Anti-synthetase syndrome in a child with pneumomediastinum: a case report and literature 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.

A 25-Year-Old Man with a History of Substance Abuse Presenting with Pneumomediastinum Due to Methamphetamine Vapor Inhalation.

BACKGROUND There has been an increase in the use of inhalation methods to abuse drugs, including freebasing crack cocaine (alkaloid) and inhaling methamphetamine vapor. This report is of a 25-year-old man with a history of substance abuse presenting with pneumomediastinum due to methamphetamine vapor inhalation. Acute pneumomediastinum is an extremely rare complication of methamphetamine use. CASE REPORT A 25-year-old man was treated for polysubstance abuse following 9 days of methamphetamine abuse. EKG did not show any ST &T change. D-dimer was normal, at 0.4 mg/L, so we did not do further work-up for pulmonary embolism. His chest pain worsened in the Emergency Department (ED), and a physical exam demonstrated crepitation of the posterior neck, trapezius, and right scapula. A portable chest X-ray revealed subcutaneous air over the right scapular region, in addition to pneumomediastinum. The urine drug screen test was positive for methamphetamine. A chest CT was ordered, which showed a moderate-volume pneumomediastinum with soft-tissue air tracking into the lower neck and along the right chest wall. The patient underwent an esophagogram, which showed no air leak, and Boerhaave's syndrome was ruled out. His symptoms improved and he did not require any surgical intervention. CONCLUSIONS Considering the higher rates of illicit substance use, especially methamphetamine, it is important to pay attention to the associated pathologies and to keep spontaneous pneumomediastinum on the list of differentials for patients using methamphetamine, particularly those who inhale it, which can cause pneumomediastinum, even without Boerhaave's syndrome.

Pneumoperitoneum, pneumoretroperitoneum and pneumomediastinum: rare complications of perforation peritonitis: a case report.

BACKGROUND: Gas extravasation complications arising from perforated diverticulitis are common but manifestations such as pneumoperitoneum, pneumoretroperitoneum, and pneumomediastinum happening at the same time are exceedingly rare. This case report explores the unique presentation of these 3 complications occurring simultaneously, their diagnosis and their management, emphasizing the importance of interdisciplinary collaboration for accurate diagnosis and effective management. CASE PRESENTATION: A 74-year-old North African female, with a medical history including hypertension, dyslipidemia, type 2 diabetes, goiter, prior cholecystectomy, and bilateral total knee replacement, presented with sudden-onset pelvic pain, chronic constipation, and rectal bleeding. Clinical examination revealed hemodynamic instability, hypoxemia, and diffuse tenderness. After appropriate fluid resuscitation with norepinephrine and saline serum, the patient was stable enough to undergo computed tomography scan. Emergency computed tomography scan confirmed perforated diverticulitis at the rectosigmoid junction, accompanied by the unprecedented presence of pneumoperitoneum, pneumoretroperitoneum, and pneumomediastinum. The patient underwent prompt surgical intervention with colo-rectal resection and a Hartmann colostomy. The postoperative course was favorable, leading to discharge one week after admission. CONCLUSIONS: This case report highlights the clinical novelty of gas extravasation complications in perforated diverticulitis. The unique triad of pneumoperitoneum, pneumoretroperitoneum, and pneumomediastinum in a 74-year-old female underscores the diagnostic challenges and the importance of advanced imaging techniques. The successful collaboration between radiologists and surgeons facilitated a timely and accurate diagnosis, enabling a minimally invasive surgical approach. This case contributes to the understanding of atypical presentations of diverticulitis and emphasizes the significance of interdisciplinary teamwork in managing such rare manifestations.

Tracheal Schwannoma Presenting as Subcutaneous Emphysema and Pneumomediastinum.

Primary tracheal schwannomas are rare benign tumours. This is a case report, and therefore, no specific methods or results are applicable. We here report a case of a tracheal schwannoma in an early adolescent girl presenting with subcutaneous emphysema and symptoms of airway obstruction. Tracheal resection and reconstruction by primary anastomosis were performed. Pathology confirmed the diagnosis of tracheal schwannoma. This is an unusual life-threatening presentation of a benign rare tracheal tumour with a challenging approach to management.

[Thyroid cartilage fracture following sneezing as a cause of spontaneous pneumomediastinum]. / Perelom shchitovidnogo khryashcha vsledstvie chikhan'ya kak prichina spontannogo pnevmomediastinuma.

OBJECTIVE: To evaluate the possible etiological factors of spontaneous pneumomediastinum and to describe a case that was unusual in its etiology: a thyroid cartilage fracture as a result of sneezing. MATERIAL AND METHODS: Six patients (four male, two female, aged 16-82 years) were hospitalized with spontaneous pneumomediastinum diagnosed with a chest X-ray in five patients and 100% with computed tomography. Treatment was symptomatic. RESULTS: The commonest symptoms (cough, shortness of breath, hoarseness) were in four patients. Spontaneous pneumomediastinum developed in three cases as a result of bronchospasm during an attack of bronchial asthma, in one patient after exercise, in one after fibrogastroscopy, in one after sneezing. We report a 30-year-old man who presenting subcutaneous emphysema on the neck, hoarseness, pain when swallowing, hemoptysis developed after sneezing. His computed tomography revealed a pneumomediastinum due to fistula of the fracture of the thyroid cartilage following sneezing while simultaneously obstructing both nostrils. At laryngoscopy, there was a linear hematoma in the resolution stage on the anterior wall of the larynx. He was treated conservatively and recovered rapidly. There are no previous published reports of spontaneous pneumomediastinum following fracture of the thyroid cartilage. CONCLUSION: Fracture of the thyroid cartilage as a result of a sharp rapid increase in airway pressure during a sneeze with blocked nasal passages can be one of the rare causes of spontaneous pneumomediastinum. Avoid closing both nostrils at the same time when sneezing.

Pneumothorax, pneumomediastinum and subcutaneous emphysema as respiratory complications of COVID-19.

BACKGROUND: Pneumothorax, pneumomediastinum and subcutaneous emphysema are respiratory complications of Coronavirus disease 2019 occurring with noteworthy frequency in patients especially with severe disease. They can be life-threatening and often complicate patient managment. METHODS: This was a retrospective, observational study of patients admitted in Nepal Armed Police Force hospital from 13/05/2020 to 28/12/2021 diagnosed with pneumothorax, pneumomediastinum or subcutaneous emphysema singly or in combination. Data were collected from clinical charts, imaging records and electronic medical records of Severe Acute Respiratory Syndrome Coronavirus-type 2 positive patients 18 years and older. The frequency and type of the defined complications, the inflammatory markers and ventilatory parameters just prior to their diagnosis, the duration of hospitalization and ICU admission and in-hospital mortality rate were studied. RESULTS: Out of 4013 COVID-19 patients admitted in the hospital during the period, a total of 28 patients were observed to develop the complications, the overall incidence being 0.7% among hospitalized patients and 5.6% among ICU patients. The proportion of subcutaneous emphysema (64.3%) was highest followed by pneumomediastinum (46.4%) and then pneumothorax (39.3%) existing singly or in combination among the 28 patients, where four patients developed the complications spontaneously. Mean Positive End Expiratory Pressure of 12.1±2.6 cmH2O and Peak Inspiratory Pressure or Pressure Support of 30.9±10.3 cmH2O were observed for patients under positive pressure ventilation. Most of the patients who developed the complications (78.6%) died during treatment. CONCLUSIONS: Pulmonary air leak complications occur frequently in COVID-19 patients treated with or without positive pressure ventilation signifying increased disease severity, risk of ICU admission and high mortality rate. Hence, clinicians should be vigilant of these complications in all patients affected with COVID-19 and institute timely management.

Spontaneous pneumomediastinum: An extra muscular manifestation of anti-MDA5 dermatomyositis. Report of 2 cases.

The use of specific antibodies in inflammatory myopathies has improved the characterization of this disease, identifying different clinical phenotypes. Patients with dermatomyositis (DM) and anti-MDA5 antibodies display typical skin symptoms, lesser muscular involvement, and a prevalence of interstitial lung disease (ILD) of up to 91%. Beyond ILD, spontaneous pneumomediastinum (SN) has been identified as a rare but potentially fatal pulmonary manifestation. Two cases of this complication in patients with anti-MDA5 DM are reported.

Neumomediastino espontáneo: una manifestación extramuscular de la dermatomiositis anti-MDA5. Reporte de 2 casos / Spontaneous pneumomediastinum: An extra muscular manifestation of anti-MDA5 dermatomyositis. Report of 2 cases

El uso de anticuerpos específicos en miopatías inflamatorias ha mejorado la caracterización de esta enfermedad identificando distintos fenotipos clínicos. Los pacientes con dermatomiositis y anticuerpos anti-MDA5 muestran síntomas típicos en la piel, un menor compromiso muscular y una prevalencia de enfermedad pulmonar intersticial de hasta el 91%. Además de la enfermedad pulmonar intersticial, se ha identificado el neumomediastino espontáneo como una manifestación pulmonar rara pero potencialmente mortal. Se reportan 2 casos de esta manifestación en pacientes con dermatomiositis anti-MDA5.(AU)

The use of specific antibodies in inflammatory myopathies has improved the characterization of this disease, identifying different clinical phenotypes. Patients with dermatomyositis and anti-MDA5 antibodies display typical skin symptoms, lesser muscular involvement, and a prevalence of interstitial lung disease of up to 91%. Beyond interstitial lung disease, spontaneous pneumomediastinum has been identified as a rare but potentially fatal pulmonary manifestation. Two cases of this complication in patients with anti-MDA5 dermatomyositis are reported.(AU)

Management of Pediatric Patients With Spontaneous Pneumomediastinum: A Retrospective Chart Review.

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.

Evaluating emergency department visits for spontaneous and traumatic pneumomediastinum: a retrospective analysis.

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.

Macklin Effect: From Pathophysiology to Clinical Implication.

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.

The unnecessary workups and admissions of adolescents and young adults with spontaneous pneumomediastinum.

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.

Spontaneous pneumomediastinum in anti-MDA5-positive dermatomyositis: Prevalence, risk factors, and prognosis.

OBJECTIVE: To depict the clinical panorama of spontaneous pneumomediastinum (SPM) in anti-MDA5 antibody-positive dermatomyositis (anti-MDA5+ DM). METHODS: A total of 1352 patients with idiopathic inflammatory myopathy (IIM), including 384 anti-MDA5+ DM patients were retrospectively enrolled. The clinical profiles of anti-MDA5+ DM-associated SPM were analyzed. RESULTS: We identified that 9.4 % (36/384) of anti-MDA5+ DM patients were complicated with SPM, which was significantly higher than that of non-anti-MDA5+ DM and other IIM subtypes (P all <0.001). SPM developed at a median of 5.5 (3.0, 12.0) months after anti-MDA5+ DM onset. Anti-MDA5+ DM patients complicated with SPM showed a significantly higher frequency of fever, dyspnea, and pulmonary infection including viral and fungal infections compared to those without SPM (P all < 0.05). Cytomegalovirus (CMV) and fungal infections were identified to be independent risk factors for SPM development in the anti-MDA5+ DM. SPM and non-SPM patients in our anti-MDA5+ DM cohort showed comparable short-term and long-term survival (P = 0.236). Furthermore, in the SPM group, we found that the non-survivors had a lower peripheral lymphocyte count, higher LDH level, and higher frequency of intensification of immunosuppressive treatment (IST) than survivors. The elevated LDH level and intensification of IST were independent risk factors for increased mortality in anti-MDA5+ DM-associated SPM patients. CONCLUSIONS: Nearly one-tenth of patients with anti-MDA5+ DM develop SPM. Both CMV and fungal infections are risk factors for SPM occurrence. The development of SPM does not worsen the prognosis of anti-MDA5+ DM patients, and the intensification of IST does harm to the SPM prognosis.

Pneumopericardium following severe thoracic trauma.

BACKGROUND: Traumatic pneumopericardium (PPC) is a rare clinical entity associated with chest trauma, resulting from a pleuropericardial connection in the presence of a pneumothorax, interstitial air tracking along the pulmonary perivascular sheaths from ruptured alveoli to the pericardium, or direct trachea-bronchial-pericardial communication.  Our objectives were to describe the modern management approach to PPC and to identify variables that could improve survival with severe thoracic injury. METHODS: We conducted a retrospective study of the trauma registry between 2015 and 2022 at a Level I verified adult trauma center for all patients with PPC. Demographics, injury patterns, and treatment characteristics were compared between blunt and penetrating trauma. This study focused on the management strategies and the physiologic status regarding PPC and the development of tension physiology. The main outcome measure was operative versus nonoperative management. RESULTS: Over a seven-year period, there were 46,389 trauma admissions, of which 488 patients had pneumomediastinum. Eighteen patients were identified with PPC at admission. Median age was 39.5 years (range, 18-77 years), predominantly male (n = 16, 89 %), Black (n = 12, 67 %), and the majority from blunt trauma (78 %). Half had subcutaneous emphysema on presentation while 39 % had recognizable pneumomediastinum on chest x-ray. Tube thoracostomy was the most common intervention in this cohort (89 %). Despite tube thoracostomy, tension PPC was observed in three patients, two mandating emergent pericardial windows for progression to tension physiology, and the remaining requiring reconstruction of a blunt tracheal disruption. The majority of PPC patients recovered with expectant management (83 %), and no deaths were directly related to PPC. CONCLUSIONS: Traumatic PPC is a rare radiographic finding with the majority successfully managed conservatively in a monitored ICU setting. These patients often have severe thoracic injury with concomitant injuries requiring thoracostomy alone; however, emergent surgical intervention may be required when PPC progresses to tension physiology to improve overall survival.

Spontaneous pneumomediastinum associated with subcutaneous emphysema, pneumopericardium and pneumothorax: a rare association complicating measles.

A unique case of severe measles complicated by multiple features of gas accumulation is described, on the ground of the available literature evidences. Complications from measles have been reported in every organ system and they may vary by age and underlying conditions. Pneumomediastinum is usually associated with subcutaneous emphysema and pneumopericardium, but rarely associated with pneumothorax. We report extremely rare simultaneous occurrence of self-limiting pneumomediastinum, pneumopericardium, subcutaneous neck and chest region emphysema, and pneumothorax, in a 19-year-old girl with measles. A review of the literature has documented only one previous report of spontaneous pneumomediastinum, subcutaneous emphysema and pneumothorax in the course of measles, and no previous cases reported the association of pneumomediastinum, subcutaneous emphysema, pneumopericardium and pneumothorax complicating measles.

Clinical characteristics of pneumothorax and pneumomediastinum in mechanical ventilated patients with coronavirus disease 2019: a case series.

BACKGROUND: Pneumothorax (PTX) and pneumomediastinum (PM) have been reported as potential complications in patients with coronavirus disease 2019 (COVID-19); however, their risk factors and etiology remain unknown. Herein, we investigated the clinical characteristics of mechanically ventilated patients with COVID-19 with PTX or PM. METHODS: We examined patients with severe COVID-19 requiring mechanical ventilation who were admitted to the intensive care unit of a tertiary-level emergency medical center in Tokyo, Japan between April 1, 2020. and October 31, 2021. We collected and analyzed the clinical characteristics of the patients who presented with either PTX or PM during mechanical ventilation. RESULTS: During the study period, a total of 165 patients required mechanical ventilation, and 15 patients with PTX/PM during mechanical ventilation were selected. Three patients with obvious causes were excluded, and the remaining 12 patients were analyzed (7.3%). The mortality rate in these patients was as high as 50%, demonstrating the difficulty of treatment in the presence of PTX/PM. PTX/PM occurred 14.5 days after intubation. A peak pressure of > 30 cmH2O was only apparent in one patient, suggesting that high positive pressure ventilation may be less involved than mentioned in the literature. In addition, the inspiratory effort was not strong in our group of patients. (P0.1 was 2.1 cm H2O [1.0-3.8]). CONCLUSION: Various factors are associated with the development of PTX/PM in patients on mechanical ventilation for COVID-19. We did not find a strong correlation between PTM/PM and barotrauma or strong inspiratory efforts, which have been identified as potential causes in previous studies.