Mediastinal lymphadenopathy due to VEXAS syndrome.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a rare disease first reported in 2020, most commonly seen in men aged 56-75 years old. Common clinical features include skin lesions (83.5%), fever (63.6%), relapsing chondritis (36.4%), venous thrombosis (34.7%) and lymph node enlargement (33.9%). The patient is a man in his 40s who presented with testicular and lower extremity pain, followed by a rash and bicytopenia. He was initiated on corticosteroids and sulfasalazine. He was found to have mediastinal lymphadenopathy and underwent an endobronchial ultrasound and transbronchial needle aspiration followed by a video-assisted thoracic surgery biopsy which were unrevealing. Eventually, an ubiquitin-like modifier activating enzyme (UBA-1) gene analysis was performed that was consistent with VEXAS syndrome. Patients with VEXAS syndrome usually present with a red or violaceous rash and dyspnoea. Laboratory abnormalities include anaemia, elevated mean corpuscular volume, thrombocytopenia and elevated inflammatory markers. Diagnosis is based on the genetic mutation and associated symptoms. The treatment includes steroids and Janus kinase (JAK) inhibitors, specifically ruxolitinib.

Role of endoscopic ultrasound guided fine needle aspiration (EUS-FNA) and Gene Xpert.

BACKGROUND: Mediastinal tubercular lymphadenitis is form of extrapulmonary tuberculosis [EPTB]. Clinical presentations are non-specific and diagnosis remains great clinical challenge. Microbiological and or histopathological evidences need to be present in order make diagnosis secure before initiation of anti-tubercular therapy (ATT). Endoscopic ultrasound guided fine needle aspiration (EUS-FNA) provides tissue samples and aids management of this difficult to diagnosed entity. Current study describe role of EUS-FNA and Gene Xpert (GXP) in mediastinal tubercular lymphadenitis. METHODS: Retrospective analysis of 72 patients with mediastinal lymphadenopathy who underwent EUS-FNA were carried out. Linear echoendoscope was used for evaluation mediastinum. EUS echo features of LNs were studied. Twenty two-G needle used was for aspiration tissue sample from pathologic lymph nodes (LNs). FNA samples were analysed by cytology, Acid-Fast Bacilli (AFB) staining and GXP study. All procedures were uneventful without any complications. RESULTS: Forty two patients were diagnosed as tuberculosis (TB) following first EUS-FNA setting. Six patients underwent repeat EUS-FNA procedure following which another 3 were diagnosed as TB while remaining 3 started on empirical ATT based on additional supportive evidences. Forty five patients showed granulomatous inflammation on cytological analysis, AFB positivity noted in 16 (33.33%) patients while GXP in 26 (57.78%) patients. Rifampicin resistance detected in 3 ((6.25%) patients. All patients were followed clinico-radiologically for response to treatment. CONCLUSION: Tuberculous lymphadenitis is the most common cause of mediastinal lymphadenopathy in TB endemic countries. EUS-FNA provides microbiological and histopathological/cytological evidences in this difficult to diagnosed EPTB and thereby avoids empirical ATT.

Mediastinal hematoma after trans-radial cerebral angiography: a case report.

BACKGROUND: Trans-radial (TRA) access has become increasingly prevalent in neurointervention. Nonetheless, mediastinal hematoma after TRA is an infrequent yet grave complication associated with a notably elevated mortality rate. While our review found no reported mediastinal hematoma cases managed conservatively within neuro-interventional literature, similar complications are documented in cardiac and vascular interventional radiology, indicating its potential occurrence across disciplines. CASE PRESENTATION: Carotid computed tomography angiography (CTA) showed calcified plaques with stenosis (Left: Severe, Right: Moderate) in the bilateral internal carotid arteries (ICAs) of an 81-year-old male presented with paroxysmal weakness in the right upper limb. Dual antiplatelet therapy with aspirin and clopidogrel was administered. On day 7, DSA of the bilateral ICAs was performed via TRA. Post-DSA, the patient experienced transient loss of consciousness, chest tightness, and other symptoms without ECG or MRI abnormalities. Hemoglobin level decreased from 110 g/L to 92 g/L. Iodinated contrast-induced laryngeal edema was suspected, and the patient was treated with intravenous methylprednisolone. Neck CT indicated a possible mediastinal hemorrhage, which chest CTA confirmed. The patient's treatment plan involved discontinuing antiplatelet medication as a precautionary measure against the potential occurrence of an ischemic stroke instead of the utilization of a covered stent graft and surgical intervention. Serial CTs revealed hematoma absorption. Discharge CT showed a reduced hematoma volume of 35 × 45 mm. CONCLUSIONS: This case underscores the need for timely identification and precise manipulation of guidewires and guide-catheters through trans-radial access. The critical components of successful neuro-interventional techniques include timely examination, rapid identification, proper therapy, and diligent monitoring.

[Mediastinitis Associated with Mediastinal Pancreatic Pseudocyst Successfully Treated by Thoracoscopic Mediastinal Drainage].

Pancreatic pseudocysts rarely extend to the mediastinum and can be fatal if mediastinitis is complicated. In this report, we describe a case of mediastinitis associated with mediastinal pancreatic pseudocyst successfully treated by the thoracoscopic mediastinal drainage. The patient was a man in his 40s with a history of alcoholic acute pancreatitis. Chest and abdominal computed tomography (CT) scan taken for his complaints of back pain and dyspnea showed a pancreatic pseudocyst extending to the mediastinum. First, an endoscopic nasopancreatic drainage( ENPD) tube was placed, and then thoracoscopic mediastinal drainage was performed through the right thoracic cavity. After the operation, the pseudocyst in the mediastinum rapidly disappeared even though there was no drainage from the ENPD tube. Postoperative recovery of the patient was uneventful, and the patient was discharged on the 17th postoperatively day. This case suggests that the importance of prompt treatment for mediastinitis and the effectiveness of the thoracoscopic surgery.

Mediastinal actinomycosis masquerading as a mass: a case of progressive dysphagia in an immunocompromised patient.

Actinomycosis is a rare endogenous infection characterised by indolent progression, contiguous spreading, abscess formation and draining sinuses. Here, we present a case of Schaalia odontolytica causing a mediastinal abscess that is unique in its acuity and location. Our patient presented with worsening dysphagia, and CT of her chest revealed a new mass in the posterior mediastinum displacing the oesophagus. Oesophagram revealed mild motility disorder, but no masses or ulcers within the oesophagus. Oesophagogastroduodenoscopy with endoscopic ultrasound revealed extrinsic compression of the oesophagus. Fine-needle aspiration of the mass yielded purulent fluid, which was cultured. A single colony of S. odontolytica was isolated. Initially, medical treatment was favoured, but as she developed worsening dysphagia, the abscess was drained. She continued on long-term antibiotic therapy after drainage and had complete resolution of the abscess at 1 year.

[EBUS-guided cryobiopsy in the diagnosis of mediastinal lesions - step by step]. / EBUS-gestützte Kryobiopsie mediastinaler Läsionen ­ Schritt für Schritt.

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is the gold standard in the diagnosis of mediastinal and hilar lesions. For certain purposes, such as the diagnosis and subtyping of lymphoproliferative disorders or molecular pathology, a larger amount of intact sample material is required. EBUS cryobiopsy is a new and efficient tool for this purpose. As it is a new approach, there is still no standardised workflow. In this review, we present the procedure step by step as it is performed at the Ruhrlandklinik in Essen.

Mediastinal Lipomatosis.

Mediastinal lipomatosis is a benign condition characterized by excessive deposition of unencapsulated mature adipose tissue in the mediastinum.1 The exact prevalence is not known. The available literature mainly consists of some case series and a bunch of case reports.

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.

Mediastinal Nontuberculous Mycobacterial Infection in Children: A Multidisciplinary Approach.

BACKGROUND: Mediastinal infections due to nontuberculous mycobacteria remain an exceedingly rare entity. Most cases in the published literature do not include pediatric patients. Due to their clinical infrequency, poor response to antimicrobial therapy and often precarious anatomical location, the optimal management of these lesions can be challenging. METHODS: Retrospective medical record review of 4 pediatric cases of mediastinal nontuberculous mycobacteria infection was undertaken. Each child presented with nonspecific respiratory symptoms, including significant acute airway obstruction and required a range of investigations to confirm the diagnosis. Nonresponsiveness to conservative measures and antimycobacterial therapy ultimately resulted in surgical intervention to obtain clinical improvement. RESULTS: All 4 children had extensive evaluation and multidisciplinary involvement in otolaryngology, respiratory medicine, pediatric surgery, infectious diseases and cardiothoracic surgery. They all eventually had their disease debulked via thoracotomy in addition to prolonged antimycobacterial therapy, with successful clinical outcomes. CONCLUSIONS: Mediastinal nontuberculous mycobacteria infections in the pediatric population are rare and diagnostically challenging. A high clinical suspicion should be maintained, and multidisciplinary input sought. Targeted surgery with adjuvant medical therapy can reduce disease burden with minimal long-term morbidity.

Surgical treatment of Boerhaave syndrome in the past, present and future: updated results of a specialised surgical unit.

INTRODUCTION: Boerhaave syndrome is a rare clinical entity associated with high rates of morbidity and mortality. Early recognition of the symptoms, and identification of the site and extension of the injury are key in improving the prognosis. METHODS: This study presents data on the mortality, morbidity and length of hospital stay in patients diagnosed with Boerhaave syndrome. The data were retrieved from a prospectively collected database in a single surgical unit between 2012 and 2022. The study makes a comparison with the surgical outcomes of the previous decade. RESULTS: Some 33 patients were diagnosed with Boerhaave syndrome and were treated surgically between 2012 and 2022 in a specialist upper gastrointestinal surgical unit. All patients underwent standard surgical repair (in-theatre diagnostic endoscopy, T-tube placement through thoracotomy and feeding jejunostomy through laparotomy). The mean size of the defects in the oesophageal lumen was 3.3cm. Delayed presentation was noted for 13 patients (39%); 8 patients (24%) died in hospital, and 19 patients (58%) developed postoperative complications. Mortality was similar to the rate recorded for the 20 patients from the previous decade (24% vs 20%, respectively). The mean length of hospital stay was 41 days, and was comparable to the 35.7 days reported between 1997 and 2011. CONCLUSIONS: Early and aggressive management of spontaneous oesophageal rupture ameliorates the postoperative recovery and prognosis. The surgical results of our unit were found comparable to the previous decade in the population of patients who were treated surgically.

Endobronchial Ultrasound-Guided Transbronchial Mediastinal Cryobiopsy versus Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration for Mediastinal Disorders: A Meta-Analysis.

INTRODUCTION: Endobronchial ultrasound-guided transbronchial mediastinal cryobiopsy (EBUS-TMC), a novel technique, has been reported to improve the diagnostic value of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for mediastinal lesions in recent studies. Current literature suggests that this procedure has greater diagnostic efficacy compared to conventional EBUS-TBNA. This systematic review and meta-analysis aimed to evaluate the diagnostic yield and complications associated with EBUS-TMC in comparison to EBUS-TBNA, thereby exploring the potential of this novel technique in enhancing the diagnostic utility for mediastinal lesions. METHODS: A comprehensive literature review was conducted by searching the PubMed, Embase, and Google Scholar databases for articles published from inception to December 31, 2023. The objective of this review was to evaluate the utilization of EBUS-TMC in diagnosing mediastinal disease, while also assessing the quality of each study using the QUADAS-2 tool. The diagnostic yield estimates were subjected to a meta-analysis utilizing inverse variance weighting. Furthermore, a comprehensive analysis of the complications associated with this procedure was performed. RESULTS: The meta-analysis included 10 studies involving a total of 538 patients. The findings of the meta-analysis demonstrated that EBUS-TMC yielded an overall diagnostic rate of 89.59% (482/538), while EBUS-TBNA yielded a rate of 77.13% (415/538). The calculated inverse variance-weighted odds ratio was 2.63 (95% confidence interval, 1.86-3.72; p < 0.0001), and I2 value was 11%, indicating a statistically significant difference between the two techniques. The associated complications consisted of pneumothorax, pneumomediastinum, mediastinitis, and bleeding, with an incidence of 0.74% (4/538), 0.37% (2/538), 0.0% (0/538), and 1.12% (6/538), respectively. Moreover, the funnel plot displayed no discernible publication bias. Further subgroup analysis revealed a notable improvement in the diagnosis value for lymphoma (86.36% vs. 27.27%, p = 0.0006) and benign disorder (87.62% vs. 60.00%, p < 0.0001). CONCLUSION: This review of the current available studies indicated that EBUS-TMC enhanced overall diagnostic yields compared to EBUS-TBNA, particularly for diagnosing benign disease and lymphoma. This procedure was not associated with any serious complications.

Rosai-Dorfman disease manifesting as a solitary mass with fat in the thymus a case report.

BACKGROUND: Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare, self-limiting disease that predominantly affects children and young adults. Moreover, the disease is characterized by painless bilateral cervical lymphadenopathy in 95% of the patients. However, few reports are available on the Rosai-Dorfman disease of the thymus. CASE PRESENTATION: We report a rare case of thymic Rosai-Dorfman disease detected using computed tomography. During a medical examination, a 50-year-old man underwent a chest computed tomography scan, which revealed an anterior mediastinal single mass with fat in the thymus. A thymectomy was performed to completely remove the tumor using a thoracoscopic technique due to a clinical suspicion of thymoma. Furthermore, Rosai-Dorfman disease was confirmed using histological and immunohistochemical analyses. CONCLUSIONS: To the best of our knowledge, this is the sixth case of thymus-affecting solitary Rosai-Dorfman disease with histological and immunohistochemical evidence. Fat in the thymus, as was present in this case, has never been described in Rosai-Dorfman disease previously. Our results highlight the challenge of diagnosing this uncommon tumor before surgery, and more cases need to be reported to help with the preoperative diagnosis of such a rare tumor.

A 49-Year-Old Man With Fever and Dyspnea After Endobronchial Ultrasound-Guided Transbronchial Fine Needle Aspiration Biopsy.

CASE PRESENTATION: A 49-year-old man, a farmer, had been experiencing coughing, phlegm, and difficulty breathing for 2 months. He underwent a CT scan at a local hospital that showed a mediastinal mass. Bronchoscopy showed no obstruction in the tracheal lumen, and an endobronchial ultrasound-guided transbronchial fine needle aspiration (EBUS-TBNA) biopsy was performed on the mediastinal mass. The cytologic smear of the mediastinal mass showed a few atypical epithelial cells; the possibility of a tumor could not be ruled out. The patient visited our thoracic surgery outpatient department; based on the advice of the thoracic surgeon, the patient underwent another endobronchial ultrasound-guided transbronchial fine needle aspiration biopsy of the mediastinal mass 4 days before this admission. The patient went home and waited for the results. Two days later, the patient experienced a fever and palpitations accompanied by chills, yellow phlegm, and orthopnea. The patient visited our ED, underwent tracheal intubation, and was admitted to our ICU. The patient had had occasional coughing and phlegm for the past 10 years, which were not taken seriously or investigated. The patient does not smoke or drink alcohol, and there is no history of cancer in the family.

Tracheomediastinal fistula induced by concurrent chemoradiotherapy in small cell lung cancer: A case report and literature review.

Tracheomediastinal fistula is a rare but life-threatening complication of cancer. We report a case of tracheomediastinal fistula induced by concurrent chemoradiotherapy in limited stage small cell lung cancer. Despite the treatment response, the metastatic paratracheal lymph node increased gradually during concurrent chemoradiotherapy, resulting in the occurrence of tracheomediastinal fistula and mediastinitis. Without any surgical intervention, the patient achieved successful recovery from mediastinitis through antibiotic treatment, although the tracheomediastinal fistula remained open. In this report, we also review previous studies of tracheomediastinal and bronchomediastinal fistulas and summarize the clinical features.

Transbronchial lymph node forceps biopsy as a novel tool in diagnosis of mediastinal lymphadenopathy: a pilot study.

BACKGROUND: Differential diagnosis of mediastinal lymphadenopathy is an issue of debate. Lymph nodes may be enlarged due to a variety of inflammatory, infectious, or malignant reasons. Therefore, obtaining samples from the affected nodes is crucial for the diagnosis. Usually, these patients are subjected to TBNA (EBUS or conventional) or mediastinoscopy if TBNA is not conclusive. This study evaluated the safety and feasibility of this new technique of transbronchial forceps biopsy for the diagnosis of mediastinal lymphadenopathy. METHODS: The study included 18 patients with confirmed mediastinal lymphadenopathy who were admitted in Chest Department, Cairo University in the period from December 2019 to December 2020. All patients were subjected to flexible bronchoscopy with conventional transbronchial needle aspiration (C-TBNA) and transbronchial forceps biopsy (LN-TBFB) from the enlarged mediastinal lymph node in the same procedure. RESULTS: we found the technique of LN-TBFB safe with no serious complications. We were able to reach a diagnosis in 7/7 (100%) cases of sarcoidosis, 6/7 (85.7%) cases of malignant lymph nodes. We had three cases where the histopathology showed hyperactive follicular hyperplasia, and a single case of tuberculous lymphadenitis. C-TBNA was diagnostic in 71.4% of sarcoidosis cases, 42.9% of malignant cases, but failed to diagnose the one patient with tuberculous lymphadenitis. CONCLUSION: Lymph node transbronchial forceps biopsy (LN-TBFB) was found to be safe and effective in the diagnosis of mediastinal lymphadenopathy. We strongly advocate the use of this minimally invasive technique for diagnosing pathologically enlarged mediastinal lymph nodes, as a last step before mediastinoscopy.

Boerhaave's Syndrome Presenting in the Setting of Third-Degree Heart Block.

Introduction: Boerhaave's syndrome, or the spontaneous transmural perforation of the esophagus, is typically thought to be due to an increase in esophageal pressure such as that which occurs during vomiting or retching. Another common etiology of esophageal perforation is esophageal instrumentation, such as during esophagogastroduodenoscopy or transesophageal echocardiography. This life-threatening condition requires prompt diagnosis and treatment to prevent patient demise. While a history of vomiting can aid in diagnosis, this history can be difficult to elicit in an unconscious patient or may be altogether absent. Additionally, Boerhaave's syndrome can present similarly to more common upper gastrointestinal or cardiac conditions. Since mortality increases with delays in diagnosis and treatment, it is imperative that clinicians maintain a high level of suspicion for Boerhaave's syndrome and initiate treatment urgently. Case Description: This report presents a 76-year-old man who presented to the emergency department after a history of several syncopal episodes and was found to be in complete heart block. Two days later, he acutely developed abdominal distention and coffee ground emesis. As the medical team was able to gather more history from the patient and his family, it was revealed that he had associated vomiting with his episodes of syncope. CT scan of the abdomen and pelvis demonstrated pneumomediastinum concerning for esophageal perforation. His clinical status subsequently deteriorated. He was intubated and a temporary transvenous pacer was placed before being transferred to our facility for emergent surgery. Discussion: Complete heart block in the setting of Boerhaave's syndrome is exceptionally rare, with only 2 cases reported in the literature. The decision to place a pacemaker in the setting of esophageal perforation/sepsis is complicated and depends on the patient's bacteremia status related to noncardiac comorbidities. Clearly this case represents the need for excellent multidisciplinary decision-making processes with excellent communication between hospital staff and all caretakers. Expeditious diagnosis and treatment of esophageal perforation is essential to prevent leaking of gastric contents into the mediastinum and worsening of cardiac complications and sepsis. Additionally, critical timing of various surgical procedures, especially the need for a permanent pacemaker implant with bacteremia is a complicated process not well described in the surgical literature.