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BMC Pulmonary Medicine Apr 2024Anti-synthetase syndrome (ASS) is a group of rare clinical subtypes within inflammatory myopathies, predominantly affecting adult females. Instances of critical illness... (Review)
Review
BACKGROUND
Anti-synthetase syndrome (ASS) is a group of rare clinical subtypes within inflammatory myopathies, predominantly affecting adult females. Instances of critical illness associated with ASS in children are even rarer.
CASE PRESENTATION
We report the case of a 7-year-old boy finally diagnosed with ASS, combined with pneumomediastinum. He presented with intermittent fever persisting for 12 days, paroxysmal cough for 11 days, chest pain, and shortness of breath for 4 days, prompting admission to our hospital. Pre-admission chest CT revealed diffuse pneumomediastinum, subcutaneous pneumatosis in the neck and bilateral chest wall, consolidation, atelectasis, and reticular nodular shadowing in both lungs, as well as pericardial effusion and bilateral pleural effusions. Laboratory tests revealed a positive result for serum MP immunoglobulin M (MP-IgM) and MP immunoglobulin G (MP-IgG). The patient was initially diagnosed with mycoplasma pneumoniae (MP) infection, and following 3 days of antibiotic treatment, the patient's tachypnea worsened. Positive results in muscle enzyme antibody tests included anti-PL-12 antibody IgG, anti-Jo-1 antibody IgG, and anti-RO-52 antibody IgG. Ultrasonography detected moderate effusions in the right shoulder, bilateral elbow, and knee joints. Corticosteroids pulse therapy was initiated on the 27th day following disease onset, and continued for 3 days, followed by sequential therapy for an additional 12 days. The child was discharged on the 43rd day, and subsequent follow-up revealed a significant improvement in consolidation and interstitial lesions in both lungs.
CONCLUSIONS
ASS in children may combine with rapidly progressive interstitial lung disease (RPILD) and pneumomediastinum. It is crucial to promptly identify concurrent immunologic abnormalities during the outbreak of MP, particularly when the disease exhibits rapid progression with ineffective conventional antibiotic therapy.
Topics: Child; Humans; Male; Anti-Bacterial Agents; Immunoglobulin G; Lung; Mediastinal Emphysema; Tomography, X-Ray Computed
PubMed: 38561741
DOI: 10.1186/s12890-024-02984-0 -
Journal of Pediatric Surgery May 2024Given the paucity of data on pediatric spontaneous pneumomediastinum (SPM), management is largely informed by extrapolation from adult studies or personal clinical...
PURPOSE
Given the paucity of data on pediatric spontaneous pneumomediastinum (SPM), management is largely informed by extrapolation from adult studies or personal clinical experience, resulting in significant heterogeneity. The purpose of this study was to describe how pediatric patients with SPM are clinically managed at our institution and propose a treatment algorithm.
METHODS
Retrospective chart review of pediatric patients with SPM from April 2002 to December 2021 at a single Canadian tertiary care center. Data on medical history, presentation characteristics, clinical management, and complications were gathered. Descriptive and inferential statistics were used to analyze data.
RESULTS
We identified 63 patients for inclusion, median age was 15 years. Twenty-nine patients were transferred from another facility. Most common presenting symptoms were chest pain (72.3%), shortness of breath (44.6%), and subcutaneous emphysema (21.5%). Initial workup included chest x-ray (93.6%), CT scan (20.6%), and upper GI series (7.9%). There was no difference in the number of initial tests between admitted and discharged patients (p = 0.10). Of admitted patients (n = 35), 31.4% had primary SPM (no underlying comorbidity/inciting event) and 68.8% were secondary SPM (underlying comorbidity/inciting event). No patients with primary SPM developed complications or recurrences. In contrast, 16.7% of those with secondary SPM developed complications and 54.2% had at least one additional intervention after admission.
DISCUSSION
There is significant variability in diagnostic investigation and treatment of pediatric SPM at our center. Amongst primary SPM, additional tests did not change complication rate or recurrence, including those transferred from another facility. An expeditious treatment algorithm is warranted.
TYPE OF STUDY
Retrospective Cohort Study.
LEVEL OF EVIDENCE
III.
Topics: Adult; Humans; Child; Adolescent; Retrospective Studies; Mediastinal Emphysema; Canada; Tomography, X-Ray Computed; Radiography
PubMed: 38519387
DOI: 10.1016/j.jpedsurg.2024.01.043 -
Scientific Reports Feb 2024Spontaneous pneumomediastinum (SPM) is a rare condition in children and young adults that raises concern for esophageal perforation or extension of an air leak,...
Spontaneous pneumomediastinum (SPM) is a rare condition in children and young adults that raises concern for esophageal perforation or extension of an air leak, resulting in admissions with multiple interventions performed. To assess our outcomes, and to evaluate our resource utilization, we reviewed our experience with SPM. We conducted a retrospective review of SPM cases in patients aged 5-25 years old occurring between 2011 and 2021 at a single academic tertiary care center. Clinical, demographic, and outcome variables were collected and analyzed, and cohorts were compared using Fischer's Exact Test and Welch's T Test. 166 SPM cases were identified-all of which were Emergency Department (ED) presentations. 84% of the cases were admitted. 70% had Computerized Tomography (CT) scans, with no defined criteria for imaging. Comparison of floor admissions with discharges from the ED showed no significant difference in presenting symptoms, demographics, or outcomes between the two groups. Recurrence was noted in 4 patients with a range of 5.9 months-4.9 years from the initial episode. In the largest SPM study in the pediatric and young adult population, we noted no significant difference in management or outcomes in admitted or ED discharge patients nor those with CT imaging. Our results suggest that a large number of SPM can be managed safely with discharge from the ED.
Topics: Adolescent; Adult; Child; Child, Preschool; Humans; Young Adult; Hospitalization; Mediastinal Emphysema; Patient Discharge; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 38402248
DOI: 10.1038/s41598-024-55134-1 -
Pediatric Pulmonology May 2024
Topics: Child; Female; Humans; Male; COVID-19; Mediastinal Emphysema; SARS-CoV-2; Tomography, X-Ray Computed; Infant; Adolescent
PubMed: 38390764
DOI: 10.1002/ppul.26931 -
Journal of Cardiothoracic and Vascular... Apr 2024Air leak syndromes (such as pneumomediastinum, pneumothorax, or subcutaneous emphysema) are frequent complications of acute respiratory distress syndrome (ARDS)....
Air leak syndromes (such as pneumomediastinum, pneumothorax, or subcutaneous emphysema) are frequent complications of acute respiratory distress syndrome (ARDS). Unfortunately, the development of air leaks is associated with worse outcomes. In addition, it has been hypothesized that the development of pneumomediastinum could be a marker of disease severity in patients with respiratory failure receiving noninvasive respiratory support or assisted ventilation. The so-called Macklin effect (or pulmonary interstitial emphysema) is the air dissection of the lung bronchovascular tree from peripheral to central airways following injury to distal alveoli. Ultimately, the progression of the Macklin effect leads to the development of pneumomediastinum, subcutaneous emphysema, or pneumothorax. The Macklin effect is identifiable on a chest computed tomography (CT) scan. The Macklin effect could be an accurate predictor of barotrauma in patients with ARDS (sensitivity = 89.2% [95% CI: 74.6-96.9]; specificity = 95.6% [95% CI: 90.6-98.4]), and may be a marker of disease severity. Accordingly, the detection of the Macklin effect on a chest CT scan could be used to select which patients with ARDS might benefit from different treatment algorithms, including advanced respiratory monitoring, early intubation, or, potentially, the institution of early extracorporeal support with or without invasive ventilation. In this video, the authors summarize the pathophysiology and potential clinical significance and applications of the Macklin effect in patients with acute respiratory failure.
Topics: Humans; Pneumothorax; Mediastinal Emphysema; Lung; Respiratory Distress Syndrome; Subcutaneous Emphysema
PubMed: 38378321
DOI: 10.1053/j.jvca.2023.12.025 -
La Revue Du Praticien Oct 2023
Topics: Humans; Mediastinal Emphysema
PubMed: 38354011
DOI: No ID Found -
World Journal of Clinical Cases Feb 2024Mediastinal emphysema is a condition in which air enters the mediastinum between the connective tissue spaces within the pleura for a variety of reasons. It can be...
BACKGROUND
Mediastinal emphysema is a condition in which air enters the mediastinum between the connective tissue spaces within the pleura for a variety of reasons. It can be spontaneous or secondary to chest trauma, esophageal perforation, medically induced factors, Its common symptoms are chest pain, tightness in the chest, and respiratory distress. Most mediastinal emphysema patients have mild symptoms, but severe mediastinal emphysema can cause respiratory and circulatory failure, resulting in serious consequences.
CASE SUMMARY
A 75-year-old man, living alone, presented with sudden onset of severe epigastric pain with chest tightness after drinking alcohol. Due to the remoteness of his residence and lack of neighbors, the patient was found by his nephew and brought to the hospital the next morning after the disease onset. Computed tomography (CT) showed free gas in the abdominal cavity, mediastinal emphysema, and subcutaneous pneumothorax. Upper gastrointestinal angiography showed that the esophageal mucosa was intact and the gastric antrum was perforated. Therefore, we chose to perform open gastric perforation repair on the patient under thoracic epidural anesthesia combined with intravenous anesthesia. An operative incision of the muscle layer of the patient's abdominal wall was made, and a large amount of subperitoneal gas was revealed. And a continued incision of the peritoneum revealed the presence of a perforation of approximately 0.5 cm in the gastric antrum, which we repaired after pathological examination. Postoperatively, the patient received high-flow oxygen and cough exercises. Chest CT was performed on the first and sixth postoperative days, and the mediastinal and subcutaneous gas was gradually reduced.
CONCLUSION
After gastric perforation, a large amount of free gas in the abdominal cavity can reach the mediastinum through the loose connective tissue at the esophageal hiatus of the diaphragm, and upper gastrointestinal angiography can clarify the site of perforation. In patients with mediastinal emphysema, open surgery avoids the elevation of the diaphragm caused by pneumoperitoneum compared to laparoscopic surgery and avoids increasing the mediastinal pressure. In addition, thoracic epidural anesthesia combined with intravenous anesthesia also avoids pressure on the mediastinum from mechanical ventilation.
PubMed: 38322697
DOI: 10.12998/wjcc.v12.i4.859 -
Mediastinum (Hong Kong, China) 2024Descending necrotizing mediastinitis (DNM) is an acute life-threatening infection that originates in the oropharyngeal region. It is an uncommon disease with a mortality...
BACKGROUND
Descending necrotizing mediastinitis (DNM) is an acute life-threatening infection that originates in the oropharyngeal region. It is an uncommon disease with a mortality rate of about 20-40%. This high mortality is mainly attributed to delays in diagnosis and treatment and poor drainage of the mediastinum. We highlight key points that may help reduce mortality.
CASE DESCRIPTION
We analyze a retrospective case series of seven patients diagnosed with DNM between March 2019 and July 2022 at Hospital de la Santa Creu i Sant Pau. The primary oropharyngeal infection was peritonsillar abscess in three cases and odontogenic abscess in four. All patients showed symptoms of severe cervical infection and symptoms suggestive of mediastinitis. A cervicothoracic computed tomography (CT) scan confirmed the presence of cervical and mediastinal collections and emphysema in all cases. All patients were simultaneously evaluated by the otorhinolaryngology and thoracic surgery teams. Broad-spectrum antibiotic therapy was instituted pending culture. All the patients underwent urgent surgery, consisting of cervicotomy to control the cervical focus and unilateral or bilateral video-assisted thoracoscopic debridement and drain of the pleural cavities and mediastinum. Regarding the outcomes, no patients died, one patient (14.2%) underwent transcervical mediastino-thoracoscopy drainage only. In six patients (85.8%) we performed a combined transcervical and transthoracic approach. Reoperation was required in 3 (43%) cases. The parameter that indicated a poor clinical evolution in these patients was an increase in C-reactive protein and the infection extension on the cervicothoracic CT scan. The follow-up was 30 days from last surgery; there were no losses.
CONCLUSIONS
Based on our experience, the key points that can help reduce the high mortality associated with DNM are a rapid multidisciplinary assessment and a combined surgical procedure, considering the minimally invasive approach as the first option to drain the pleural cavities and mediastinum.
PubMed: 38322187
DOI: 10.21037/med-23-32 -
Ulusal Travma Ve Acil Cerrahi Dergisi =... Feb 2024Pneumomediastinum signifies the accumulation of air within the mediastinum. This condition can develop sponta-neously or as a secondary condition due to trauma or... (Observational Study)
Observational Study
BACKGROUND
Pneumomediastinum signifies the accumulation of air within the mediastinum. This condition can develop sponta-neously or as a secondary condition due to trauma or iatrogenic causes. Although rare, it is part of a wide differential diagnosis scale due to its most common presenting symptoms: chest pain and shortness of breath.
METHODS
Our study is a retrospective, observational, and cohort investigation. It included patients who presented to the emer-gency department and were diagnosed with pneumomediastinum through computed tomography. The study evaluated patients' so-ciodemographic features, methods of presentation, chest tube insertion, other surgical procedures, outcomes, and patient dispositions. The primary outcome of the study focused on the results of traumatic and spontaneous pneumomediastinum: hospital admission, the necessity for thoracostomy tube insertion, requirement for surgical procedures, and mortality. The secondary aim was to determine the relationship between other clinical features and laboratory parameters and their impact on the outcomes.
RESULTS
The study comprised 67 cases. The average age of the cases was 44.89±2.41 years. Of the cases, 67.2% (n=45) were male. In terms of development, 40.3% (n=27) of cases were classified as spontaneous, and 59.7% (n=40) were post-trauma pneumomediasti-num diagnoses. Among symptoms, 50.7% (n=34) of patients experienced dyspnea, and 49.3% (n=33) presented with chest pain, while symptoms like cough, fever, nausea, vomiting, and swallowing difficulty were reported in varying proportions. Among the patients, 9.0% (n=6) had lung disease, 29.9% (n=20) had comorbidities, 3.0% (n=2) had a history of substance use, 14.9% (n=10) underwent thoracostomy tube insertion, and 20.9% (n=14) required surgical procedures. While 35.8% (n=24) of the patients were admitted to the intensive care unit, 13.4% (n=9) died. The mean total hospital stay was calculated as 8.68±1.12 days. No statistically significant relationship was found between the development of pneumomediastinum and hospital admission (p=0.507).
CONCLUSION
Upon examining the causes of pneumomediastinum cases, it was observed that patients with a history of trauma required thoracostomy tube insertion and surgical intervention more frequently. However, when classified as spontaneous or trau-matic, both groups exhibited similar clinical courses and outcomes. Both groups demonstrated favorable clinical outcomes.
Topics: Humans; Male; Adult; Middle Aged; Female; Mediastinal Emphysema; Retrospective Studies; Emergency Room Visits; Chest Pain; Dyspnea
PubMed: 38305659
DOI: 10.14744/tjtes.2024.66059 -
ANZ Journal of Surgery May 2024Tension pneumomediastinum is uncommon but it is a rapidly progress condition that can lead to cardiogenic shock. Mediastinal decompression is an emergency procedure and...
Tension pneumomediastinum is uncommon but it is a rapidly progress condition that can lead to cardiogenic shock. Mediastinal decompression is an emergency procedure and the knowledge of this technique is a life-saving treatment.
Topics: Humans; Mediastinal Emphysema; Decompression, Surgical; Mediastinum; Male; Shock, Cardiogenic; Point-of-Care Systems; Tomography, X-Ray Computed
PubMed: 38305066
DOI: 10.1111/ans.18881