation and CXCL2 expression, boosted innate IFN responses, and ameliorated airway hyperreactivity in the absence and in the presence of allergic lung inflammation. Inhibition of miR-122 in the lung increased the levels of suppressor of cytokine signaling 1 (SOCS1), which is an in vitro-validated target of miR-122. Importantly, gene silencing of SOCS1 in vivo completely reversed the protective effects of miR-122 inhibition on RV-induced lung disease. Higher miR-122 expression in nasopharyngeal aspirates was associated with a longer time on oxygen therapy and a higher rate of treatment failure in 87 infants hospitalized with moderately severe bronchiolitis. These results suggest that miR-122 promotes RV-induced lung disease via suppression of its target SOCS1 in vivo. Higher miR-122 expression was associated with worse clinical outcomes, highlighting the potential use of anti-miR-122 oligonucleotides, successfully trialed for treatment of hepatitis C, as potential therapeutics for RV-induced bronchiolitis and asthma exacerbations.