Rosai-Dorfman disease (RDD) rarely involves the trachea, and active pulmonary tuberculosis (TB) has not been reported with tracheal RDD. A 35-year-old woman presented with progressive cough, wheezing, and shortness of breath for 1 month. Chest CT revealed a tracheal mass causing > 90% airway narrowing and left lung nodules suggestive of TB. Emergency bronchoscopic resection using high-frequency electrocautery relieved the obstruction. Histopathology showed emperipolesis and immunohistochemistry was consistent with RDD. Bronchial aspirate was positive for Mycobacterium tuberculosis complex by culture and GeneXpert, without rifampicin resistance. Standard anti-TB therapy was given. Two-month CT and one-year follow-up bronchoscopy and CT showed a patent airway without recurrence. The patient remained symptom-free with no other organ involvement. This is the first report of primary tracheal RDD coexisting with active pulmonary TB. Bronchoscopic resection combined with anti-TB therapy achieved durable disease control at 1 year.

Primary tracheobronchial tumors are rare and are frequently diagnosed late because their presenting symptoms are nonspecific and may mimic more common respiratory conditions. We report a case of a 74-year-old man with a significant smoking history who presented with progressive dyspnea, cough, hemoptysis, and stridor, initially treated with multiple courses of antibiotics and corticosteroids, without improvement. Chest radiography was reportedly normal, but a contrast-enhanced CT demonstrated an endoluminal soft tissue mass centered at the distal trachea/carina with extension into the bilateral mainstem bronchi, causing near-complete obstruction of the left mainstem bronchus and moderate-to-severe narrowing of the right mainstem bronchus. Coronal FDG PET/CT demonstrated intense metabolic activity centered at the carina. Predebulking bronchoscopy confirmed a verrucous fungating carinal mass causing severe central airway compromise, whereas postdebulking bronchoscopy showed marked restoration of airway patency. Histopathologic evaluation established squamous cell carcinoma. This case highlights the diagnostic limitations of chest radiography and the essential role of CT, FDG PET/CT, and bronchoscopy in evaluating malignant central airway obstruction.

Backgroud: Pathologies of the trachea and large bronchi are a heterogeneous group of rare structural disorders of the airway wall that are frequently underdiagnosed because their symptoms overlap with common respiratory diseases. Methods: We present three patients with chronic cough and dyspnea lasting approximately three years who had previously received multiple incorrect diagnoses, including COPD, asthma, gastroesophageal reflux, and acute bronchitis. Case Description: Following hospitalization for acute respiratory failure, comprehensive differential diagnostic workups revealed Mounier-Kuhn syndrome (tracheobronchomegaly) in Case 1, idiopathic tracheobronchomalacia in Case 2, and tracheobronchopathia osteochondroplastica in Case 3. Tracheobronchomegaly involves marked dilatation of the trachea and main bronchi; tracheobronchomalacia is characterized by expiratory airway collapse due to loss of cartilage stability; and tracheobronchopathia osteochondroplastica is a disease of unknown etiology featuring abnormal submucosal chondrification and ossification with luminal obstruction. All three impair mucociliary clearance and predispose to recurrent infections and progressive respiratory dysfunction. Conclusions: Through these cases we underscore the diagnostic challenges, characteristic radiological findings, and diverse management strategies of these conditions, emphasizing the critical need to consider tracheobronchial anomalies in patients with chronic respiratory symptoms and the key role of computed tomography in establishing an accurate diagnosis.

Juvenile xanthogranuloma (JXG) is a rare benign histiocytic disorder, typically affecting the skin of infants and young children. Extracutaneous manifestations are uncommon, and laryngeal involvement is exceedingly rare but potentially life-threatening. We report the case of a young girl with a one-year history of dysphonia and dyspnea. Laryngoscopy revealed a JXG of the left vocal fold which was endoscopically removed. The patient remains to this date symptom-free without recurrence. Only eleven cases of upper airway or tracheal JXG have been published so far. Therapeutic strategies range from tracheostomy to endoscopic excision. Our case underscores the risks of airway obstruction in this otherwise benign condition. It provides guidance for general management and highlights the importance of complete resection and close follow-up, given the notable risk of recurrence.

Emergency front-of-neck access (FONA) is a last-resort intervention in "cannot intubate, cannot oxygenate" situations. The underlying assumption is that establishing an airway at the cricothyroid membrane will restore ventilation. We report a fatal case in which FONA via percutaneous cricothyroidotomy failed to restore ventilation in a patient with cardiac arrest due to mochi (sticky rice cake) aspiration. A 51-year-old man presented in pulseless electrical activity cardiac arrest after choking on mochi. Bag-mask ventilation was ineffective, and the oropharynx was densely packed with mochi, precluding visualization of the epiglottis. Percutaneous cricothyroidotomy was performed, but ventilation remained ineffective despite technically successful placement. Subsequent endotracheal intubation after oral clearance also failed to achieve ventilation. Postmortem computed tomography revealed a patent airway at the cricothyroid level with obstructing foreign material in the mid-trachea and near-complete occlusion at the carina. This case illustrates that FONA cannot overcome distal tracheobronchial obstruction and highlights a structural limitation of front-of-neck airway access in the management of mochi aspiration.

Invasive tracheal mucormycosis is a rare but rapidly progressive central airway fungal infection associated with high mortality. Its early clinical manifestations and radiological features are often nonspecific, which may result in delayed diagnosis. We report a case of invasive tracheal mucormycosis in a patient with diabetic ketosis. The patient underwent emergency tracheostomy for necrotizing upper airway obstruction. Bronchoscopic examination revealed extensive white necrotic tissue in the subglottic region and trachea. Broad, ribbon-like, pauci-septate hyphae were identified in both bronchoalveolar lavage fluid and biopsy specimens, establishing the diagnosis. Following systemic antifungal therapy with amphotericin B, adjunctive nebulized local treatment, and repeated bronchoscopic interventions, the infection was controlled, and maintenance therapy was sequentially transitioned to oral isavuconazole. However, the patient ultimately developed irreversible glottic closure and severe subglottic stenosis, resulting in long-term tracheostomy dependence. This case highlights that mucormycosis should be considered early in the differential diagnosis of patients with diabetes, particularly those with diabetic ketosis, who present with necrotizing or pseudomembranous airway lesions on bronchoscopy and non response to broad spectrum antibiotics. Deep respiratory specimens should be obtained promptly to establish etiological and histopathological diagnoses, while adequate antifungal therapy and correction of metabolic disturbances should be initiated simultaneously to improve outcomes. Nevertheless, even after infection control is achieved, patients may still be left with severe structural airway damage. Therefore, airway function preservation and long-term prognostic assessment should also be emphasized in clinical management.

Almost all patients with mucopolysaccharidosis (MPS) develop respiratory dysfunction of varying severity during disease progression. While respiratory disease in MPS has traditionally been attributed to upper airway obstruction caused by glycosaminoglycan (GAG) accumulation in the trachea and bronchi, involvement of the intrapulmonary conducting airways and lung parenchyma remains poorly defined. Here, we characterised lung disease in a mouse model of MPS I using a combination of non-invasive X-ray Velocimetry (XV) functional lung imaging and gold-standard flexiVent respiratory mechanics testing, complemented by lung volume measurements and histological analysis. XV provides regional ventilation information across the entire lung during tidal breathing. MPS I mice demonstrated reduced mean specific ventilation (the average regional expansion of lung tissue across the respiratory cycle), driven predominantly by reduced ventilation in the inner (mediastinal-adjacent) lung regions, with evidence of spatially heterogeneous ventilation distribution. Lung mechanics testing showed increased conducting airway resistance, increased respiratory system compliance and reduced tissue elastance, consistent with impaired elastic recoil and expiratory flow limitation. Lung volume analysis revealed reduced opening pressure following degassing together with increased residual volume, functional residual capacity and vital capacity. Histological analysis demonstrated heterogeneous parenchymal architecture with regions of enlarged airspaces. Together, these findings demonstrate that respiratory dysfunction in MPS I is not limited to upper airway obstruction but also involves intrinsic abnormalities of the intrapulmonary conducting airways and lung parenchyma. This intrinsic pulmonary pathology likely contributes to obstructive lung disease and may underlie the susceptibility to respiratory failure observed in patients with MPS I.

Congenital high airway obstruction syndrome (CHAOS) is a rare, life-threatening condition involving complete or near-complete airway obstruction. Fetal laryngoscopy enables direct airway endoluminal visualization of the airway, thereby complementing prenatal imaging to enhance diagnosis and counseling. In this case, CHAOS was diagnosed at 17 weeks of gestation following ultrasound and fetal magnetic resonance imaging, which revealed tracheal dilation and echogenic lungs. Diagnostic fetal laryngoscopy performed at 24 weeks demonstrated a blind-ended trachea that was not amenable to fetal decompression. Despite counseling regarding the limited feasibility of airway establishment, emergent delivery with an ex utero intrapartum treatment (EXIT) procedure was performed at 30 weeks. Airway establishment was unsuccessful, and the neonate died shortly after birth. Significant maternal hemorrhage occurred after delivery, requiring transfusion. This case underscores the complexity of prenatal counseling and family-centered decision-making. Fetal laryngoscopy may be considered an adjunct to improve diagnostic evaluation and counseling, potentially aiding prenatal decision-making in selected cases of CHAOS.