Tracheobronchial Cartilage Research Articles

Endobronchial optical coherence tomography helps to estimate the cartilage damage of the central airway in TBTB patients.

At present, there are few examination methods used to evaluate tracheobronchial cartilage damage. In our study, we explored whether endobronchial optical coherence tomography (EB-OCT) can be used to estimate central airway cartilage damage in tracheobronchial tuberculosis (TBTB) patients. In our study, we used the OCTICS Imaging system to perform EB-OCT scanning for TBTB patients. The thickness of the central airway wall and cartilage was measured by the OCTICS software system workstation. There were 102 TBTB patients included in our study cohort. Their EB-OCT images of the central airway cartilage showed that abnormal cartilage manifests as thinning of the cartilage, cartilage damage, cartilage destruction, and even cartilage deficiency. The cartilage morphology becomes irregular and discontinuous. Some parts of the cartilage become brighter in grayscale. The intima of the cartilage is thickened and discontinuous, and the boundary with submucosa and mucosa is unclear. Our study conducted EB-OCT examination of the central airway cartilage of TBTB patients in vivo for the first time. EB-OCT helps to estimate the cartilage damage of the central airway in TBTB patients to some extent.

A Case of Relapsing Polychondritis with Tracheobronchial Involvement

Relapsing Polychondritis (RPC) is a rare systemic inflammatory disorder of unknown etiology and characterized by recurrent and progressive inflammation of the cartilaginous structures, particularly involving the auricles, nose and respiratory tract as well as extra-cartilaginous tissues, including eyes, heart, skin, central nervous and hematological systems. Its diagnosis can be difficult when the typical clinical features such as auricular chondritis are absent. Here, we report on a case of 43-year-old woman who presented with recurrent sore thorat, dysphagia, extertional dyspnea, cough and noisy breathing initially misdiagnosed as acute laryngitis who was eventually diagnosed as Relapsing polychondritis with tracheobronchial involvement. Chest computed tomography showed the diffuse involvement of tracheobronchial cartilage. Based on the, Damiani’s criteria, she was diagnosed as relapsing polychondritis even though there was no unique involvement of auricular cartilage, and high dose steroid and immunosuppressive therapy were then started. This case indicated that patients who have tracheobronchial cartilage involvement without definite auricular chondritis should be considered for relapsing polychondritis as a differential diagnosis. This case is reported to raise awareness of airway involvement in RPC and discuss its current management.

Three-Dimensional Analysis of Human Laryngeal and Tracheobronchial Cartilages during the Late Embryonic and Early Fetal Period

Laryngeal and tracheobronchial cartilages are present as unique U-shaped forms around the respiratory tract and contribute to the formation of rigid structures required for the airway. Certain discrepancies still exist concerning cartilage formation in humans. To visualize the accurate timeline of cartilage formation, tracheobronchial and laryngeal cartilages were 3D reconstructed based on serial tissue sections during the embryonic period (Carnegie stage [CS] 18–23) and early fetal period (crown rump length [CRL] = 35–45 mm). The developmental phases of the cartilage were estimated by histological studies, which were performed on the reconstructed tissue sections. The hyoid greater horns were recognizable at CS18 (phase 2). Fusion of 2 chondrification centers in the mid-sagittal region was observed at CS19 in the hyoid bone, at CS20 in the cricoid cartilage, and in the specimen with CRL 39 mm in the thyroid cartilage. Phase 3 differentiation was observed at the median part of the hyoid body at CS19, which was the earliest among all other laryngeal and tracheobronchial cartilages. Most of the laryngeal cartilages were in phase 3 differentiation at CS22 and in phase 4 differentiation at CS23. The U-shaped tracheobronchial cartilages with phase 2 differentiation covered the entire extrapulmonary region at CS20. Phase 3 differentiation started on the median section and propagates laterally at CS21. The tracheobronchial cartilages may form simultaneously during the embryonic period at CS22-23 and early fetal periods, similar to adults in number and distribution. The spatial propagation of the tracheal cartilage differentiation provided in the present study indicates that cartilage differentiation may have propagated differently on phase 2 and phase 3. This study demonstrates a comprehensible timeline of cartilage formation. Such detailed information of the timeline of cartilage formation would be useful to improve our understanding of the development and pathophysiology of congenital airway anomalies.

Severe tracheobronchial harm due to lithium button battery aspiration: An in vitro study of the pathomechanism and injury pattern

BackgroundButton battery incidents have become a rising medical issue in recent years, especially for infants. The increasing number of these cases can be explained by the expanding use of objects of everyday life and toys. As a result, button batteries in many households are ubiquitous in different states of charge. The extremely long shelf-life and the increasing energy densities of lithium button batteries boost the potential medical complications of accidental swallowing. ObjectiveThe study aimed to analyze the pathophysiology of damage to tracheobronchial structures by button batteries aspiration over time. MethodsCR2032 and CR927 lithium button batteries (3.2/3.0 V) were exposed to porcine trachea preparations intraluminal at 37 °C in intervals up to 36 h. Measurements were made of the voltage curve, the discharge current, and the resulting pH values around the electrodes. The effects on tissue were examined using macroscopic time-lapse images and microscopic pictures of sections of the fixed specimens over time. FindingsThe examinations showed a tissue electrolysis reaction directly after the beginning of battery exposure, which led to an immediate coagulation impairment of the respiratory epithelium. Over time, a strongly alkaline environment was established around the batteries. The resulting tissue colliquation caused profound tissue damage beyond the basal membrane of the mucosa, affecting the tracheobronchial cartilage after only 4 h of exposure time. After 12 h, there was significant necrosis of the annular ligaments of the trachea and the peribronchial pulmonary tissue. After completion of the experimental exposure time of 36 h, there was still a sufficient residual voltage on all button batteries of the experiments. ConclusionsBesides accidental ingestion, the aspiration of button batteries is a life-threatening situation. The partial or complete acute airway obstruction in the trachea or the bronchi initially is the leading symptom, as with any foreign body aspiration. However, the results of the investigations show that even after a short exposure time, relevant tissue damage can be caused by the electrolysis reaction of the battery. After 12 h, a profound destruction of cartilage, connective tissue, and smooth muscles was observed in vitro, which may cause significant consequential damage in vivo. These findings reveal the need for rapid diagnosis and immediate foreign body removal after any battery ingestion. Moreover, the results show how relevant prevention of these accidents is, and that future safety modifications of these types of battery by the manufacturers would be appropriate.

A rare diagnosis: Keutel Syndrome

Tracheobronchial cartilage calcification is a rare finding in the pediatric population. Keutel syndrome (OMIM 245150) is a very rare syndrome characterized with diffuse calcification of cartilage, brachytelephalangia, pulmonary stenosis, midline defects, stippled epiphysis in infancy, and hearing loss accompanied by recurrent respiratory infections and asthma-like attacks. Here, we present a 14-year-old patient who was followed up with the diagnosis of asthma, but did not respond to appropriate asthma treatment. She was subsequently diagnosed as having Keutel syndrome with cartilage calcification on the tracheobranchial tree and auricula, atypical facial features, recurrent otitis media, hearing loss, and recurrent asthma-like symptoms.

Diagnosis and treatment of 34 cases of congenital tracheobronchial cartilage remnants of esophagus

PurposeTo describe the diagnosis and treatment of 34 cases of congenital tracheobronchial cartilage remnants of esophagus. MethodsThe diagnosis and treatment of 34 cases of congenital tracheobronchial cartilage remnants of esophagus were analyzed retrospectively in our hospital. ResultsCongenital tracheobronchial cartilage remnants of esophagus could be specifically diagnosed by clinical situation and esophageal barium meal examination. The anterior wall of the esophagus was cut longitudinally with the posterior wall of the esophagus preserved. And the cartilage was removed and the open anterior wall of the esophagus was sutured horizontally with full layer. In our study, 34 patients who underwent the operation had a satisfactory outcome. However, one patient underwent submucosal cartilage stripping, which resulted in a complication involving fistulae from the esophagus to the abdominal cavity that were healed after gastrostomy and nutritional support. ConclusionCongenital tracheobronchial cartilage remnants of esophagus caused symptoms when the infants were started on adjunct foods. Vomiting the food without gastric fluid and bile was the leading clinical manifestation. Distinguishing signs on esophageal barium contrast could be used as preoperative diagnosis evidence. Surgically removing the cartilage and horizontally suturing the esophagus provides a reliable outcome. Level of EvidenceLevel IV. Type of StudyRetrospective study.

Two Cases of Warfarin-Induced Tracheobronchial Calcification After Fontan Surgery

This study identified tracheobronchial cartilage calcification in children with congenital heart disease. Calcification of the tracheobronchial airways has been found previously in adults receiving warfarin and in children receiving warfarin after mitral valve replacement. A 9-year-old girl who had received a Fontan repair 6 years previously underwent a cardiac computed tomography (CT) scan to evaluate pulmonary artery size. The result was an incidental finding of extensive tracheobronchial cartilage calcification. A retrospective review of all pediatric Fontan patients who had undergone cardiac CT was conducted to search for calcification of the tracheobronchial cartilage. The study investigated ten pediatric Fontan patients who had undergone cardiac CT scanning. Two patients with extensive calcification of the tracheobronchial airways were identified. The index case had hypoplastic left heart syndrome, and the patient had undergone a staged repair with the Fontan at the age of 3 years. A 16-year-old boy with tricuspid atresia had undergone staged repair and Fontan at the age of 3.5 years. These two patients had received continuous warfarin therapy for 6 and 13 years, respectively. Other common causes of airway calcification were excluded from the study. This report describes warfarin-induced tracheobronchial calcification in patients after the Fontan procedure. This finding has possible implications for airway growth and vascular calcification.

Endobronchial Ultrasonography and Magnetic Resonance Imaging in Tracheobronchial Stenosis From Ulcerative Colitis

A 49-year-old woman presented with continuous cough, progressive dyspnea on exertion, and hoarseness. She had a total colectomy for ulcerative colitis 17 years earlier. Bronchoscopy showed circumferential mucosal erythema. The surface of the tracheal mucosa was irregular and bled easily on contact. Endobronchial ultrasonography and magnetic resonance imaging (MRI) showed characteristic findings that suggested that the lesion was located within the tracheal mucosa and submucosa. Endobronchial ultrasonography images showed circumferential thickening of the mucosa, but tracheobronchial cartilage was preserved intact. Moreover, the comparison between tracheal tissues from tracheostomy and colon tissues resected 17 years earlier showed similarities in pathologic findings. These findings suggested that inflammatory bowel disease can cause the tracheobronchial stenosis.

An unusual cause of recurrent hemoptysis: tracheopatia osteoplastica

An otherwise healthy 55-year-old man was referred to the hospital by his general practitioner due to recurrent episodes of hemoptysis. His medical history was silent, although his working activity (textile branch) exposed him to organic dusts. Shortly after an acute bronchitis treated with aspirin, the patient had a first episode of hemoptysis. Even after aspirin withdrawal, thoracic sense of oppression and cough persisted for several days, and a second hemoptysis event occurred. Cardiothoracic physical examination was unremarkable, and his general practitioner performed electrocardiogram, chest X-ray, and blood chemistry panel (all these examinations were normal), and asked for cardiologic and otorhinolaryngoiatric consultations, with no findings of pathological remarks. Hemoptysis is a relatively common symptom of cardiopulmonary disease. Although bronchitis is a very common cause, a careful evaluation may be necessary to exclude more serious pathologic conditions, such as bronchogenic carcinoma and tuberculosis [1]. Moreover, the decision on whether to perform further investigations has not been influenced by the amount of bleeding. In fact, there is no correlation between quantity of blood and seriousness of the underlying thoracic disease [1]. In this case, given the patient’s age, his working history, the recurrence of hemoptysis episodes, and prior negative results, we decided not to dismiss hemoptysis as simply due to bronchitis, and proceed with a more advanced workup including CT scan and bronchoscopy. Thoracic high-resolution computerized tomography (HRCT) showed limited ‘‘polished glass’’ areas, localized to the posteriorbasal segments of inferior lobes. Bronchoscopy revealed the presence of multiple calcifications in the tracheobronchial cartilages, with normal mucosal surface, extending also to the larger bronchial ramifications and the carina (Fig. 1, upper and lower panels). Cultural examination and cytology of bronchoalveolar lavage were negative. The bronchoscopic picture was self-explaining, since the presence of sessile bony or cartilagineous nodules of the tracheal wall is the hallmark of tracheopatia osteoplastica. Tracheopatia osteoplastica (or tracheopatia osteochondroplastica) (TO) is an uncommon disease of the upper airways, characterized by the presence of multiple cartilaginous or bony projections into the tracheobronchial lumen [2]. It usually affects people aged [50 years, but incidence and prevalence in the general populations are unknown, since the great majority of cases are diagnosed only post-mortem [3]. The cause is unknown, and more often this disease is asymptomatic. However, the most frequent symptoms at presentation are chronic cough (54% of cases), sputum production (34%), and hemoptysis (20%) [2]. Computerized tomography may sometimes reveal tracheal soft tissue masses or beaded calcification in the tracheobronchial cartilages, although calcifications, luminal stenosis, wall thickening, and nodules need for differential diagnosis with other uncommon diseases involving the central airways, i.e., Wegener’s granulomatosis, relapsing polychondritis, amyloidosis, sarcoidosis, and tuberculosis. Thus, bronchoscopy remains the main diagnostic tool: nodules are most frequently distributed along the proximal tracheobronchial tree, especially in the upper and middle S. Savelli M. L. Grata A. Ricci S. Gamberini R. Manfredini (&) U.O. di Medicina Interna, Ospedale del Delta, Azienda U.S.L. di Ferrara, Ferrara, Italy e-mail: r.manfredini@ausl.fe.it

Relapsing Polychondritis

Relapsing Polychondritis

Diffuse Tracheobronchial Thickening

Abstract:Relapsing polychondritis is a rare inflammatory disorder of cartilaginous and proteoglycan-rich tissues, commonly affecting the nasal and auricular cartilages, joints, tracheobronchial cartilage, cardiovascular system, as well as a number of other organ systems. The etiology of relapsing po

Characteristics of airway involvement in relapsing polychondritis

To investigate the characteristics of airway involvement in relapsing polychondritis (RP). The clinical data, including clinical manifestations, respiratory function test, computerized tomography (CT), and bronchoscopy of 38 out of the 56 RP patients who had airway involvement, 20 males and 18 females, with the mean onset age of 45 +/- 11 (27 - 71), and 3 RP patients without airway involvement were retrospectively analyzed. Three patients out of the 16 RP patients who did not have respiratory involvement but underwent respiratory function test, CT, and bronchoscopy were used as controls. The symptoms of airway involvement included cough, expectoration, hoarseness, feeling of suffocation, asthma, and dyspnea. Obstructive disturbance of ventilation was found in 10 and mixed disturbance of ventilation was seen in 2 of the 18 RP patients with airway involvement. The forced expiratory volume in one second, ratio of forced expiratory volume in one second to forced vital capacity, peak expiratory flow, FEF50/FIF50, and maximal mild-expiratory flow of the patients with airway involvement were 1.4 L +/- 0.23 L, 46.0 +/- 4.86, 3.3 L/s +/- 0.67 L/s, 0.3 +/- 0.08, and 0.4 L/s +/- 0.18 L/s respectively, all significantly lower than those of the RP patients without airway involvement (2.5 L +/- 0.09 L, 83.7 +/- 2.24, 6.9 L/s +/- 0.52 L/s, 1.3 +/- 0.51, and 2.8 L/s +/- 0.73 L/s, all P = 0.01). Flow volume loop showed remarkable decrease of PEF and formation of a plateau in the expiratory phase in the RP patients with airway involvement. CT performed in 27 RP patients with airway involvement showed trachea stenosis in 11, and thickened airway wall in 8 of them. Bronchoscopy performed in 23 patients with airway involvement showed inflammation in 16, destruction of tracheobronchial cartilage in 6, collapsed tracheobronchial wall in 7, tracheal stenosis in 15, left major bronchial stenosis in 13, and right major bronchial stenosis in 12 of them. Respiratory function test is sensitive in early detection of airway involvement in RP. Bronchoscopy and CT are useful in evaluation of the severity of airway involvement in patients with RP.

Anaesthetic Management of a Patient with Relapsing Polychondritis Undergoing Laparoscopic Surgery

We describe the anaesthetic management of a patient with relapsing polychondritis who underwent laparoscopic cholecystectomy. We failed to secure a patent airway with a ProSeal laryngeal mask airway, probably because of the deformity of the larynx. The glottis was small and it was only possible to pass a 5.5 mm cuffed endotracheal tube into the trachea. Positive pressure ventilation with 5 cm H2O positive end-expiratory pressure and surgery were safely performed. In relapsing polychondritis, recurrent inflammation and destruction of laryngeal and tracheobronchial cartilage causes airway obstruction, and various sizes of tracheal tubes and other airway manipulation devices should be prepared.

Relapsing polychondritis.

Relapsing polychondritis (RP) is a rare multisystem autoimmune disease of unknown origin characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, vertebral fibrocartilage and tracheobronchial cartilage, as well as proteoglycan-rich structures of the eye, heart, blood vessels or inner ear may all be affected. In most patients RP manifests in a fluctuating but progressive course which eventually results in a significant shortening of life expectancy. The relatively uncommon occurrence, the unknown etiopathogenesis, the ambiguous clinical pattern, as well as the variety in its course and response to therapy may all contribute to the difficulties the physician must overcome when managing RP. Beside describing the main features of RP and seven clinical cases of our own, in the present review we focus on recent findings in the etiopathogenesis and novel treatment options.

Endobronchial Ultrasonography in the Diagnosis and Treatment of Relapsing Polychondritis With Tracheobronchial Malacia

Relapsing polychondritis (RP) with tracheobronchial involvement has a poor prognosis, and a delay in diagnosis increases morbidity and mortality; however, the diagnosis is difficult to make. Endobronchial ultrasonography (EBUS) revealed changes in the tracheobronchial cartilage in two patients who met the criteria for RP, and facilitated the diagnosis. In these cases, EBUS revealed a poorly defined bronchial wall structure with two patterns of cartilaginous damage: fragmentation and edema. These cases were successfully treated by the implantation of nitinol stents, the sizes of which were determined by EBUS. EBUS was found to be useful in the diagnosis and treatment of RP.

A lethal osteochondrodysplasia with mesomelic brachymelia, round pelvis, and congenital hepatic fibrosis: two siblings born to consanguineous parents

We report a hitherto unknown, lethal osteochondrodysplasia in two Japanese siblings born to consanguineous parents. The skeletal abnormalities are characterised by mesomelic brachymelia with bowed forearms, a round pelvis with shortened greater sciatic notches, an ossification defect of the pubic bones, and absence of ossification centers in the cervical vertebral bodies. The associated visceral anomalies comprised periportal fibrosis and cystic dysplasia of the intrahepatic bile ducts, pancreatic ductal ectasia, a simple renal cyst, microcephaly with multifocal laminar necrosis and ectopic gray matter, dysplastic tracheobronchial cartilage, abnormal lobulation of the lung, diaphragmatic hernia, and stenotic pulmonary valve. Thrombocytopenia was present but megakaryocytes were slightly increased in the bone marrow. The patients showed various dysmorphic features including aniridia, a long palpebral fissure, prominent nasal bridge, beaked nose, flat philtrum, low-set fleshy ears, micrognathia with submucosal cleft palate, and multiple joint contractures.

Granular cell tumours of the lower respiratory tract.

Granular cell tumours rarely involve the lower respiratory tract. We report eight cases surgically resected at our institution. There were four females and four males, aged between 18 to 56 years (mean 40). One tumour associated with a peripheral lung adenocarcinoma was asymptomatic. The other lesions presented with obstructive pneumonitis (3 cases), haemoptysis (2), dyspnea (1) or cough (1). These tumours were tracheal (1) or bronchial (6) and one case was located in the lung parenchyma. Four cases were multicentric with associated lesions located in a bronchus (2), the oesophagus (1) or a mediastinal lymph node (1). All tumours, with the largest diameter ranging from 0.5-4.5 cm, were histologically invasive. The tumours were positive for S-100 protein, neuron specific enolase, KP1 (CD68) and vimentin. No tumour expressed desmin, keratin or p53 oncoprotein. Our study demonstrates that, in spite of marked anatomical and clinical polymorphism, the rare granular cell tumours of the lower respiratory tract have a constant histological appearance. Our observations confirm that large tumours (> 8-10 mm) usually extend beyond the tracheo-bronchial cartilages and, therefore, only surgical treatment may avoid recurrence.

Tracheopathia Osteoplastica: Familial Occurrence

Tracheopathia osteoplastica is an unusual disease characterized by cartilaginous or bony projections into the tracheobronchial lumen, with sparing of the posterior membranous portion of the tracheobronchial tree. The cause of this disorder is unknown. The diagnosis is seldom made because of the chronic and asymptomatic nature of the condition. More than 90% of the cases are diagnosed at postmortem examination. Symptoms may include dyspnea, coughing, hemoptysis, hoarseness, and wheezing. Tomography of the trachea may reveal beaded calcification of the tracheobronchial cartilages. Bronchoscopy is diagnostic. Histologically, the abnormal growths show heterotopic bone formation. No known treatment is available. We describe two female patients, one with hemoptysis and another with asthmalike symptoms, in both of whom bronchoscopy established the diagnosis of tracheopathia osteoplastica. The first patient had recurrent episodes of pneumonia attributable to bronchial obstruction by bony projections. In the second patient, removal of large lesions that obstructed the upper part of the trachea relieved the dyspnea. Of interest is the fact that the first patient was the biologic mother of the second. To our knowledge, this constitutes the first report of familial occurrence of tracheopathia osteoplastica.

Primary Tracheomalacia

Tracheomalacia is a rare congenital malformation of the tracheobronchial cartilages in which the supporting cartilaginous rings permit expiratory collapse of the airway. The condition is usually mild and self-limited. There is a severe variant, however, that is life-threatening and warrants separate categorization. Four children with severe primary tracheomalacia were treated recently. The clinical symptoms, diagnostic findings, and eventual treatment of these patients were highly distinctive and almost identical in all 4, permitting us to make the following observations: (1) primary severe tracheomalacia must be suspected in infants with unexplained respiratory distress manifested by stridor and cyanosis; (2) symptoms are not present at birth but appear insidiously after the first weeks of life, are markedly aggravated by respiratory tract infections, and are made worse by agitation; (3) bronchoscopy is essential for definitive diagnosis and should be employed early in the diagnostic process; (4) tracheostomy is probably essential in most instances; and (5) resolution, although spontaneous, does not occur until after 2 years of age.

The campomelic syndrome: Review, report of 17 cases, and follow‐up on the currently 17‐year‐old boy first reported by Maroteaux et al in 1971

We report 17 cases of the campomelic syndrome (CS) and a follow-up of one of the original patients of Maroteaux et al who is now 17 years old. Our review is based on 97 patients, including our own. An infant with the CS presents at birth with spectacularly short and bowed femora and tibiae. The initial chest radiograph confirms the diagnosis by demonstrating extremely small bladeless scapulae and hypoplastic pedicles of many thoracic vertebrae. Ossification of the sternal segments, pubis, talus, and knee epiphyses is also retarded. Usually the hips are dislocated and talipes equinovarus deformities are present. There is a small chondrocranium and a disproportionately large neurocranium. The bell-shaped chest, narrow superiorly, does not explain the degree of respiratory distress that soon ensues. Narrow airways from defective tracheo-bronchial cartilage can often be demonstrated on the radiograph, but micrognathia, retroglossia, cleft palate, hypoplastic lungs, and even CNS-based hypotonia contribute to the respiratory problem. Internal anomalies include frequent absence of olfactory bulbs and tracts and dilatation of cerebral ventricles, heart defects (PDA, VSD, stenosis of aortic isthmus), hydroureter and hydronephrosis, renal hypoplasia, renal hypoplasia, and rarely renal cysts.