Etiology and clinical features of infection-associated plastic bronchitis in children.

OBJECTIVE: To investigate the etiological characteristics of plastic bronchitis (PB) caused by pulmonary infections in children and to identify any differences in the clinical features of PB cases caused by different pathogens. METHOD: We collected data on children diagnosed with PB and admitted to the Respiratory Department at Soochow University Children's Hospital between July 2021 and March 2023 utilizing electronic bronchoscopy. We analyzed clinical characteristics and the species of pathogens causing the illness in these children. RESULT: A total of 45 children were enrolled. The main clinical symptoms observed were cough (100%), fever (80%), shortness of breath (28.9%), and wheezing (20.0%). Pathogens were identified in 38 (84.4%) patients. Mycoplasma pneumoniae (MP) had the highest detection rate at 53.3%, followed by the Boca virus at 26.7%. MP-induced PB typically occurs in older children with an average age of 7.46 ± 2.36 years, with the main symptoms including high fever (85.7%) and local hyporespiration (42.9%). In contrast, Boca virus-induced PB tends to occur in younger children, with the main symptoms of moderate fever (54.5%), and wheezing (54.5%). The MP group exhibited a higher incidence of both internal and external pulmonary complications, including pleural effusion (42.9%), elevated aspartate aminotransferase (52.4%), lactic dehydrogenase (76.2%), and D-D dimer (90.5%). Conversely, the Boca virus group primarily showed pulmonary imaging of atelectasis (81.8%), with no pleural effusion. The average number of bronchoscopic interventions in the MP group was 2.24 ± 0.62, which was significantly higher than that required in the Boca virus group (1.55 ± 0.52). During the second bronchoscopy, 57.1% of children in the MP group still had visible mucus plugs, while none were observed in the Boca virus group. CONCLUSION: MP and Boca virus are the primary pathogens responsible for PB among children. The clinical manifestations of PB typically vary significantly based on the pathogen causing the condition.

Clinical features and risk factors of plastic bronchitis caused by refractory Mycoplasma pneumoniae pneumonia in children: a practical nomogram prediction model.

Early assessment of refractory Mycoplasma pneumoniae pneumonia (RMPP) with plastic bronchitis (PB) allows timely removal of casts using fiberoptic bronchoscopic manipulation, which relieves airway obstruction and limit sequelae development. This study aimed to analyze clinical data for risk factors and develop a nomogram for early predictive evaluation of RMPP with PB. The clinical data of 1-14 year-old patients with RMPP were retrospectively analyzed. Patients were classified into a PB or non-PB group. The general characteristics, clinical symptoms, laboratory test results, imaging findings, and microscopic changes of the two groups were compared. A statistical analysis of the risk factors for developing PB was performed, and a nomogram model of risk factors was constructed. Of 120 patients with RMPP included, 68 and 52 were in the non-PB and PB groups, respectively. Using multivariate logistic regression analysis, fever before bronchoscopy, extrapulmonary complications, pleural effusion, cough duration, and lactate dehydrogenase (LDH) levels were identified as risk factors. A nomogram was constructed based on the results of the multivariate analysis. The area under the receiver operating characteristic curve value of the nomogram was 0.944 (95% confidence interval: 0.779-0.962). The Hosmer-Lemeshow test displayed good calibration of the nomogram (p = 0.376, R2 = 0.723). CONCLUSION: The nomogram model constructed in this study based on five risk factors (persistent fever before bronchoscopy, extrapulmonary complications, pleural effusion, cough duration, and LDH levels) prior to bronchoscopy can be used for the early identification of RMPP-induced PB. WHAT IS KNOWN: • Refractory Mycoplasma pneumoniae pneumonia (RMPP) in children has been increasingly reported and recognized, which often leads to serious complications. • Plastic bronchitis (PB) is considered to be one of the causes of RMPP, and bronchoscopic treatment should be improved as soon as possible to remove plastic sputum thrombus in bronchus. WHAT IS NEW: • This study determined the risk factors for RMPP-induced PB. • The nomogram model constructed in this study prior to bronchoscopy can be used for the early identification of RMPP-induced PB, which facilitate the early bronchoscopic removal of casts, thereby promoting recovery and reducing cases with poor RMPP prognosis.

Clinical features and risk factors of plastic bronchitis caused by Mycoplasma pneumoniae pneumonia in children.

BACKGROUND: We analyzed the clinical characteristics of children with plastic bronchitis (PB) caused by Mycoplasma pneumoniae (MP) and explored its risk factors. METHODS: We prospectively analyzed clinical data of children with MP pneumonia (MPP) treated with fiberoptic bronchoscopy (FB). Patients were classified into a PB and non-PB group. General information, clinical manifestations, laboratory tests, results of computed tomography scan, and FB findings were compared between groups. We conducted statistical analysis of risk factors for developing PB. RESULTS: Of 1169 children who had MPP and were treated with FB, 133 and 1036 were in the PB and non-PB groups, respectively. There were no significant differences in sex, age, and incident season between groups (P > 0.05). The number of children in the PB group decreased during the COVID-19 pandemic. Compared with children in the non-PB group, those in the PB group had longer duration of hospitalization, increased levels of neutrophil (N), C-reactive protein (CRP), procalcitonin (PCT), D-dimer, lactate dehydrogenase (LDH), alanine transaminase (ALT) and aspartate transaminase (AST); lower levels of lymphocyte (L) and platelet (PLT); and higher incidence of lack of appetite, decreased breath sounds, single lobar infiltrate, pleural effusion, pericardial effusion, mucosal erosion and/or necrosis, and bronchial embolization. L levels and pleural effusion were identified as risk factors in multivariate logistic regression. CONCLUSIONS: Children with PB caused by MPP had a strong and local inflammatory response. L levels and pleural effusion were independent risk factors of PB with MPP in children. Our findings will help clinicians identify potential PB in pediatric patients for early and effective intervention.

Plastic bronchitis associated with respiratory syncytial virus infection: a case report.

BACKGROUND: The etiology of Plastic bronchitis (PB) is unknown. The incidence of pulmonary infection associated with PB has increased year by year, but respiratory syncytial virus (RSV) as a pathogen causes PB has rarely been reported. CASE PRESENTATION: A 2-year-old immunocompromised girl was admitted to the hospital with cough, fever for 5 days, and aggravated with shortness of breath for 1 day. With mechanical ventilation, her respiratory failure was not relieved, and subcutaneous emphysema and mediastinal pneumatosis appeared. Extracorporeal membrane oxygenation (ECMO) was administrated, but the tidal volume was low. Therefore, a bronchoscopy was performed, by which plastic secretions were found and removed. Pathology of the plastic secretions confirmed the diagnosis of type I PB. RSV was the only positive pathogen in the alveolar lavage fluid by the next-generation sequencing test. After the bronchoscopic procedure, her dyspnea improved. The patient was discharged with a high-flow nasal cannula, with a pulse oxygen saturation above 95%. Half a year after discharge, she developed sequelae of bronchitis obliterans. CONCLUSION: RSV could be an etiology of PB, especially in an immunocompromised child. In a patient with pulmonary infection, if hypoxemia is presented and unresponded to mechanical ventilation, even ECMO, PB should be considered, and bronchoscopy should be performed as soon as possible to confirm the diagnosis and to treat.

Non-Contrast MR Lymphography and Intranodal Dynamic Contrast MR Lymphangiography in Children with Congenital Heart Disease-Imaging Findings as well as Impact on Patient Management and Outcome.

Lymphatic flow disorders are rare but devastating complications in children with congenital heart disease. T2-weighted magnetic resonance lymphography and intranodal dynamic contrast magnetic resonance lymphangiography are imaging modalities that can depict central lymphatic anatomy and flow pattern. Our objective was to describe the technical aspects and our imaging findings of central lymphatic abnormalities and their impact on patient management and outcomes: We conducted a retrospective review of 26 children with congenital heart disease who presented for lymphatic imaging between 2015 and 2020 at our institution. Eleven had postoperative chylothorax, six had plastic bronchitis, seven had protein-losing enteropathy and three had Noonan syndrome. Our lymphatic imaging demonstrated severely abnormal lymphatic flow in all of the children, but only minor abnormalities in protein-losing enteropathy. No major procedure-related complication occurred. Lymphatic interventions were performed in six patients, thoracic duct decompression in two patients and chylothorax revision in three patients. This led to symptomatic improvements in all of the patients: Lymphatic imaging is safe and essential for the diagnosis of lymphatic flow disorders and therapy planning. Our intranodal lymphangiography depicts an abnormal lymphatic flow pattern from the central lymphatics but failed to demonstrate an abnormal lymphatic flow in protein-losing enteropathy. These imaging techniques are the basis for selective lymphatic interventions, which are promising to treat lymphatic flow disorders.

[Clinical characteristics of plastic bronchitis and risk factors for recurrence in children]. / å„¿ç«¥å¡‘åž‹æ€§æ”¯æ°”ç®¡ç‚Žä¸´åºŠç‰¹å¾åŠå¤å‘å±é™©å› ç´ åˆ†æž.

OBJECTIVES: To study the clinical characteristics of plastic bronchitis (PB) in children and investigate the the risk factors for recurrence of PB. METHODS: This was a retrospective analysis of medical data of children with PB who were hospitalized in Children's Hospital of Chongqing Medical University from January 2012 to July 2022. The children were divided into a single occurrence of PB group and a recurrent PB group and the risk factors for recurrence of PB were analyzed. RESULTS: A total of 107 children with PB were included, including 61 males (57.0%) and 46 females (43.0%), with a median age of 5.0 years, and 78 cases (72.9%) were over 3 years old. All the children had cough, 96 children (89.7%) had fever, with high fever in 90 children. Seventy-three children (68.2%) had shortness of breath, and 64 children (59.8%) had respiratory failure. Sixty-six children (61.7%) had atelectasis and 52 children (48.6%) had pleural effusion. Forty-seven children (43.9%) had Mycoplasma pneumoniae infection, 28 children (26.2%) had adenovirus infection, and 17 children (15.9%) had influenza virus infection. Seventy-one children (66.4%) had a single occurrence of PB, and 36 cases (33.6%) had recurrent occurrence of PB (≥2 times). Multivariate logistic regression analysis showed that involvement of ≥2 lung lobes (OR=3.376) under bronchoscopy, continued need for invasive ventilation after initial removal of plastic casts (OR=3.275), and concomitant multi-organ dysfunction outside the lungs (OR=2.906) were independent risk factors for recurrent occurrence of PB (P<0.05). CONCLUSIONS: Children with pneumonia accompanied by persistent high fever, shortness of breath, respiratory failure, atelectasis or pleural effusion should be highly suspected with PB. Involvement of ≥2 lung lobes under bronchoscopy, continued need for invasive ventilation after initial removal of plastic casts, and concomitant multi-organ dysfunction outside the lungs may be risk factors for recurrent occurrence of PB.

Clinical characteristics of plastic bronchitis in children: a retrospective analysis of 43 cases.

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 â„ƒ 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.

Histological characteristics of matrix metalloproteinase-9 and tissue inhibitor of metalloproteinases-1 in asthmatic murine model during A(H1N1)pdm09 infection.

Pandemic influenza virus A(H1N1)pdm09 infection occurred in healthy children and young adults, but asthmatic patients presented more rapid progression of respiratory distress and plastic bronchitis. To investigate the pathogenesis of worsening respiratory symptoms after A(H1N1)pdm09 infection, we focused on matrix metalloproteinase-9 (MMP-9) and tissue inhibitor of metalloproteinases-1 (TIMP-1). MMP-9 and TIMP-1 levels in bronchoalveolar lavage fluid and serum from mice with and without asthma were evaluated after A(H1N1)pdm09 or seasonal A(H1N1) infection. MMP-9 levels were more elevated in Asthma/A(H1N1)pdm09-infected mice than in non-Asthma/A(H1N1)pdm09-infected mice on both 3 and 7 days post-infection. Immunohistochemical findings in this pneumonia model showed that MMP-9 and TIMP-1 positive cells were observed in blood vessels and bronchus of lung tissue in severe pathological findings of pneumonia with asthma. Microscopically, shedding cells and secretions were conspicuous in the trachea on days 3 and 7 post-infection, in the A(H1N1)pdm09-infected mice with asthma. Our results suggest that MMP-9 and TIMP-1 expressions are related to severe pneumonia in the A(H1N1)pdm09 infection with asthma, leading to cause epithelial cell shedding.

Development and validation of a nomogram to predict plastic bronchitis in children with refractory Mycoplasma pneumoniae pneumonia.

BACKGROUND: Early identification of plastic bronchitis (PB) is of great importance and may aid in delivering appropriate treatment. This study aimed to develop and validate a nomogram for predicting PB in patients with refractory Mycoplasma pneumoniae pneumonia (RMPP). METHODS: A total of 547 consecutive children with RMPP who underwent fiberoptic bronchoscopy (FOB) intervention from January 2016 to June 2021 were enrolled in this study. Subsequently, 374 RMPP children (PB: 137, without PB: 237) from January 2016 to December 2019 were assigned to the development dataset to construct the nomogram to predict PB and 173 RMPP children from January 2020 to June 2021 were assigned to the validation dataset. The clinical, laboratory and radiological findings were screened using Least Absolute Shrinkage and Selection Operator (LASSO) regression and logistic regression was applied to construct a nomogram. The performance of the nomogram was evaluated by discrimination, calibration and clinical utility. Comparsion of ROC analysis and decision curve analysis (DCA) between nomogram and other models was performed to evaluate the discrimination ability and clinical utility. RESULTS: The development dataset included 374 patients with a mean age of 6.6 years and 185(49.5%) were men. The validation dataset included 173 patients and the mean age of the dataset was 6.7 years and 86 (49.7%) were men. From 26 potential predictors, LASSO regression identified 6 variables as significant predictive factors to construct the nomogram for predicting PB, including peak body temperature, neutrophil ratio (N%), platelet counts (PLT), interleukin-6 (IL-6), actic dehydrogenase (LDH) and pulmonary atelectasis. The nomogram showed good discrimination, calibration and clinical value. The mean AUC of the nomogram was 0.813 (95% CI 0.769-0.856) in the development dataset and 0.895 (95% CI 0.847-0.943) in the validation dataset. Through calibration plot and Hosmer-Lemeshow test, the predicted probability had a good consistency with actual probability both in the development dataset (P = 0.217) and validation dataset (P = 0.183), and DCA showed good clinical utility. ROC analysis indicated that the nomogram showed better discrimination ability compared with model of peak body temperature + pulmonary atelactsis and another model of N% + PLT + IL-6 + LDH, both in development dataset (AUC 0.813 vs 0.757 vs 0.754) and validation dataset (AUC 0.895 vs 0.789 vs 0.842). CONCLUSIONS: In this study, a nomogram for predicting PB among RMPP patients was developed and validated. It performs well on discrimination ability, calibration ability and clinical value and may have the potential for the early identification of PB that will help physicians take timely intervention and appropriate management.

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.

Direct EBUS-guided transtracheal lymphosclerosis for plastic bronchitis after Fontan.

We report on a new puncture technique with direct transtracheal mediastinal lymphatic access to treat plastic bronchitis after Fontan repair. High resolution contrast-enhanced spiral CT identified enlarged lymph nodes in the paratracheal region. Inguinal intranodal Gadolinium Dynamic Contrast-enhanced Magnetic Resonance lymphangiography (DCMRL) confirmed the pathologic centrifugal lymph flow passing through these lymph nodes before leaking into the bronchial tree. The abnormal hypertrophic paratracheal, subcarinal, and hilar lymph nodes were punctured with a 22G needle through an endobronchial ultrasound bronchoscope. Occlusion of the lymph vessels was obtained by injecting a mixture of lipiodol/NBCA N-butyl cyanoacrylate (Histoacryl) 5/1 under fluoroscopic control. There was a total remission of PB with now 10 months of follow-up.

Fontan-associated plastic bronchitis waitlist and heart transplant outcomes: A PHTS analysis.

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.

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.

Pseudomembranous Tracheobronchitis With Severe Tracheal Stenosis and Masked Bronchial Obstruction.

BACKGROUND: Pseudomembranous tracheobronchitis (PMTB) is a rare condition characterized by the formation of endobronchial pseudomembranes. PMTB overlaps with necrotizing tracheobronchitis or plastic bronchitis. The reported infectious etiology mainly includes invasive aspergillosis. PMTB can cause serious airway obstruction; however, urgent tracheotomy is rarely required. CASE REPORT: A 46-year-old woman was transferred to the emergency department (ED) with a 1-week history of progressive dyspnea and cough that was preceded by fever and sore throat. She was previously healthy except for a 20-year history of mild palmoplantar pustulosis. Stridor was evident. Nasolaryngoscopy performed in the ED revealed severe tracheal stenosis caused primarily by mucosal edema and secondarily by pseudomembranes. Initially, tracheitis was considered the sole cause of dyspnea. Although she underwent urgent tracheotomy to prevent asphyxia, her respiration deteriorated progressively. Bronchoscopy revealed massive pseudomembranes obstructing the bilateral bronchi, which led to the clinical diagnosis of PMTB. Subsequent toilet bronchoscopy markedly improved her ventilation. The causative pathogen was not identified despite extensive work-up, including molecular biological testing. Histopathologic examination of the pseudomembranes revealed fibrin with abundant neutrophils, which was consistent with PMTB. Associated conditions, including immunodeficiency, were not found. Her condition improved with antibiotics and repeated toilet bronchoscopy. WHY SHOULD AN EMERGENCY PHYSICIANS BE AWARE OF THIS?: PMTB is an important differential diagnosis of airway emergencies. PMTB can present with critical edematous tracheal stenosis and masked bronchial pseudomembranous obstruction. Emergency physicians should include PMTB in the differential diagnosis in adult patients with acute central airway obstruction because it requires prompt multimodal treatment.

Dynamic contrast-enhanced magnetic resonance lymphangiography in pediatric patients with central lymphatic system disorders.

Central conducting lymphatics (CCLs) disorders represent a broad spectrum of clinical entities ranging from self-limiting traumatic leaks treated by conservative strategies, to complex lymphatic circulation abnormalities that are progressive and unresponsive to currently available treatments. Dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) performed by intranodal injection of gadolinium-based contrast material is a recently developed technique which allows a minimally invasive evaluation of the CCL abnormalities providing a dynamic assessment of lymph flow and its pathways. In our institution, DCMRL is performed after bilateral cannulation of inguinal lymph nodes, using a MR protocol which includes volumetric 3D T2-SPACE (sampling perfection with application-optimized contrasts using different flip-angle evolution) and free-breathing respiratory navigated sequence and TWIST (time-resolved angiography with Interleaved stochastic trajectories) MR angiography sequence, during intranodal injection of paramagnetic contrast medium. Although DCMRL applications in clinical practice are still improving, a minimally invasive assessment of lymphatic pathways is particularly important both in pediatric patients with primitive lymphatic system disorders and in children with complex congenital heart disease associated with CCL impairment.

[Clinical Analysis of 86 Cases of Children with Plastic Bronchitis].

OBJECTIVE: To investigate the clinical features of pediatric patients who had plastic bronchitis (PB) and to explore the risk factors for respiratory support in the pediatric patients with PB in order to improve the ability to identify PB in children. METHODS: The basic information and clinical manifestations of 86 children diagnosed with PB at West China Second University Hospital of Sichuan University from March 2014 to December 2019 were collected and analyzed retrospectively. The patients were divided into the respiratory support (RS) group and non-respiratory support (NRS) group as per their need for respiratory support. Logistic regression was conducted to analyze the risk factors for respiratory support in PB patients. RESULTS: A total of 86 children with PB were included in the study, including 62 (72.1%) who were over 3 years old. 57 patients (66.3%) had complications. 56 patients were given respiratory support after admission. All the 86 children had a history of fever and cough, and 76 (88.4%) experienced fever peaks≥39.5°C. Chest imaging showed large lung consolidation or atelectasis in 82 cases (95.3%) and pleural effusion in 63 cases (73.3%). 70 cases (81.4%) were tested positive for pathogens, with the highest infection rate of 68.6% for mycoplasma pneumoniae. There were 30 patients (34.9%) in the NRS group and 56 patients (65.1%) in the RS group. Logistic regression analysis showed that patient being younger than 3 years old ( OR=4.99) and having complications ( OR=7.22) were independent risk factors for respiratory support in children with PB (all P<0.05). CONCLUSIONS: Clinically, severe clinical symptoms combined with other systemic complications, large lung consolidation or atelectasis, pleural effusion, and positive lab results for mycoplasma pneumoniae should be an alert indicating the possibility of having PB. Young age and complications were independent risk factors for respiratory support in PB patients.

Pediatric pulmonary lymphatic flow Disorders: Diagnosis and management.

Pulmonary lymphatic flow disorders involve the abnormal lymphatic flow via lymphatic channels to the lungs and pleural space. Plastic bronchitis and chylothorax are the main complications of this abnormal lymphatic perfusion, which has been termed pulmonary lymphatic perfusion syndrome (PLPS). Following lymphatic access, dynamic contrast MR lymphangiography is the imaging modality of choice to diagnose these disorders. Management includes medical therapy, percutaneous interventions under fluoroscopy, and surgical interventions.

Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment.

Pulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the thoracic cavity, presenting clinically as chylothorax, chylopericardium, chyloptysis, interstitial lung disease and plastic bronchitis. These conditions include: neonatal chylothorax, cardiac and non-cardiac plastic bronchitis, non-traumatic chylothorax, post congenital cardiac surgery chylothorax and complex lymphatic malformations. Recently developed lymphatic imaging techniques, such as intranodal lymphangiography and dynamic contrast enhanced magnetic resonance lymphangiography demonstrated abnormal pulmonary lymphatic flow from thoracic duct into pulmonary parenchyma as a pathophysiological mechanism of these diseases. Novel minimally invasive lymphatic interventions, such as thoracic duct embolization, interstitial lymphatic embolization and surgical lympho-venous anastomosis, provide an effective treatment of these conditions.

Innominate Vein Turn-down Procedure for Failing Fontan Circulation.

After the Fontan, systemic venous hypertension induces pathophysiologic changes in the lymphatic system that can result in complications of pleural effusion, ascites, plastic bronchitis, and protein losing enteropathy. Advances in medical therapy and novel interventional approaches have not substantially improved the poor prognosis of these complications. A more physiological approach has been developed by decompression of the thoracic duct to the lower pressure common atrium with a concomitant increase of preload. Diverting the innominate vein to the common atrium increases the transport capacity of the thoracic duct, which in most patients enters the circulation at the left subclavian-jugular vein junction. Contrary to the fenestrated Fontan circulation, in which the thoracic duct is drained into the high pressure Fontan circulation, turn down of the innominate vein to the common atrium effectively decompresses the thoracic duct to the lower pressure system with "diastolic suctioning" of lymph. Innominate vein turn-down may be considered for medical-refractory post-Fontan lymphatic complications of persistent chylothorax, plastic bronchitis, and protein losing enteropathy. Prophylactic innominate vein turn-down may also be considered at time of the Fontan operation for patients that are higher risk for lymphatic complications.

Lymphatic Management in Single-Ventricle Patients.

Lymphatic complications in patients with single ventricle include plastic bronchitis, protein-losing enteropathy, and chylous pleural effusion are a source of significant morbidity and mortality with historically limited therapeutic options. Novel lymphatic imaging techniques such as intranodal lymphangiography, dynamic contrast enhanced magnetic resonance lymphangiography and liver lymphangiography have allowed visualization of the lymphatic system and discovery of the pathophysiological mechanism of these conditions. This mechanism includes the combination of 2 factors: increased lymphatic flow in patients with elevated central venous pressure and presence of the lymphatic anatomical variant that allows the lymph to flow in close proximity to the serous (pleural space in chylothorax) or mucosal (plastic bronchitis and protein losing enteropathy) surfaces. Novel minimally invasive lymphatic interventional techniques, such as thoracic duct embolization, interstitial embolization and liver lymphatic embolization have allowed the obliteration of these abnormal lymphatic networks, resulting in resolution of the symptoms. Further refinement of the imaging techniques and interventional methods have subsequently allowed better patient selection and improved long term outcome of these procedures.