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Background: This study evaluates whether dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) and thoracic duct lymphangiography (TDL) findings in adults with lymphatic plastic bro...

Congenital heart disease can lead to notable lymphatic complications such as chylothorax, plastic bronchitis, protein-losing enteropathy, and ascites. Recent improvements in lymphatic imaging and the development of new lymphatic procedures can help alleviate symptoms and improve outcomes.

Background Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are devastating complications after Fontan palliation that lead to uncontrolled loss of protein-rich lymphatic fluid into extra lymphatic compartments. Decompression of the thoracic duct is a new treatment option that effectively restores lymphatic system integrity by redirecting lymphatic flow into the low-pressure levels of the common atrium. Case summary We report a patient with severe failing Fontan circulation where surgical thoracic duct decompression leads to resolution of PLE and PB symptoms but worsening hypoxaemia that could be managed with banding of internal jugular vein. Discussion Thoracic duct decompression in patients with failing Fontan circulation can be a simple and effective treatment for PLE and PB. Hypoxaemia may occur but can be managed with banding of internal jugular vein.

Dynamic contrast-enhanced magnetic resonance lymphangiography is a novel technique to image central conducting lymphatics. It is performed by injecting contrast into groin lymph nodes and following passage of contrast through lymphatic system using T1-weighted MR images. Currently, it has been successfully applied to image and plan treatment of thoracic duct pathologies, lymphatic leaks, and other lymphatic abnormalities such as plastic bronchitis. It is useful in the assessment of chylothorax and chyloperitoneum. Its role in other areas such as intestinal lymphangiectasia and a variety of lymphatic anomalies is likely to increase.

Plastic bronchitis (PB) is a rare disease characterized by the presence of mucofibrinous plugs which may occlude and conform the shape of tracheobronchial tree. These casts are exteremely cohesive. Most common presenting symptoms include cough, fever and dyspnea and if the cohesive casts occlude the airway totally life-threatening complications and even death may occur. The aim of treatment is to remove the casts and adress the symptoms. In this study a 6-year-old boy with PB is presented. In addition to medical treatment obstructing casts were removed via rigid bronchoscope. It is aimed to review the clinical and radiographic features and choices of treatment in this disease.

Plastic bronchitis (PB) is a severe acute respiratory disease that develops as a result of the formation of branching mucus plugs in the bronchial tree. PB is known as a complication of influenza A virus infection, but some cases have been associated with influenza B virus infections. This patient was a 3-year-old boy with no history of allergic disease who developed PB requiring ventilator management after influenza B virus infection. He was hospitalized and managed with ventilator support because of acute respiratory failure. Influenza B virus infection was diagnosed via rapid antigen test and real-time reverse-transcription polymerase chain reaction (RT-PCR). A bronchoscopy performed after a chest X-ray and computed tomography confirmed the presence of extensive atelectasis in the right lung field and mucus plugs in the right bronchus. The patient's respiratory condition improved rapidly after removal of the plugs. Quantitative real-time RT-PCR performed with nasal and aspirated sputum samples obtained at hospitalization revealed a higher viral RNA load in the upper rather than in the lower respiratory tract. Viral replication in the lower respiratory was not found to be a major contributor toward mucus plug formation. The finding of increased serum IgE in the absence of a history of allergic disease suggests that an allergic reaction contributed to the formation of mucus plugs.

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.

Plastic bronchitis is a rare and fatal disease that is characterized by the presence of long dendritic bronchial casts in the airway. It is encountered most frequently in children with congenital heart disease after correction surgery. We reported a case of plastic bronchitis after mitral valve surgery in a 70‐year‐old woman., After after mitral valve surgery in a 70‐year‐old woman, there was a rubbery and mucinous bronchial cast, which was extracted by bronchoscopy. On histologic examination, the cast mainly comprised mucous material and few histiocytes; there was no eosinophils and neutrophils, which indicated acellular plastic bronchitis.

Background In the past few years, Mycoplasma pneumoniae (Shi et al. Lancet 390:946–958, 2017) infection has been reported more in China. However, there are few studies on the clinical characteristics and prognosis of necrotizing pneumonia (NP) (Griffiths et al. Nature 583:615–619, 2020) caused by different pathogens. Methods A retrospective analysis was performed, including 31 children with a clinical diagnosis of NP in the hospital from January 1, 2013 to January 31, 2020. A total of 11 children with MPNP were included in the observation group and the other 20 children with other pathogens were included in the control group. The clinical manifestations, laboratory data, imaging findings, treatments and outcomes were analyzed. Results The proportion of dyspnea cases was significantly higher in the non-Mycoplasma pneumoniae necrotizing pneumonia (N-MPNP) group than that in the Mycoplasma pneumoniae necrotizing pneumonia (MPNP) group (P = 0.02).The LDH level of all patients in the MPNP group was higher than the normal value, with a median value of 805.0 U/L, which was significantly higher than those in the N-MPNP group (414.0 [299.9–540.6] U/L; Z = − 2.518; P = 0.012). The white blood cells (WBCs) count of the N-MPNP group was 17.8 (11.1–21.7) × 109/L, which was significantly higher than that of the MPNP group (10.2 [6.3–14.1] × 109/L; P t = 3.101; P = 0.004). The incidence of pleural effusion in the N-MPNP group (19 patients, 95%) was significantly higher than that in the MPNP group (six patients, 54.55%) (P = 0.013). Among them, two patients received bronchoscopy lavage at a maximum four times, and the cases of plastic bronchitis were seen only in the MPNP group (3 cases; P = 0.037).The length of stay was 18 (10–22) days in the MPNP group and 23.5 (13.5–47) days in the N-MPNP group and no significant difference was observed between the two groups (Z = − 1.923, P = − 0.055). Conclusions MP infection is the most common infection in children with NP in the Suzhou area. There is no gender and age difference between MPNP and N-MPNP, but the bacterial infection was mainly observed in the N-MPNP group. Children in the N-MPNP group have more severe clinical symptoms, were more prone to shortness of breath, had a longer hospital stay, and had earlier imaging manifestations of necrosis, whereas children in the MPNP group were more likely to have plastic bronchitis. The level of WBC and LDH and the nature of pleural effusion can be used to identify MPNP and N-MPNP to some extent. The prognosis of MPNP was better than that of N-MPNP. There were no death cases. Pleural thickening, pulmonary fibrosis, and bronchiectasis were the most common sequelae. Compared with N-MPNP, the recovery time of lung imaging in MPNP was shorter.

BackgroundPlastic bronchitis (PB) combined with nephrotic syndrome (NS) is rare, and the pathophysiological relationship between PB and NS has not been elucidated. We report a case of an 8-year-old boy with life-threatening PB caused by an influenza infection during a relapse of NS. Case presentationThe patient was on immunosuppressive drugs for NS. He developed fever due to an influenza A virus infection, followed by respiratory distress and frequent vomiting. Prednisolone was administered for possible bronchial asthma and NS. His respiratory status deteriorated rapidly, which required ventilator management. A large mucus plug was aspirated using bronchoscopy. He was then diagnosed with PB caused by the influenza A virus. Increased lower airway secretion and fluid leakage into the airways by relapse of NS were considered the causes of mucus plug formation. Besides, the decreased circulating blood volume might have made the bronchial secretions viscous with cast formation.ConclusionsPediatric patients with NS may be at a higher risk of developing PB. As PB is a life-threatening condition, patients with NS should be closely monitored when simultaneously infected with influenza virus.

Plastic bronchitis (PB) is a rare pediatric respiratory disease, characterized by the formation of obstructive casts in the bronchial tract that causes partial or extensive airway obstruction, leading to obstructive dyspnea mimicking a foreign body in the trachea. The clinical presentation and radiologic examination of plastic bronchitis are nonspecific, and the confirmation of the diagnosis is only possible via the direct observation of the casts via bronchoscopy or expectorating. So far, no effective treatment for PB has been demonstrated in controlled clinical trials, and presently, there are no reports regarding the use of α-chymotrypsin as a treatment modality. α-Chymotrypsin, as a mucolytic agent, liquefies the mucus and decreases the viscosity of sputum by acting directly on mucus. Here, we report a PB case that is associated with influenza A virus infection, developing in an eight-year-old boy. The diagnosis of PB was confirmed via cast observation following its removal via bronchoscopy. Specifically, the casts were successfully removed via bronchoscopy coupled with endotracheal instilled α-chymotrypsin. Thereafter, the patient gradually improved and successfully extubated. In the clinical follow-up, the patient was asymptomatic and without recurrent casts. Therefore, α-chymotrypsin may be one modality of treatment to remove casts in PB.

Background. To analyze the clinical features of children with plastic bronchitis (PB) and identify the risk factors of multiple flexible fiberoptic bronchoscopy (FOB) therapy.Methods. Retrospective analysis was performed on 269 PB children from 2016 to 2019, 144 cases were in single FOB group, 125 cases were in the multiple FOB group. The clinical manifestations, laboratory datas, imaging findings and management were investigated. The different features were compared between the single FOB group and multiple FOB group.Results. A total of 269 PB children were collected with a mean age of 6.7 ± 2.8 years. 257 (95.5%) cases were diagnosed as Mycoplasma pneumonia (MP) infection. The mean duration of fever was 10.6 ± 3.7 days. All the patients presented with fever, and 62 (23.0%) suffered from hypoxemia, 144 (53.5%) had extrapulmonary complications. Higher levels of ESR, CRP, PCT, IL-6, LA, LDH, FER and D-dimer were observed. The proportion of pulmonary consolidation, segmental or lobar atelectasis, pleural effusion and pleural thickening were 97.4%, 46.5%, 47.9% and 63.2%, respectively. Furthermore, multivariate logistic regression analysis showed that N% >75.5%, LDH >598.5U/L, and D-dimmer>0.45mg/L were independent isk factors for multiple FOB therapy. Conclusions. MP is a significant pathogen of PB in children. Patients with PB are more likely to suffer from persistent fever, excessive inflammation and severe radiological findings. N% >75.5%, LDH >598.5U/L and D-dimmer > 0.45mg/L may be predictors of multiple FOB treatment.

Plastic bronchitis is a rare post-Fontan complication with limited treatment options. Heart transplantation has evolved as a potential curative option, but outcomes have not been well-defined. This study aims to assess contemporary waitlist and post-transplant outcomes in patients with plastic bronchitis. All Fontan patients were identified in the PHTS database (2010 - 2018). Waitlist and post-transplant outcomes were compared between Fontan patients with and without plastic bronchitis. Competing outcomes and Kaplan-Meier analyses were used to assess the impact of plastic bronchitis on waitlist and post-transplant survival. A secondary analysis excluded those with PLE from the comparison cohort. Of 645 Fontan patients listed for heart transplant, 69 (11%) had plastic bronchitis. At listing, patients with plastic bronchitis were younger (8.9 vs 11.1 years, P = .02), but had few other differences in baseline characteristics. A fewer Fontan patients with plastic bronchitis were listed in the more recent era (46 [15.4%] in 2010-2014 vs 23 [6.6%] in 2015-2018, P < .01). Overall, there was no difference in waitlist (P = .30) or post-transplant (P = .66) survival for Fontan patients with and without plastic bronchitis. The results were similar after excluding patients with PLE. Contrary to prior reports, this relatively large series showed that plastic bronchitis did not have a negative impact on survival to or after heart transplantation in Fontan patients. Our study also found a 50% reduction in listing in the current era, which may indicate evolution in management of Fontan patients.

Plastic bronchitis is an uncommon condition with a poor prognosis characterized by an obstructive respiratory pattern due to airway blockage by the presence of bronchial casts. Therefore, it is important to include it in the differential diagnosis of respiratory distress, particularly in patients with underlying pathologies predisposing to this condition. The etiology is multifactorial, but typically occurs in children with congenital heart disease, particularly post‐Fontan procedure. The treatment is based on improving the underlying condition, which can be challenging in this type of patients.

Background With an increase in the diagnosis of plastic bronchitis (PB) cases, to enhance paediatricians’ knowledge and add to the few existing studies, we explored the clinical characteristics, diagnosis, and treatment of PB in children. Methods The clinicopathological data of 43 children admitted to the Xiamen Children’s Hospital and the Women and Children’s Hospital, affiliated to the Xiamen University from December 2016 to December 2019, were retrospectively analysed. Results All the children had cough, with 41 of them having associated fever. A peak temperature > 40 ℃ was observed in 25 children. Twenty-six children had shortness of breath, 27 had reduced respiratory sounds on the affected side, and 35 had audible moist rales on the affected side. Lactate dehydrogenase in all children increased to different degrees, and 29 had elevated D-dimer and fibrinogen degradation products. Lung imaging showed pulmonary consolidation and atelectasis, mainly in the bilateral lower lung lobes, in all the children. However, 31 had pleural effusion, mainly a small parapneumonic effusion. The infections were mainly caused by adenovirus and Mycoplasma pneumoniae. The casts in all 43 children were sucked or clamped out under bronchoscopy, and 10 were found to have type I PB on pathological examination. All children were treated with anti-infective therapy in addition to bronchoscopic cast removal. Thirty-one children were treated with methylprednisolone, and 16 with gamma globulin. Except for one child who was non-adherent to treatment, all other children showed improvement, or were cured and discharged from the hospital. Follow-up lung imaging at 3 months revealed that the lungs were fully re-expanded in 40 children. At the 6-month follow-up, six children had small airway lesions, four had obliterative bronchiolitis, and one had bronchiectasis. Conclusions Paediatric PB often occurs secondary to respiratory tract infections and progresses rapidly, with hyperpyrexia, cough, and shortness of breath as the main clinical manifestations. Pulmonary consolidation, atelectasis, and pleural effusion are seen on lung imaging, and early bronchoscopy and removal of casts in the trachea and bronchi are effective treatment options.

Plastic bronchitis (BP) is usually an evolutionary complication of respiratory condi-tions such as asthma and / or pre-existing cardiac, and its occurrence in a patient without chronic respiratory pathology remains exceptional.?We report the observation of a seven-year-old child, followed for asthma, who presented with a pneumatic appearance of commonplace, massive atelectasis of the entire left lung. Endoscopic exploration of the tracheobronchial tree confirmed the extensive obstruction of the left tree by bronchial mussels. The evolution was favorable after the extraction and spontaneous rejection of intrabronchial mussels.

Plastic bronchitis (PB) is characterized by the formation of bronchial casts. It most frequently occurs in children with congenital heart disease, particularly post-Fontan procedure. Several medical and surgical therapies have been described in the literature with variable success. To our knowledge, this is the first time that overnight use of home high-flow nasal cannula is reported as a therapy to prevent recurrence of bronchial cast production in a child with PB post-Fontan.

Plastic bronchitis is a rare condition characterised by endobronchial cast formation. We report the case of a 53-year-old women who deteriorated following an elective bronchoscopy procedure. She developed refractory ventilatory failure and required repeated bronchoscopy, which identified thick tenacious casts as the cause of her airway compromise. She did not respond to conventional therapies including endoscopic clearance, mucolytic therapy and nebulised tissue plasminogen activator (TPA). Total parenteral nutrition and a fat-free enteral diet were instituted while the patient was on extracorporeal membrane oxygenation (ECMO), which led to substantial improvement in her condition and demonstrated the importance of dietary strategies in this case. LEARNING POINTS Plastic bronchitis can rarely present in adults with acute ventilatory failure and life-threatening airway obstruction. Although there are no established guidelines on management, dietary intervention (e.g., a fat-free diet) should be strongly considered as a therapeutic option. Extracorporeal membrane oxygenation (ECMO) is feasible and should be considered in plastic bronchitis with airway compromise.

Статья посвящена малоизвестной и актуальной проблеме пульмонологии – диагностике редкого хронического заболевания. Описываются этиология, клинические симптомы и методы диагностики пластического бронхита, на основании которых педиатр может заподозрить пластический бронхит. Приведен случай собственного клинического наблюдения у ребенка 10 лет. Отмечены трудности интерпретации клинических симптомов пластического бронхита в дебюте заболевания и недостаточная настороженность врачей педиатров в отношении данной патологии у детей., The article is devoted to the little-known and relevant problem of pulmonology, the diagnosis of a rare chronic disease. The etiology, clinical symptoms and diagnostic methods for plastic bronchitis are described, on the basis of which the pediatrician may suspect plastic bronchitis. A case of own clinical observation in a child of 10 years is given. Difficulties in interpreting the clinical symptoms of plastic bronchitis in the debut of the disease and insufficient alertness of pediatricians regarding this pathology in children are noted.

Key Clinical Message Any case of unresolving chronic pneumonia with expectoration of thick mucoid plugs should have high index of suspicion for plastic/cast bronchitis, requiring early flexible bronchoscopy with lavage and histopathologic evaluation. Associated presence of idiopathic chronic eosinophilic pneumonia is unusual and could be considered as a cause for atypical plastic bronchitis complicated by bronchiectasis.

Plastic bronchitis is the expectoration of bronchial casts in the mold of the tracheobronchial tree. It is a rare occurrence of unknown etiology that has been primarily described in children with congenital heart disease. In this case report, we present the first reported case of plastic bronchitis in a patient with pulmonary Kaposi sarcoma and underlying HIV infection.

We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Notably, clinical symptoms related to PLE were dramatically improved after starting BCV. A brief period of BCV increased stroke volume from 26±1.4 to 39±4.0 mL. This case suggests that BCV could be an effective treatment for PLE in patients with failing Fontan circulation.

Background Influenza A(H1N1)pdm09 virus infections often manifest severe respiratory symptoms, particularly in patients with a past history of allergic disease. Most of these findings were reported during the 2009 pandemic. The purpose of this study was to detail the clinical characteristics of influenza virus-induced lower respiratory infection (LRI) during the A(H1N1)pdm09-predominant 2015–2016 season. Methods We retrospectively reviewed the clinical characteristics of influenza-induced LRI cases in children admitted to a tertiary children's hospital. Molecular diagnostic evaluation was performed on samples obtained from the most severe cases. Results We identified 66 patients with influenza-associated hospitalization and included 21 patients with influenza virus-induced LRI for analyses. Twelve patients (57%) were admitted to the pediatric intensive care unit, seven (33%) required mechanical ventilation, and three (14%) required extracorporeal membrane oxygenation. Plastic bronchitis (PB) was identified in six patients (29%), among whom a past medical history of asthma or food allergy were noted in all six patients. A past history of allergic disease was more common among patients with, than among those without, PB (p = 0.009). A(H1N1)pdm09 was detected from all the PB cases, and phylogenetic analyses of the hemagglutinin and neuraminidase genes demonstrated that this virus belonged to subclades 6B.1 and 6B.2. In the six PB cases, we found one patient with H275Y mutation in neuraminidase. Conclusion Allergic disease was a risk factor for developing PB due to influenza A(H1N1)pdm09 infection during the 2015–16 season.

Impacted pediatric tracheobronchial thrombus is an uncommon diagnosis. There are no clearly proven therapeutic options for airway casts due to a similar process, plastic bronchitis. Cryotherapy, specifically cryoextraction, has shown potential as a therapeutic option in adults with tracheobronchial thrombus and cast. We describe the novel application of this method in a complex pediatric patient.

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.

Plastic Bronchitis is a rare condition in the pediatric population in which large casts fill the bronchi leading to airway obstruction. The classification scheme proposed by Seear divides the bronchial casts into inflammatory and acellular types. Inflammatory casts are most commonly seen with primary lung diseases, while acellular casts are frequently seen in children with congenital heart disease. Here, we present the case of a previously healthy child that presented with complete bronchial obstruction from plastic bronchitis requiring endoscopic removal.

Plastic bronchitis is a rare disease characterized by the formation of bronchial casts that can cause partial or complete obstruction of the airway.

Magnetic resonance imaging is increasingly being used to evaluate the lymphatic system. Advances in magnetic resonance (MR) software and hardware allow improved visualization of lymph nodes and lymphatic vessels. We describe how MR lymphangiography can be used to diagnose central lymphatic system anatomy and pathology, which can be used for diagnostic purposes or for pre-procedural planning.

Despite peritoneal dialysis being the preferred mode of renal replacement therapy in neonates and infants, long-term haemodialysis may be necessary in a minority of patients with its attendant risks. This case identifies plastic bronchitis as a rare yet serious complication of long-term large bore vascular access when a vessel-sparing approach is not possible. An appropriately sized catheter should be used for the dialytic therapy required and to optimize access survival.

Failing Fontan continues to be major problem for patients on the univentricular pathway. Failing Fontan is often complicated by chylothorax, plastic bronchitis and protein loosing enteropathy. The role of lymphatic circulation in Fontan circulation is still being researched. Newer imaging modalities give insight into the role of abnormal dilatation and retrograde flow in lymphatic channels post Fontan. Interventional strategies targeting abnormal lymphatic channels, provides an alternative management strategy for patients with failing Fontan. This review focuses on the role of lymphatic system in adaptations to Fontan circulation.