Congenital bronchial atresia (CBA) is a rare airway anomaly that may radiologically resemble other congenital pulmonary lesions, particularly when it occurs in the lower lobes. A 41-year-old woman was referred for surgical treatment of suspected pulmonary sequestration based on imaging findings. Computed tomography (CT) at the referring hospital showed a cavitary lesion with fluid retention in Segment 7 (S7) of the right lower lobe, with surrounding ground-glass opacity, suggesting infection associated with pulmonary sequestration. Re-evaluation at our institution identified the lesion as a bronchocele with focal hyperinflation, interruption of the segmental bronchus (B7), and a normally distributed pulmonary artery (A7) without aberrant systemic supply. Three-dimensional CT clearly demonstrated these bronchovascular features and supported the diagnosis of CBA. Thoracoscopic right lower lobectomy was performed, and histopathological findings confirmed the diagnosis of CBA. This case highlights the importance of systematic evaluation of bronchial continuity and vascular supply in congenital lower-lobe lesions, for which three-dimensional CT reconstruction may be useful.
Cheyne-Stokes respiration (CSR) is a potentially dangerous abnormal breathing pattern that can be associated with serious cardiac events and poor prognosis. This case describes the unexpected recording of CSR events by a continuous positive airway pressure (CPAP) apparatus in a patient who was consistently CPAP compliant and had a stable medical history except for recent nasal obstruction. Respiratory polygraphy (RP) suggested the CPAP device had erroneously recorded obstructive sleep apnea (OSA) as CSR due to mixed apnea features that mimicked CSR. Treatment of the nasal obstruction resolved the problem with the erroneous readings, suggesting the nasal obstruction was the cause of the aberrant CPAP recording. This case highlights the limits of CPAP device reliability for detection of CSR and the need for standardized CSR detection criteria across CPAP devices.
Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive technique for sampling mediastinal lesions but may be inadequate for diagnosing lymphoma because of limited tissue acquisition. We report a case of T-lymphoblastic lymphoma (T-LBL) successfully diagnosed using endobronchial ultrasound-guided transbronchial mediastinal cryobiopsy (EBUS-Cryo). An 18-year-old woman presented with an anterior mediastinal mass causing severe tracheal compression. EBUS-TBNA followed by EBUS-Cryo using a 1.1-mm cryoprobe was performed under endotracheal intubation without complications. Although EBUS-TBNA specimens were nondiagnostic because of crush artefacts and blood contamination, EBUS-Cryo yielded well-preserved tissue, enabling comprehensive immunohistochemical evaluation and a definitive diagnosis of T-LBL. This case demonstrates that EBUS-Cryo can provide sufficient tissue for diagnosing mediastinal T-LBL and may be safely performed in carefully selected patients, even in the presence of substantial airway compression.
This case report highlights a novel and successful therapeutic strategy for a patient with acute exacerbation of Chronic Obstructive Pulmonary Disease (AECOPD) and severe central airway stricture (CAS). We demonstrate the use of extracorporeal CO2 removal (ECCO2R) as a critical physiological bridge, stabilizing the patient's hypercapnic respiratory failure and allowing for the safe and definitive management of the airway stenosis through bronchoscopic stenting.
Injectable breast fillers are increasingly used as minimally invasive alternatives to implants. Delayed complications such as migration and lymphadenopathy are increasingly reported. Mediastinal lymphadenopathy due to hyaluronic acid (HA) filler migration is rare. A woman in her 50s presented with progressive facial puffiness and chest wall venous distension. Six months earlier, an evaluation at another center had revealed mediastinal lymphadenopathy causing superior vena cava (SVC) compression. Video-assisted thoracoscopic (VATS) biopsy revealed chronic inflammatory infiltrates. Further targeted history revealed prior bilateral breast augmentation using HA fillers. Repeat computed tomography demonstrated interval enlargement of the mediastinal lymphadenopathy with worsening SVC compression, together with radiological features suggestive of migration of filler material into the mediastinal nodes. Endobronchial ultrasound-guided transbronchial needle aspiration yielded polymorphous lymphocytes. Magnetic resonance imaging of the breast demonstrated multiple bilateral lesions with irregular capsules. Following corticosteroid therapy, the patient experienced marked improvement in clinical symptoms, with a reduction in the mediastinal lesion on follow-up imaging.
An immunocompetent young male in his early 20s presented with a 1-month history of central chest pain, dyspepsia and significant weight loss. Initial evaluation revealed a large, deep oesophageal ulcer and extensive necrotic mediastinal lymphadenopathy. Endobronchial ultrasound-guided fine-needle aspiration of the mediastinal nodes confirmed necrotising granulomatous inflammation, suggestive of tuberculosis. Following initiation of anti-tuberculosis treatment (ATT), he initially showed systemic improvement. However, within 2 weeks, he developed drug-induced liver injury along with new-onset focal neurological deficits, including right-sided hemiparesis and vocal cord palsy. Neuroimaging revealed multiple new intracranial lesions involving the left temporal lobe, right posterior parietal region and brainstem, whilst repeat cross-sectional imaging of the chest and abdomen was suggestive of interval improvement. A diagnosis of paradoxical isolated central nervous system tuberculosis-associated immune reconstitution inflammatory syndrome was made. He was managed with high-dose systemic corticosteroids and a modified hepatoprotective ATT regimen, resulting in clinical improvement.
Striatin-anaplastic lymphoma kinase (STRN-ALK) is a rare fusion variant in non-small cell lung cancer (NSCLC), and the efficacy of first-line lorlatinib for this subgroup is poorly defined. We report a 45-year-old treatment-naïve male with STRN-ALK-positive lung adenocarcinoma and symptomatic brain metastases. Following stereotactic radiosurgery for intracranial lesions, the patient initiated lorlatinib at 100 mg once daily as the first systemic therapy. After starting treatment, neuropsychiatric side effects necessitated a gradual reduction in dosage to 75 mg and then to 50 mg. The patient achieved a durable partial response with sustained intracranial disease control for over 1 year. Adjusting the dosage improved tolerability without compromising efficacy, allowing for ongoing treatment. This case demonstrates that first-line lorlatinib can achieve durable systemic and intracranial disease control in STRN-ALK-positive NSCLC, including in patients presenting with brain metastases, while maintaining antitumor efficacy despite dose reduction.
COPA syndrome is a rare autosomal dominant autoimmune disorder caused by COPA gene mutations, leading to immune dysregulation and multisystem involvement. We report a 45-year-old man with recurrent respiratory failure, haemoptysis, renal dysfunction and arthritis. Symptoms began at Age 27 with diffuse alveolar damage requiring mechanical ventilation. Imaging showed progressive interstitial lung disease and biopsy revealed follicular bronchiolitis. Immunosuppression provided temporary stabilisation. In 2024, next-generation sequencing confirmed a likely pathogenic COPA variant. The patient later discontinued therapy and remained untreated for 1 year, returning with worsening renal dysfunction and significant proteinuria, while pulmonary findings remained stable. Cyclophosphamide was initiated for suspected renal relapse. During treatment, he developed acute diverticulitis complicated by sepsis, progressing to septic shock and multiorgan failure despite surgery and antibiotics, resulting in death. Early recognition and multidisciplinary management are essential to improve outcomes of the disease.
Background:Pneumothorax with persistent air leak (PAL) is a debilitating condition. Patients with PAL often require prolonged chest drainage, with associated complications, such as, hospital-acquired infections, thromboembolism, and deconditioning. Endobronchial valves (EBVs) are an approved bronchoscopic treatment for PAL and may allow earlier chest tube removal and hospital discharge. Current evidence supporting the use of EBV is limited to case series and uncontrolled studies. EBVs are usually used as a last resort when other treatments failed. Whether early referral for EBV placement is feasible and can reduce hospital length of stay in patients with pneumothorax and PAL has not been tested in a randomised controlled trial (RCT). Methods:EVAPoRATe is a prospective, open-labelled, pilot, multi-centre RCT. Forty patients with pneumothorax of any cause and PAL who are unsuitable for or unwilling to undergo surgical management will be randomised 1:1 to the Early EBV Referral arm or the Standard Care arm. Patients in the Early EBV Referral arm will be assessed by an interventional clinician for EBV placement at the earliest feasible time from Day 5 of pneumothorax. Patients allocated to the Standard Care arm will receive conventional management by the attending team; EBV is allowed if clinically appropriate when air leak persists after 12 days. Participants will be followed for 6 months after discharge. Discussion:The primary outcome is the feasibility of the trial protocol, assessed by recruitment rate, adherence to the intervention arm, and retention at follow-up. Secondary outcomes include total hospital length of stay, PAL-related length of stay, interventions for PAL and EBV complications, time to air leak cessation, time to chest drain removal, pneumothorax recurrence, adverse events and serious adverse events, removal of EBVs, performance status, and mortality. This pilot feasibility study will assess whether a future full-scale RCT of EBV for PAL is practical and will provide data to guide protocol refinement and sample size calculation. Trial Registration:Australian New Zealand Clinical Trials Registry: ACTRN12625000360415; https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=388086&isReview=true.
Airway compression is a recognized complication after pulmonary artery banding (PAB) in infants with congenital heart disease; however, conventional imaging primarily provides anatomical information. We report an infant with respiratory instability after PAB who underwent computational fluid dynamics (CFD) as an adjunctive functional assessment. After extubation, recurrent intercostal retractions and oxygen desaturation occurred during crying. Contrast-enhanced CT and bronchoscopy revealed mild-to-moderate tracheobronchial narrowing caused by dilated pulmonary arteries without intrinsic obstruction. A patient-specific CFD model simulated inspiratory airflow at 100 mL/s, approximating crying. Total airway resistance was markedly elevated (15 Pa/mL/s vs. approximately 0.07 Pa/mL/s in healthy infants), with pronounced airflow asymmetry and reduced flow to the right main bronchus, suggesting functional impairment despite relatively preserved airway lumens. These findings were considered complementary to the overall clinical assessment, rather than an independent surgical indication. The patient's symptoms resolved after pulmonary artery plication and ventricular septal defect closure.
Acute exacerbation of interstitial lung disease (AE-ILD) is often idiopathic, although viral infections are recognized precipitating factors. We report an adult patient with underlying ILD who developed acute respiratory deterioration in the setting of primary parvovirus B19 infection. A 70-year-old Japanese man was admitted to our department with a low-grade fever and a facial rash. Two years prior to admission, the patient had been diagnosed with ILD, which had been treated with systemic corticosteroids that were subsequently discontinued following clinical improvement. High-resolution computed tomography (HRCT) revealed newly developed bilateral ground-glass opacities. Infection with parvovirus B19 was confirmed by serological testing and bronchoalveolar lavage results. Although the precise contribution of parvovirus B19-associated lung injury, infection-triggered AE-ILD and acute exacerbation of the underlying ILD could not be definitively determined, steroid pulse therapy led to clinical and radiological improvement. This case highlights the importance of considering parvovirus B19 infection as a potential contributor to acute respiratory deterioration in patients with underlying ILD.
A 69-year-old man who was a former plumber with a history of occupational asbestos exposure was diagnosed with unresectable pleural mesothelioma. Six months after initiation of combination therapy with ipilimumab and nivolumab, he presented with an altered level of consciousness and extrapyramidal symptoms and was diagnosed with immune-related encephalitis. Although high-dose corticosteroid therapy and intravenous immunoglobulin led to partial improvement in his level of consciousness, the response was limited. Immune-related encephalitis is rare, especially in association with pleural mesothelioma. Optimal management of the condition needs to be established.
Endobronchial aspergillosis (EBA) is an uncommon manifestation of Aspergillus airway disease characterized by fungal involvement of the tracheobronchial lumen. It may present with non-specific respiratory symptoms, including dyspnea, cough and hemoptysis, and can mimic endoluminal airway tumours. Predisposing factors include immunosuppressive conditions such as corticosteroid therapy, malignancy, chemotherapy and neutropenia. Definitive diagnosis relies on bronchoscopic evaluation with histopathological confirmation. Due to its rarity, evidence regarding optimal management remains limited. This case emphasizes that isolated airway Aspergillus infection may occur without parenchymal lung involvement and may be associated with a rapidly fatal clinical course despite prompt antifungal therapy.
Negative pressure pulmonary oedema (NPPE) is well recognised in peri-operative settings but is infrequently reported following brief non-operative airway obstruction, particularly in adolescents. We report the case of an adolescent male who developed NPPE with features of concurrent negative pressure pulmonary haemorrhage (NPPH) following approximately 90 s of neck compression. He experienced transient loss of consciousness with hypoxic myoclonic activity, followed by haemoptysis and hypoxia requiring supplemental oxygen. Chest X-ray (CXR) revealed bilateral opacities, which was followed up by computed tomography (CT) of the chest demonstrating diffuse bilateral ground-glass and confluent opacities consistent with pulmonary oedema and probable superimposed haemorrhage. He recovered with supplemental oxygen alone within 48 h. This case shows that even brief neck compression can cause significant pulmonary injury, that chokeholds are an under-recognised precipitant, and that concurrent NPPH may mimic aspiration or infection, risking unnecessary intervention.
Differentiating tuberculous (TB) pleurisy from malignant pleural mesothelioma is challenging due to similar clinical presentations. We report an 83-year-old man with sarcomatoid mesothelioma initially treated for TB pleurisy. He presented with lymphocyte-predominant exudative pleural effusion and a positive Interferon-Gamma Release Assay. Thoracoscopy revealed purulent effusion, but biopsies were non-diagnostic. Despite empiric anti-tuberculosis therapy, the effusion persisted. A subsequent surgical biopsy showed fibrosis and scattered spindle cells. However, 2 months later, a rapidly growing chest wall mass with bone invasion appeared. A CT-guided percutaneous biopsy showed proliferation of atypical spindle cells and fluorescence in situ hybridization (FISH) analysis allowed for the diagnosis of sarcomatoid mesothelioma. This case highlights the aggressive nature of sarcomatoid mesothelioma and its diagnostic difficulty. For treatment-refractory pleurisy, clinicians must maintain high suspicion, ensuring vigilant follow-up and prompt repeat biopsies despite negative initial results, while consulting pathologists to consider additional FISH testing.
Non-invasive ventilation (NIV) is standard respiratory support for amyotrophic lateral sclerosis (ALS), but intolerance may limit its use. High-flow nasal cannula (HFNC) has emerged as a potential alternative in selected patients, but evidence regarding its use remains scarce. We describe 18 ALS patients' management with HFNC between 2014 and 2024 after documented intolerance to NIV. Mean age at HFNC initiation was 65.1 ± 12.5 years, and 66.7% of patients were female. Partial NIV intolerance occurred in 38.9% of cases, while 33.3% tolerated intermittent combined NIV and HFNC. During follow-up, 50.0% of patients required hospitalization, 33.3% experienced respiratory infections, and 27.8% died. Arterial blood gas parameters remained stable, including PaCO2 (p = 0.314). Functional status declined significantly, with Barthel Index decreasing (35.0 ± 20.5 vs. 11.7 ± 11.3; p < 0.001), whereas respiratory muscle strength remained unchanged. HFNC was feasible as home-based support in ALS patients intolerant to NIV, warranting prospective comparative evaluation.
Histopathological confirmation is essential to determine the optimal treatment strategy for patients with multiple pulmonary lesions. We report a case of synchronous small cell lung cancer (SCLC) and non-small cell lung cancer (NSCLC) diagnosed during the same bronchoscopic session using endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and balloon dilation for bronchoscope delivery (BDBD) technique. A 70-year-old man with a history of gastric cancer presented with mediastinal lymphadenopathy and a nodule in the right upper lobe. EBUS-TBNA confirmed SCLC in the mediastinal lymph nodes. For the peripheral pulmonary lesion, BDBD enabled further advancement of the thin bronchoscope, allowing direct visualization and biopsy with standard forceps, which confirmed NSCLC. No procedure-related complications occurred. This case suggests that combining EBUS-TBNA with BDBD facilitates the accurate diagnosis of multiple pulmonary lesions during the same bronchoscopic session.
Pulmonary sarcoidosis is a systemic inflammatory disease characterized by the formation of non-caseating granulomas; it typically presents with bilateral hilar lymphadenopathy and upper-lobe predominant interstitial infiltrates. However, atypical radiological presentations can mimic fibrotic interstitial lung diseases, posing a significant diagnostic challenge. We present a case of pulmonary sarcoidosis in a young female who had a confounding basal-peripheral phenotype, eventually diagnosed through endobronchial biopsy showing non-necrotizing granulomas with asteroid bodies.
A 65-year-old woman with primary Sjögren's syndrome without a history of immunosuppressive therapy presented with progressively enlarging pulmonary nodules showing calcification and cystic changes, mimicking malignancy. Histological examination confirmed AL λ-type nodular pulmonary amyloidosis. Recognition of this radiological pattern is important to avoid misdiagnosis and unnecessary invasive investigations.
Secondary spontaneous pneumothorax (SSP), a complication of chronic obstructive pulmonary disease (COPD), recurs more frequently in underweight patients. This case report describes a 63-year-old malnourished man with COPD presenting with recurrent left-sided SSP shortly after hospital discharge for a previous episode. Following chest tube reinsertion and complete lung re-expansion, chemical pleurodesis was performed using 1000 mg doxycycline in saline. Transient pleuritic pain was controlled with intravenous ketorolac and intrapleural lidocaine. No other immediate or delayed adverse events occurred. In parallel, pulmonary rehabilitation, including breathing exercises, posture correction, moderate-intensity endurance and inspiratory muscle training, was initiated alongside nutritional repletion with a respiratory-specific oral nutritional supplement. Over a 22-month follow-up, the patient remained free of SSP recurrence, with improved functional capacity and weight gain. This report describes a long-term recurrence-free outcome after doxycycline pleurodesis combined with pulmonary rehabilitation and nutritional support in an underweight patient with COPD.
