Osteochondroplastic tracheobronchopathy: Four case reports. / Traqueobroncopatía osteocondroplástica: reporte de cuatro casos clínicos.

Introduction: Osteochondroplastic tracheobronchopathy is a rare benign chronic disease of unknown etiology. Bronchoscopy remains the gold standard for diagnosing osteochondroplastic tracheobronchopathy. Its typical findings are described as a cobblestone, rock garden, mountainscape, or stalactite cave appearance. The present work aims to show the main clinical features of this rare pathology. Clinical cases: The clinical data of four middle-aged patients, three men and one woman, were analyzed. The main clinical symptoms were chronic cough, dyspnea, and dysphonia. The patient's preliminary diagnosis was made by computed axial tomography of the chest, confirmed by bronchoscopy and histopathological examination. Treatment included medication for symptoms and, in one case, cryosurgery and argon plasma coagulation. Discussion: Diagnosing osteochondroplastic tracheobronchopathy was not easy, given its uncommon nature and non-specific symptoms often found in other pathologies. No case series articles on this pathology have been published in Peru. Therefore, we used the original articles published in other countries to reference our findings. Conclusion: Osteochondroplastic tracheopathy is a benign disease that typically affects adults. Men are more likely to be affected. Its clinical manifestations are non-specific and frequently of pharyngeal origin, and the cause is not yet defined. Chest computed axial tomography combined with bronchoscopy are the main diagnostic procedures. There is no standard treatment with consistent therapeutic effects.

Introducción: La traqueobroncopatía osteocondroplástica es una rara enfermedad crónica benigna de etiología desconocida. La broncoscopía sigue siendo el estándar de oro para el reconocimiento de traqueopatía osteocondroplástica. Sus hallazgos típicos se describen como un empedrado, un jardín de rocas, una apariencia de paisaje montañoso o de una cueva con estalactitas. El objetivo del presente trabajo es mostrar las principales características clínicas de una patología poco conocida. Casos clínicos: Se analizaron los datos clínicos de cuatro pacientes de mediana edad, tres fueron hombres y una mujer. Los principales síntomas clínicos fueron tos crónica, disnea, disfonía. Los pacientes tuvieron un diagnóstico preliminar mediante tomografía axial computarizada de tórax, confirmado por examen video broncoscópico e histopatológico. El tratamiento incluyó medicamentos para los síntomas y en un solo caso criocirugía y coagulación con argón plasma. Discusión: El diagnóstico de traqueobroncopatía osteocondroplástica no fue sencillo por ser una entidad rara, cuyos síntomas son inespecíficos y muy frecuentes en otras patologías. En Perú no se han publicado artículos de serie de casos sobre esta patología. Por lo tanto, tomamos como referencia artículos originales publicados en otros países para compararlos con nuestros hallazgos. Conclusión: La traqueopatía osteocondroplástica es una enfermedad benigna que predispone a los adultos, los hombres tienen más probabilidades de verse afectados. Sus manifestaciones clínicas son inespecíficas; frecuentemente de origen faríngeo y la causa no está aún definida. La tomografía axial computarizada de tórax combinada con video broncoscopía son los principales procedimientos para el diagnóstico. No existe un estándar de tratamiento con efectos terapéuticos consistentes.

[Risk factors for pulmonary atelectasis in adults with tracheobronchial tuberculosis].

Objective: To investigate the risk factors for pulmonary atelectasis in adults with tracheobronchial tuberculosis(TBTB). Methods: Clinical data of adult patients (≥18 years old) with TBTB from February 2018 to December 2021 in Public Health Clinical Center of Chengdu were retrospectively analyzed. A total of 258 patients were included, with a male to female ratio of 1∶1.43. The median age was 31(24, 48) years. Clinical data including clinical characteristics, previous misdiagnoses/missed diagnoses before admission, pulmonary atelectasis, the time from symptom onset to atelectasis and bronchoscopy, bronchoscopy and interventional treatment were collected according to the inclusion and exclusion criteria. Patients were divided into two groups according to whether they had pulmonary atelectasis. Differences between the two groups were compared. Binary logistic regression was used to analyze the risk factors for pulmonary atelectasis. Results: The prevalence of pulmonary atelectasis was 14.7%, which was most common in the left upper lobe (26.3%). The median time from symptom onset to atelectasis was 130.50(29.75,358.50)d, and the median time from atelectasis to bronchoscopy was 5(3,7)d. The median age, the proportion of misdiagnosis of TBTB before admission, and the time from symptom onset to bronchoscopy in the atelectasis group were higher than those without atelectasis, and the proportion of receiving bronchoscopy examination and interventional therapy previously, and the proportion of pulmonary cavities were lower than those without atelectasis (all P<0.05). The proportions of cicatrices stricture type and lumen occlusion type in the atelectasis group were higher than those without atelectasis, while the proportions of inflammatory infiltration type and ulceration necrosis type were lower than those without atelectasis (all P<0.05). Older age (OR=1.036, 95%CI: 1.012-1.061), previous misdiagnosis(OR=2.759, 95%CI: 1.100-6.922), longer time from symptom onset to bronchoscopy examination (OR=1.002, 95%CI: 1.000-1.005) and cicatrices stricture type (OR=2.989, 95%CI: 1.279-6.985) were independent risk factors for pulmonary atelectasis in adults with TBTB (all P<0.05). Of the patients with atelectasis who underwent bronchoscopy interventional therapy, 86.7% had lung reexpansion or partial reexpansion. Conclusions: The prevalence of pulmonary atelectasis is 14.7% in adult patients with TBTB. The most common site of atelectasis is left upper lobe. The TBTB type of lumen occlusion is complicated by pulmonary atelectasis in 100% of cases. Being older, misdiagnosed as other diseases, longer time from onset of symptoms to bronchoscopy examination, and being the cicatrices stricture type are factors for developing pulmonary atelectasis. Early diagnosis and treatment are needed to reduce the incidence of pulmonary atelectasis and increase the rate of pulmonary reexpansion.

A case of tracheobronchial amyloidosis with emphasis on differential diagnosis.

Tracheobronchial amyloidosis is a manifestation of amyloidosis of the respiratory tract characterized by focal or diffuse deposition of amyloid in the submucosa of the trachea and proximal bronchi. Tracheobronchial amyloidosis is not associated with systemic amyloidosis or pulmonary parenchymal involvement. It affects predominantly men aged over fifty. Depending on the part of the tracheobronchial tree that is affected, stenosis of the airways causes a variety of unspecific symptoms. Diagnosis is reached by means of typical presentation in CT scan followed by bronchoscopy and histopathological confirmation. Tracheobronchial amyloidosis should be borne in mind in the differential diagnosis of patients with chronic cough and/or dyspnea or recurrent respiratory infections.

Tracheobronchopathia Osteochondroplastica: A Case Report.

BACKGROUND: Tracheobronchopathia osteochondroplastica (TO) is a rare idiopathic disease with a stable course that involves the mucous membrane of the tracheobronchial tree. Most cases present no specific symptoms, and there are currently no established guidelines for diagnosis and treatment. In this report, we discuss a single case of a patient with TO who was diagnosed based on clinical imaging and histopathology. CASE SUMMARY: A patient with a history of smoking and alcohol consumption, but no specific clinical symptoms, was diagnosed with TO after undergoing fiber-optic bronchoscopy. Nodular processes with smooth surface mucosa and detached bronchial mucosa were observed. The presence of TO was confirmed by pathological examination. CONCLUSION: The diagnosis of TO is difficult, and early fiber-optic bronchoscopy and pathological examination should be performed to facilitate the diagnosis.

Benign Intratracheal Thyroid: A Systematic Review of 43 Cases With Five New Case Reports.

OBJECTIVE/HYPOTHESIS: To examine the clinical features of benign intratracheal thyroid (ITT) and their management strategies and outcomes. STUDY DESIGN: Case series study. METHODS: This systemic review was conducted in two international academic centers. This review includes 43 patients: one new case from the Massachusetts Eye and Ear Infirmary, four new cases from Beijing Tongren Hospital, and 38 previously published cases. We analyzed these 43 cases and summarized the patients' epidemiological data, clinical features, and treatment regimens. RESULTS: ITTs were less common in men than in women (male:female ratio of 3:10). ITT was observed in patients as young as neonates and as old as 85 years. Orthotopic thyroid nodules were present in 55.8% of the patients with ITT. Malignancy was incidentally found in 4.6% of all ITTs. Imaging examinations showed that the ITTs were typically attached to the posterolateral/lateral tracheal wall of the first, second, or third tracheal rings. Tissue attachment between the ITT and normal thyroid lobes was seen in 59.5% of the patients. Thirty-seven patients underwent surgery: 30 underwent open surgery, and seven underwent endoscopic debulking resections. One neonate received thyroid suppression therapy. One patient with ITT and papillary thyroid cancer was treated with radiotherapy and ultimately died after recurrence. CONCLUSIONS: Surgical resection is an effective treatment for benign ITT. We hypothesized that abnormalities during the embryonic development of Berry's ligament might play a role in ITT pathogenesis. LEVEL OF EVIDENCE: NA Laryngoscope, 131:E2609-E2617, 2021.

A 57-Year-Old Man With Stridor and Critical Tracheal Narrowing.

CASE PRESENTATION: A 57-year-old man with a history of polysubstance use presented with shortness of breath, wheezing, productive cough, subjective fever, and chills of 3-day duration. Additionally, he reported worsening shortness of breath for the last 3 months. Of note, the patient was reported to have had, in the previous 6 months, two episodes of pneumonia that was treated with antibiotics and steroids. He was also diagnosed several years prior with adult-onset asthma due to intermittent wheezing and was prescribed an albuterol inhaler. The albuterol did not help relieve his wheezing, and he stopped refilling it.

Localised laryngotracheal amyloidosis: a differential diagnosis not to forget.

We present a case of multifocal laryngotracheal amyloidosis (LTA) in a 43-year-old man with persistent and progressive dysphonia and dyspnoea, and a first inconclusive histology. Although laryngeal amyloidosis accounts for fewer than 1% of all benign laryngeal tumours, it is in fact the most common site of amyloid deposition in the head, neck and respiratory tract. The clinical scenario is non-specific and diagnosis depends on a high degree of suspicion and on histology. Imaging is useful in mapping lesions, which are often more extensive than they appear during laryngoscopy. Despite being a benign entity, the prognosis is variable with a high-rate and long-latency recurrences, requiring long-term follow-up.

Surgical Management in Tracheobronchopathia Osteochondroplastica: A Case Study.

Tracheobronchopathia Osteochondroplastica is a benign condition characterized by osseous and cartilaginous submucosal growths of the tracheobronchial tree. This is a case report of an individual that was to undergo elective surgery using general anesthesia with endotracheal tube intubation. However, the anesthesiologist encountered a large osseous mass of the precricoid region and could not be intubated. This case report describes the technique for removal of the obstructing lesion using a Sonopet ultrasonic aspirator. Laryngoscope, 131:E911-E913, 2021.

Genotype-Phenotype Correlation of Tracheal Cartilaginous Sleeves and Fgfr2 Mutations in Mice.

OBJECTIVES: To characterize tracheal cartilage morphology in mouse models of fibroblast growth factor receptor (Fgfr2)-related craniosynostosis syndromes. To establish relationships between specific Fgfr2 mutations and tracheal cartilaginous sleeve (TCS) phenotypes in these mouse models. METHODS: Postnatal day 0 knock-in mouse lines with disease-specific genetic variations in the Fgfr2 gene (Fgfr2C342Y/C342Y , Fgfr2C342Y/+ , Fgfr2+/Y394C , Fgfr2+/S252W , and Fgfr2+/P253R ) as well as line-specific controls were utilized. Tracheal cartilage morphology as measured by gross analyses, microcomputed tomography (µCT), and histopathology were compared using Chi-squared and single-factor analysis of variance statistical tests. RESULTS: A greater proportion of rings per trachea were abnormal in Fgfr2C342Y/+ tracheas (63%) than Fgfr2+/S252W (17%), Fgfr2+/P253R (17%), Fgfr2+/Y394C (12%), and controls (10%) (P < .001 for each vs. Fgfr2C342Y/+ ). TCS segments were found only in Fgfr2C342Y/C342Y (100%) and Fgfr2C342Y/+ (72%) tracheas. Cricoid and first-tracheal ring fusion was noted in all Fgfr2C342Y/C342Y and 94% of Fgfr2C342Y/+ samples. The Fgfr2C342Y/C342Y and Fgfr2C342Y/+ groups were found to have greater areas and volumes of cartilage than other lines on gross analysis and µCT. Histologic analyses confirmed TCS among the Fgfr2C342Y/C342Y and Fgfr2C342Y/+ groups, without appreciable differences in cartilage morphology, cell size, or density; no histologic differences were observed among other Fgfr2 lines compared to controls. CONCLUSION: This study found TCS phenotypes only in the Fgfr2C342Y mouse lines. These lines also had increased tracheal cartilage compared to other mutant lines and controls. These data support further study of the Fgfr2 mouse lines and the investigation of other Fgfr2 variants to better understand their role in tracheal development and TCS formation. LEVEL OF EVIDENCE: NA Laryngoscope, 131:E1349-E1356, 2021.

Tracheal A-Frame Deformities Following Airway Reconstruction.

OBJECTIVES: Airway reconstruction for subglottic and tracheal stenosis is often successful in achieving tracheostomy decannulation and improving airway symptoms. However, one common reason for late failure is development of a tracheal A-frame deformity, which can necessitate additional surgery. Although knowledge of this deformity exists, the incidence and risk factors have not been reported. This study seeks to determine the incidence of A-frame following airway reconstruction and define factors that correlate with development of this deformity. STUDY DESIGN: Retrospective case series. METHODS: Patients under 21 years of age undergoing open airway reconstruction at our institution between January 2005-December 2006 were retrospectively reviewed. Demographic data, comorbidities, airway history/reconstruction type, and follow-up airway findings were examined using multivariable logistic regression. Kaplan-Meier curves were used to examine time to A-frame repair. RESULTS: Two hundred patients underwent airway reconstruction and 69 (34.5%) developed an A-frame deformity. History of tracheostomy was the most significant contributor to A-frame development (P < .0001). Double- versus single-stage procedures were not associated with increased odds of A-frame development (P = .94), however, patients undergoing resection procedures as opposed to laryngotracheal reconstruction (LTR) with cartilage grafts had a significantly lower chance of developing this deformity (P = .004). Of the patients with an A-frame, 27 (39%) required further surgical intervention. CONCLUSION: Approximately one-third of patients undergoing airway reconstruction developed a tracheal A-frame deformity, with a significantly higher rate among patients with a history of tracheostomy and those undergoing LTR. Patients should be followed long term to assess for the development of an A-frame. LEVEL OF EVIDENCE: IV Laryngoscope, 131:E1363-E1368, 2021.

Tracheobronchial amyloidosis in primary Sjögren syndrome: A case report.

RATIONALE: Tracheobronchial amyloidosis (TBA) associated with Sjögren syndrome is very rare. Here, we describe a case with this phenomenon, in order to better understand the condition. PATIENT CONCERNS: A 52-year-old woman presented after 6 months of coughing, sputum, and dyspnea. Chest computed tomography revealed thickened bronchial walls, which were irregular on the left side the trachea. She had a history of dry eye and dry mouth of at least 3 years' duration. DIAGNOSES: Sjögren syndrome was diagnosed based on her symptoms, ophthalmological and parotid examination, and immunological and autoantibody tests. The diagnosis of TBA was confirmed by Congo red staining of a tracheal biopsy. INTERVENTIONS: The patient was given glucocorticoids without any other immunosuppressants. OUTCOMES: The symptoms improved after 6 months. LESSONS: TBA associated with Sjögren syndrome is a rare condition. TBA is characterized by amyloid deposition to the trachea in the absence of systemic amyloidosis. Diagnosis requires tissue biopsy with demonstration of amyloid deposition.

Tracheobronchopathia Osteochondroplastica-Clinical, Radiological, and Endoscopic Correlation: Case Series and Literature Review.

Tracheobronchopathia osteochondroplastica (TO) is a rare idiopathic and benign disease that is often underdiagnosed. TO is characterized by multiple submucosal cartilaginous and osseous tracheobronchial nodules that spare the posterior wall. It usually affects the elderly, developing when the person is around 60 years old without gender preference and has a reported incidence of 0.11%. TO can be symptomatic and should be considered in patients with chronic cough, dyspnea, and recurrent pulmonary infections. Diagnosis is usually incidental by computed tomography or bronchoscopy, the latter being the gold standard diagnostic test for TO. Many thoracic imagers are not well acquainted with TO; thus, these patients are often underdiagnosed or misdiagnosed. We came across 5 patients in our institution who were incidentally diagnosed with TO, inspiring us to review the available literature on this disease. A total of 33 patients diagnosed with TO between 2009 and 2019 were identified by our retrospective review. Clinical and imaging data were collected on these patients. We also included the clinical, radiological, and endoscopic data of our 5 cases. TO should be considered in patients with chronic cough, dyspnea, and recurrent pulmonary infections. Our experience is that both computed tomography and bronchoscopy can be used to make a reliable diagnosis. It is crucial for physicians, especially radiologists and pulmonologists, to be aware of the existence of TO in order to ensure proper diagnosis.

Tracheobronchopathia Osteochondroplastica: Five Cases Report and Literature Review.

Tracheobronchopathia osteochondroplastica (TO) is a rare disease. Here, we report 5 TO cases treated at our hospital. Bronchoscopy showed typical multiple firm and glossy nodules in all the 5 cases. Conservative treatment effectively alleviated the symptoms. Tracheobronchopathia osteochondroplastica is a manageable disease. Awareness in clinicians is critical to avoid unnecessary treatment in patients with TO.

Laryngeal Mask as the Primary Airway Device During Laryngotracheal Surgery: Data From 108 Patients.

BACKGROUND: Airway management during repair of laryngotracheal stenosis is demanding, and there is currently no accepted standard of care. Recently an increasing number of airway centers have started to use a laryngeal mask until the airway is surgically exposed and cross-table ventilation can be initiated. However detailed data on this approach are missing in the literature. METHODS: Patients receiving laryngotracheal surgery from November 2011 until October 2018 were retrospectively included in this single-center study, except for patients who presented with a preexisting tracheostomy at time of surgery. Airway management uniformly consisted of laryngeal mask ventilation until cross-table ventilation was established. Clinical variables, perioperative complications, and airway complications were analyzed. RESULTS: One hundred eight patients (65 women, 43 men) receiving tracheal resection (n = 50), cricotracheal resection (n = 49), or single-stage laryngotracheal reconstruction (n = 9) were included in the analysis. Of the included patients 23 (21.3%) had malignant disease and 85 (78.7%) a benign pathology. In the subgroup of patients with subglottic disease 85.1% had high-grade stenosis (Myer-Cotton III°). Airway management with a laryngeal mask was successful in all except 1 patient (99.1%). Mean pulse oximetry and mean end-tidal CO2 during laryngeal mask ventilation was 98.7% ± 2.4% and 34.8 ± 7.6 mm Hg, respectively. At the end of surgery 95 patients (88%) were successfully weaned from the respirator using the laryngeal mask. CONCLUSIONS: The laryngeal mask as the primary airway device is feasible and safe in patients undergoing laryngotracheal surgery even in cases with high-grade stenosis.

Transplantation of a 3D-printed tracheal graft combined with iPS cell-derived MSCs and chondrocytes.

For successful tracheal reconstruction, tissue-engineered artificial trachea should meet several requirements, such as biocompatible constructs comparable to natural trachea, coverage with ciliated respiratory mucosa, and adequate cartilage remodeling to support a cylindrical structure. Here, we designed an artificial trachea with mechanical properties similar to the native trachea that can enhance the regeneration of tracheal mucosa and cartilage through the optimal combination of a two-layered tubular scaffold and human induced pluripotent stem cell (iPSC)-derived cells. The framework of the artificial trachea was fabricated with electrospun polycaprolactone (PCL) nanofibers (inner) and 3D-printed PCL microfibers (outer). Also, human bronchial epithelial cells (hBECs), iPSC-derived mesenchymal stem cells (iPSC-MSCs), and iPSC-derived chondrocytes (iPSC-Chds) were used to maximize the regeneration of tracheal mucosa and cartilage in vivo. After 2 days of cultivation using a bioreactor system, tissue-engineered artificial tracheas were transplanted into a segmental trachea defect (1.5-cm length) rabbit model. Endoscopy did not reveal granulation ingrowth into tracheal lumen. Alcian blue staining clearly showed the formation of ciliated columnar epithelium in iPSC-MSC groups. In addition, micro-CT analysis showed that iPSC-Chd groups were effective in forming neocartilage at defect sites. Therefore, this study describes a promising approach for long-term functional reconstruction of a segmental tracheal defect.

APEC infection affects cytokine-cytokine receptor interaction and cell cycle pathways in chicken trachea.

Avian pathogenic Escherichia coli (APEC) can lead to extraintestinal disease in avian species via respiratory tract infection. However, the regulatory mechanism of APEC on the pathogenicity of chicken trachea epithelium remains unknown. In this study, we examined pathological changes in chicken trachea at different infection times (4, 8, 12 and 24 h). The RNA sequencing of APEC infection group and the PBS group (negative control) of chicken trachea epithelium were analysed. Our studies revealed that the oedema, heterophil infiltration and hyperaemia appeared at 8 and 12 h post APEC infection. And the hyperaemia phenomenon and heterophilic granulocyte infiltration disappeared at 24 h post infection. Then RNA sequencing showed many genes were dynamically expressed in the APEC infection group. At 4, 8 and 12 h post infection, the mRNA of differentially expressed genes were enriched by cytokine-cytokine receptor interaction and the toll-like receptor signalling pathway. The cell cycle pathway was enriched at 24 h post infection. Altogether, these findings suggest that APEC infection induces pathological change in the chicken trachea, the mRNA of differentially expressed genes participating in inflammation and hyperplasia signalling pathways. Which not only provide more evidence for regulatory mechanism of APEC on the pathogenicity of chicken trachea epithelium, but also facilitate the effective management of APEC infections in poultry through trachea.