Pneumomediastinum and subcutaneous emphysema secondary to amyopathic dermatomyositis with cryptogenic organizing pneumonia in invasive breast cancer: a case report and review of literature

Amyopathic dermatomyositis (ADM) is recognized as a variant phenotype of dermatomyositis and characterized by typical skin manifestations without evidence of muscular inflammation. While interstitial lung disease (ILD) is occasionally found as one of the lung manifestations in ADM patients, the development of a pneumomediastinum and/or subcutaneous emphysema in this disease entity is one of the extremely rare pulmonary complications. These latter complicated pulmonary manifestations have been usually reported in idiopathic ADM with ILD without any other associated medical conditions. We report a case presented with the spontaneous pneumomediastinum and subcutaneous emphysema in both ADM and cryptogenic organizing pneumonia during adjuvant chemotherapy based on cyclophosphamide for breast cancer.     

Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature

We describe a 42-year-old man with dermatomyositis and interstitial lung disease who presented with anterior neck pain and dyspnoea. Chest radiographs showed subcutaneous emphysema, pneumomediastinum and diffuse reticulonodular infiltration in both lungs. After the administration of high doses of prednisolone, an improvement of pulmonary function and respiratory symptoms was observed but the pneumomediastinum persists 12 months after diagnosis, and without any complication. We review the cases that have been reported thus far of pneumomediastinum associated with dermatomyositis and discuss the possible mechanisms involved. We conclude that pneumomediastinum is not an uncommon complication of dermatomyositis and that its aetiopathogenesis remains very unclear. Received: 26 July 2000 / Accepted: 14 March 2001     

Spontaneous cervicothoracolumbar pneumorrhachis,pneumomediastinum and pneumoperitoneum

Introduction: Pneumorrhachis, or epidural pneumatosis, is a rare entity that is usually traumatic or iatrogenic. Usually, the epidural emphysema is limited to a few vertebral spaces. Less commonly, it is secondary to mediastinal air that tracks into the epidural space. Mediastinal air is usually associated with subcutaneous emphysema, but rarely is it associated with pneumopericardium or pneumoperitoneum. The cause of pneumomediastinum is usually identifiable on history or radiology. Methods: Report of a case. Results: We report the extremely rare simultaneous occurrence of self‐limiting cervicothoracolumbar pneumorrhachis, pneumomediastinum, cervical subcutaneous emphysema and pneumoperitoneum in a healthy 20‐year‐old gentleman in the absence of an identifiable cause. Conclusion: Extensive pneumorrhachis and pneumoperitoneum may simultaneously accompany pneumomediastinum and subcutaneous emphysema in the absence of an identifiable cause and still follow a benign clinical course. Please cite this paper as: Al‐Mufarrej F, Gharagozloo F, Tempesta B and Margolis M. Spontaneous cervicothoracolumbar pneumorrhachis, pneumomediastinum and pneumoperitoneum. The Clinical Respiratory Journal 2009; 3: 239–243.     

Spontaneous pneumomediastinum in a dermatomyositis patient with anti‐melanoma differentiation‐associated gene‐5 antibody and interstitial lung disease despite an initial response to immunosuppressant

We report a 24‐year‐old man with anti‐melanoma differentiation‐associated gene‐5 (MDA5) antibody‐positive dermatomyositis (DM) and interstitial lung disease (ILD) who developed spontaneous pneumomediastinum. By comparing serial thoracic high‐resolution computed tomography scans, we demonstrated the distinct time course showing a paradoxical occurrence of pneumomediastinum despite a radiological improvement of ILD. Our case shows that pneumomediastinum in DM can occur regardless of associated ILD and it is a serious complication that should be considered in DM patients presenting with pulmonary manifestations. Cutaneous vasculopathy may be associated with pneumomediastinum and could potentially be a useful indicator of future disease.     

A case of dermatomyositis complicated with pneumomediastinum

We herein describe a 16-year-old boy with pneumomediastinum, pneumothorax, and subcutaneous emphysema as the initial symptoms of dermatomyositis (DM). His pneumomediastinum disappeared after strict bed rest and he was thereafter successfully treated with oral prednisolone and cyclosporine A. His condition was further complicated with mild interstitial lung disease, arrhythmia, and skin ulcers on his fingertips, right elbow, ear, and sacral region. Pneumomediastinum is a rare complication of DM and its pathogenesis remains unclear. We review the literature and discuss the possible mechanism of this disease.     

Spontaneous Pneumomediastinum: Experience in 18 Adult Patients

Spontaneous pneumomediastinum (SPM) is defined as the presence of air in the mediastinum, developing in the absence of traumatic, iatrogenic, or preceding pulmonary pathologies (emphysema, chronic bronchitis, and lung cancer). The aim of this study was to review our experiences with SPM, underlining its symptomatology, diagnosis, treatment, and followup, and defining a reasonable course of assessment and management. A retrospective case series was conducted to identify adult patients with SPM who were diagnosed and treated in our institution between 1998 and 2005. Eighteen patients (10 males) were identified (average age = 25 ± 4.8 years). Acute onset of chest pain was the predominant symptom at presentation. All patients developed clinically evident subcutaneous emphysema and underwent chest computerized tomography. Fiber bronchoscopy and echocardiogram were used selectively (8 patients). The average hospital stay was 6 (±1.4) days. Sixteen patients were conservatively treated, and only two patients were treated with thoracic drainage due to a related pneumothorax. The disease followed a benign evolution in all patients and, as of today, no relapse has been reported. SPM is an uncommon pathology with a usually benign course. The authors discuss SPM. A diagnostic algorithmic approach is necessary to rule out severe secondary entities and consequences that need urgent treatment.     

A case of dermatomyositis complicated with pneumomediastinum

Abstract

We herein describe a 16-year-old boy with pneumomediastinum, pneumothorax, and subcutaneous emphysema as the initial symptoms of dermatomyositis (DM). His pneumomediastinum disappeared after strict bed rest and he was thereafter successfully treated with oral prednisolone and cyclosporine A. His condition was further complicated with mild interstitial lung disease, arrhythmia, and skin ulcers on his fingertips, right elbow, ear, and sacral region. Pneumomediastinum is a rare complication of DM and its pathogenesis remains unclear. We review the literature and discuss the possible mechanism of this disease.     

Characteristics and prognosis of interstitial pneumonias complicated with pneumomediastinum

BackgroundThe clinical characteristics and prognostic impact of complication with pneumomediastinum in patients with interstitial pneumonias (IPs) are not well studied due to the relatively limited nature of available reports. The purpose of this study was to clarify the characteristics and prognostic factors of IPs complicated with pneumomediastinum.MethodsConsecutive patients with IPs complicated with pneumomediastinum detected by computed tomography (CT) between July 1, 2011, and April 30, 2014 were retrospectively reviewed. Clinical data including symptoms associated with pneumomediastinum, laboratory data, lung function tests, treatments, and mortality were collected from medical records.ResultsForty-five patients (25 males, 20 females), including 32 with idiopathic IP (IIPs) and 13 connective tissue disease-associated interstitial lung diseases (CTD-ILDs) were identified. The median age of onset of pneumomediastinum was 72 years (interquartile range [IQR] 68–79 years). The most common symptom associated with occurrence of pneumomediastinum was appearance or worsening of dyspnoea. No specific treatment was performed for most (84%) of the cases. The median period between occurrence and improvement of pneumomediastinum was 29 days (IQR 5–69 days). Multivariate analysis revealed that IIPs and no improvement of pneumomediastinum were associated with poor prognosis.ConclusionsPatients with IIPs complicated with pneumomediastinum and those without improvement of pneumomediastinum had poor prognosis.     

Cerebral Air Embolism Following the Removal of a Central Venous Catheter in the Absence of Intracardiac Right-to-Left Shunting: A Case Report

Air embolism following central venous catheter (CVC) removal is a relatively uncommon complication. Despite its rare occurrence, an air embolism can lead to serious outcomes. One of the most fatal complications is cerebral air embolism.We report a case of cerebral air embolism that occurred after the removal of a CVC in a patient with an underlying idiopathic pulmonary fibrosis, subcutaneous emphysema, pneumomediastinum, and a possible intrapulmonary shunt. Although the patient had a brief period of recovery, his condition deteriorated again, and retention of carbon dioxide was sustained due to aggravation of pneumonia. Despite full coverage of antibiotics and maximum care with the ventilator, the patient died about 5 weeks after the removal of the CVC.We suggest that strict compliance to protocols is required even while removing the catheter. Furthermore, additional caution to avoid air embolism is demanded in high-risk patients, such as in this case.     

Subcutaneous emphysema, pneumomediastinum, pneumoperitoneum and retropneumoperitoneum following a colonoscopy with mucosectomy

The occurrence of associated intraperitoneal and retroperitoneal colonic perforation is uncommon after colonoscopy. We report a case of this complication revealed by subcutaneous emphysema, pneumomediastinum, pneumoperitoneum and retro-pneumoperitoneum after colonic mucosectomy.     

A case of polymyositis complicated with interstitial pneumonitis and pneumomediastinum

Pneumomediastinum as a complication of interstitial pneumonia with leakage of air into the mediastinum or subcutaneous tissues is a rare complication of dermatomyositis (DM). Herein we report a case of pneumomediastinum complicating polymyositis (PM), which is usually associated with DM. A 61-year-old man was hospitalized in our department because of deterioration of interstitial pneumonia. Treatment with high-dose corticosteroid and cyclosporin A steadily improved his interstitial pneumonia. Two weeks later, he developed subcutaneous emphysema and chest X-ray showed pneumomediastinum. Both subcutaneous emphysema and pneumomediastinum improved gradually without any additional treatment.     

Thin-section CT assessment of spontaneous pneumomediastinum in interstitial lung disease: correlation with serial changes in lung parenchymal abnormalities

PURPOSE: The purpose of this study was to analyze thin-section computed tomography (CT) appearances of interstitial lung diseases before and at the time of detection of pneumomediastinum, and to evaluate the relationship between pneumomediastinum and parenchymal changes on thin-section CT. MATERIALS & METHODS: We reviewed CT images before and at the time of detection of pneumomediastinum in 13 patients with idiopathic pulmonary fibrosis (8 patients) and collagen vascular diseases (5 patient). The extent of the total area of reticular opacity, increased opacity (ground-glass opacity and consolidation), and honeycombing were scored, and these scores were compared before and at the time of detection of pneumomediastinum. We also divided patients into two groups according to therapy received. Patients in group 1 experienced pneumomediastinum after or during treatment with corticosteroids or immunosuppressive agents for acute or subacute exacerbation of interstitial lung disease. Patients in group 2 experienced pneumomediastinum without therapy. RESULTS: The mean score of all patients for honeycombing significantly increased at the time of detection of pneumomediastinum (P=0.003). In group 1, the extent of increasing opacity had been decreased significantly at the time of detection of pneumomediastinum (P=0.028). In group 2, the mean CT score of reticular opacity, increasing opacity, and honeycombing significantly increased at the time of detection of pneumomediastinum (P=0.028, 0.018, and 0.018, respectively). CONCLUSIONS: Spontaneous pneumomediastinum associated with interstitial lung disease appears to have a tendency to occur under conditions of altered of parenchymal interstitial lesions.     

Spontaneous resolution of diffuse persistent pulmonary interstitial emphysema

Persistent pulmonary interstitial emphysema (PPIE) is a rare condition that occurs in both preterm and term infants. It is thought to arise from a disruption of the basement membrane of the alveolar wall allowing air entry into the interstitial space. The characteristic CT scan appearance of PPIE can be used to differentiate it from other congenital cystic lesions that may present similarly. Although conservative management is accepted as the initial form of management in most cases, a review of the published literature found that a significant proportion of localized PPIE cases eventually require surgical resection. This case illustrates that extensive bilateral PPIE associated with a persistent pneumomediastinum can resolve spontaneously thus demonstrating that conservative management without surgical intervention may be appropriate for some children.     

Mixed connective tissue disease-associated interstitial pneumonia complicated by pneumomediastinum during prednisolone therapy]

A 59-year-old woman was admitted to our hospital with exertional dyspnea. Linear and reticular opacities in the middle and lower fields of both lungs were observed on chest roentgenograms and chest computed tomograms (CT). The presence of anti RNP-antibody and Raynaud's phenomenon, sclerosis of the fingers, and leukopenia yielded a diagnosis of mixed connective tissue disease associated with interstitial pneumonia. The symptoms and lung involvement were alleviated after the administration of prednisolone (40 mg/day). However, the patient experienced the sudden onset of dyspnea during pulmonary function tests. A chest X-ray film disclosed mediastinal air around the left pulmonary artery, and a chest CT scan demonstrated some blebs in the left lower lung field. After tapering the dosage of prednisolone in 5 mg increments per week, the pneumomediastinum disappeared without treatment. The clinical features and laboratory data findings suggested the patient's interstitial pneumonia was associated with systemic lupus erythematosus rather than with progressive systemic sclerosis or dermatomyositis. The pneumomediastinum may have been due to the rupture of blebs secondary to interstitial pneumonia during pulmonary function tests or as a result of steroid therapy.     

Smoking-related interstitial lung diseases: a concise review.

Interstitial lung diseases (also known as diffuse infiltrative lung diseases) are a heterogeneous group of parenchymal lung disorders of known or unknown cause. These disorders are usually associated with dyspnoea, diffuse lung infiltrates, and impaired gas exchange. The majority of interstitial lung diseases are of unknown cause. Known causes of interstitial lung disease include inhalation of organic and inorganic dusts as well as gases or fumes, drugs, radiation, and infections. This review summarizes the clinical, radiological, and histopathological features of four interstitial lung disorders that have been linked to smoking. These disorders include desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, pulmonary Langerhans' cell histiocytosis, and idiopathic pulmonary fibrosis. Available evidence suggests most cases of desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, and pulmonary Langerhans' cell histiocytosis are caused by cigarette smoking in susceptible individuals. Smoking cessation should be a main component in the initial therapeutic approach to smokers with these interstitial lung diseases. In addition, smoking appears to be a risk factor for the development of idiopathic pulmonary fibrosis.     

Pneumorrhachis and pneumomediastinum in connective tissue disease-related interstitial lung disease: case series from a tertiary care teaching hospital in South India

Pneumomediastinum has been described as a rare complication of connective tissue diseases. Here, we report four cases of pneumomediastinum: three of which are associated with dermatomyositis and one with mixed connective tissue disease. All our patients had interstitial lung disease. The first case of dermatomyositis described below was complicated by epidural emphysema (pneumorrhachis) in addition to pneumomediastinum. Pneumorrhachis is reported in many isolated case reports and series in the setting of asthma, pneumothorax, blunt chest trauma, etc. Less than 10% of pneumomediastinum cases develop this complication and vast majority of cases resolve spontaneously. The mechanism behind this has been postulated to be the passage of air through the intervertebral foramen. Others suggest entrapment of air which dissects between paraspinal soft tissues and along the vascular and nerve sheaths into the epidural space. This is the first ever reported case of epidural emphysema in connective tissue disease to the best of our knowledge.     

Glossopharyngeal insufflation causes lung injury in trained breath‐hold divers

Background and objective: Glossopharyngeal insufflation (GI) is a technique practised by competitive breath‐hold divers to enhance their performance. Using the oropharyngeal musculature, air is pumped into the lungs to increase the lung volume above physiological TLC. Experienced breath‐hold divers can increase their lung volumes by up to 3 L. Although the potential for lung injury is evident, there is limited information available. The aim of this study was to examine whether there is any evidence of lung injury following GI, independent of diving. Methods: Six male, competitive breath‐hold divers were studied. CT of the thorax was performed during breath‐holding at supramaximal lung volumes following GI (CT _GI ), and subsequently at baseline TLC (CT _TLC ). CT scans were performed a minimum of 3 days apart. Images were analysed for evidence of pneumomediastinum or pneumothorax by investigators who were blinded to the procedure. Results: None of the subjects showed symptoms or signs of pneumomediastinum. However, in five of six subjects a pneumomediastinum was detected during the CT _GI . In three subjects a pneumomediastinum was detected on the CT _GI , but had resolved by the time of the CT _TLC . In two subjects a pneumomediastinum was seen on both the CT _GI and the CT _TLC , and these were larger on the day that a maximal GI manoeuvre had been performed. The single subject, in whom a pneumomediastinum was not detected, was demonstrated separately to not be proficient at GI. Conclusions: Barotrauma was observed in breath‐hold divers who increased their lung volumes by GI. The long‐term effects of this barotrauma are uncertain and longitudinal studies are required to assess cumulative lung damage.     

Cervical emphysema, pneumomediastinum, and pneumothorax following self-induced oral injury: report of four cases and review of the literature.

Spontaneous rupture of the pulmonary alveoli after a sudden increase in intra-alveolar pressure is a common cause of pneumomediastinum, which is usually seen in healthy young men. Other common causes are traumatic and iatrogenic rupture of the airway and esophagus; however, pneumomediastinum following cervicofacial emphysema is much rarer and is occasionally found after dental surgical procedures, head and neck surgery, or accidental trauma. We present four cases of subcutaneous emphysema and pneumomediastinum with two secondary pneumothoraces after self-induced punctures in the oral cavity. They constitute an uncommon clinical entity that, to our knowledge, has not been reported in the literature. Its radiologic appearance, clinical presentation, and diagnosis are described.     

Pneumomediastinum and diffuse alveolar pain. Severe interstitial pneumopathy due to dermatomyositis

We have recently observed the case of a 36-year-old man with dermatomyositis of recent onset, who developed masive pneumomediastinum and subcutaneous emphysema at the onset of a progresive and severe pulmonary disease. Although there were no sign of parenchymal cysts, after the bronchoscopy it was possible to observe endobronchial necrotic injury which was considered as the likely source of the air leak. He was treated with high dose of corticosteroids, cyclophosphamide and cyclosporin A which resulted in the disappearance of the pneumomediastinum and subcutaneous emphysema and the progressive improvement of both parenchymal lung disease and respiratory insufficiency, enabled us to progressively taper the dose of corticosteroids.     

Clinical analysis of 71 spontaneous pneumomediastinum cases: an observational study from a tertiary care hospital in Japan

BackgroundSpontaneous pneumomediastinum is characterized by the presence of interstitial air in the mediastinum without any underlying disease. Some cases of spontaneous pneumomediastinum have been reported in the past, although only few reports are available, and its management remains uncertain. This study reviewed our experience in the diagnosis and treatment of spontaneous pneumomediastinum.MethodsA retrospective study of 71 cases treated for spontaneous pneumomediastinum at the Yodogawa Christian Hospital between April 2005 and March 2020 was conducted.ResultsThe patients’ mean age was 19.3 years (range, 7–48 years). A triggering event was noted in 69% of the cases. Seventy-six percent of the patients were admitted to the hospital, and 24% were outpatients. Treatment included analgesia, rest, antibiotics, and/or oxygen therapy. Thirty-six patients (51%) were treated with antibiotics. None of the cases presented any complications, including mediastinitis or worsening respiratory condition. Two patients (3%) had a recurrence of spontaneous pneumomediastinum.ConclusionsAll the patients, with or without antimicrobial treatment and hospitalization, had favorable outcomes. We should therefore reconsider the need for hospitalization and antimicrobial therapy for patients with mediastinitis prophylaxis.