Tracheal lipomas, though exceedingly rare among benign tracheal masses, present unique diagnostic and management challenges due to their unusual clinical course. This case report documents the presentation and surgical management of a 56-year-old male with a tracheal lipoma, a first documented case in Puerto Rico. The patient presented with progressive dyspnea and stridor, initially misdiagnosed and treated as asthma exacerbations. Imaging confirmed a pedunculated tracheal mass, prompting emergency surgical intervention to secure the airway and excise the mass successfully. Histopathological analysis confirmed the mass as a benign tracheal lipoma. This case emphasizes the importance of considering rare tracheal tumors in the differential diagnosis of unexplained respiratory distress and advocates for tailored management strategies informed by comprehensive multidisciplinary approaches.

Broncholithiasis due to pulmonary histoplasmosis causing central airway obstruction and broncho-mediastinal fistula is a rare complication in the pediatric population. A 16-year-old previously healthy female was referred to a university hospital for worsening cough and shortness of breath for over a two-year period. Radiologic investigation revealed a calcified subcarinal lymph node eroding into the left mainstem bronchus causing central airway obstruction and collapse of the left lower lobe. Direct laryngoscopy and bronchoscopy showed a large obstructive lesion in the left mainstem bronchus. Debulking of the endobronchial lesion was performed with neodymium-doped yttrium aluminum garnet (Nd:YAG) laser and cryotherapy. Pathology examination was consistent with broncholith. Great clinical and radiological response to the procedure was evident with complete re-expansion of the left lung and resolution of symptoms.

Rosai-Dorfman disease (RDD) is a rare condition that causes massive lymphadenopathy, most commonly in the cervical area. Cowden syndrome (CS) causes hamartomas in the skin and mucosa and predisposes individuals to various malignancies. Lhermitte-Duclos disease (LDD), or dysplastic cerebellar gangliocytoma, is often associated with CS. A 41-year-old female with all three conditions presented with abnormal uterine bleeding and endometrial intraepithelial neoplasia (EIN). Precautions should be considered when evaluating patients with RDD and CS preoperatively and during airway management owing to the potential for multisystem involvement, anatomical distortion, and difficult airways. The likelihood of having all three conditions is extremely rare.

Bronchopulmonary dysplasia (BPD), a disorder characterized by arrested lung development, is a frequent cause of morbidity and mortality in premature infants. Parenchymal lung changes in BPD are relatively well-characterized and highly studied; however, there has been less emphasis placed on the role that airways disease plays in the pathophysiology of BPD. In preterm infants born between 22 and 32 weeks gestation, the conducting airways are fully formed but still immature and therefore susceptible to injury and further disruption of development. The arrest of maturation results in more compliant airways that are more susceptible to deformation and damage. Consequently, neonates with BPD are prone to developing airway pathology, particularly for patients who require intubation and positive-pressure ventilation. Airway pathology, which can be divided into large and small airways disease, results in increased respiratory morbidity in neonates with chronic lung disease of prematurity.

Subglottic stenosis (SGS) may result from prolonged intubation where fibrotic scar tissue narrows the airway. The scar forms by differentiated myofibroblasts secreting excessive extracellular matrix (ECM). TGF-β1 is widely accepted as a regulator of fibrosis; however, it is unclear how biomechanical pathways co-regulate fibrosis. Therefore, we phenotyped fibroblasts from pediatric patients with SGS to explore how key signaling pathways, TGF-β and Hippo, impact scarring and assess the impact of inhibiting these pathways with potential therapeutic small molecules SB525334 and DRD1 agonist dihydrexidine hydrochloride (DHX).
Laryngeal fibroblasts isolated from subglottic as well as distal control biopsies of patients with evolving and maturing subglottic stenosis were assessed by α-smooth muscle actin immunostaining and gene expression for α-SMA, FN, HGF, and CTGF markers. TGF-β and Hippo signaling pathways were modulated during TGF-β1-induced fibrosis using the inhibitor SB525334 or DHX and analyzed by RT-qPCR for differential gene expression and atomic force microscopy for ECM stiffness.
SGS fibroblasts exhibited higher α-SMA staining and greater inflammatory cytokine and fibrotic marker expression upon TGF-β1 stimulation (p < 0.05). SB525334 restored levels to baseline by reducing SMAD2/3 nuclear translocation (p < 0.0001) and pro-fibrotic gene expression (p < 0.05). ECM stiffness of stenotic fibroblasts was greater than healthy fibroblasts and was restored to baseline by Hippo pathway modulation using SB525334 and DHX (p < 0.01).
We demonstrate that distinct fibroblast phenotypes from diseased and healthy regions of pediatric SGS patients respond differently to TGF-β1 stimulation, and SB525334 has the superior potential for subglottic stenosis treatment by simultaneously modulating TGF-β and Hippo signaling pathways.
NA Laryngoscope, 134:287-296, 2024.

Bilateral vocal cord paralysis is a potentially life-threatening condition, depending on the position in which the vocal cords are paralyzed. When the vocal cords are fixed in adduction, patients develop respiratory distress, inspiratory stridor, aspiration, and minimal phonation deficits. This condition can result from acute injuries to the right and left recurrent laryngeal nerves, or from chronic bilateral recurrent laryngeal nerve palsy. The clinical presentation is variable with such nerve injuries. Traumatic injuries to the cervical spine are an uncommon cause of this condition. In this report, we describe a patient who developed progressive respiratory distress, inspiratory stridor, and dysphagia to liquids several weeks after suffering major trauma to the head and neck. Laryngoscopy revealed immobile bilateral vocal cords fixed in the paramedian position, resulting in severe airway obstruction that warranted an emergency tracheostomy.

In recent years, with increased survival of infants with severe bronchopulmonary dysplasia (BPD), long term ventilation due to severe BPD has increased and become the most common indication for tracheostomy in infants less than one year of age. Evidence shows that tracheostomy in severe BPD may improve short- and long-term respiratory and neurodevelopmental outcomes. However, there is significant variation among centers in the indication, timing, intensive care management, and follow-up care after hospital discharge of infants with severe BPD who received tracheostomy for chronic ventilation. The timing of liberation from the ventilator, odds of decannulation, rate of rehospitalization, growth, and neurodevelopment are all clinically important outcomes that can guide both clinicians and parents to make a well-informed decision when choosing tracheostomy and long-term assisted ventilation for infants with severe BPD. This review summarizes the current literature regarding the indications and timing of tracheostomy placement in infants with severe BPD, highlights center variability in both intensive care and outpatient follow-up settings, and describes outcomes of infants with severe BPD who received tracheostomy.

In class II malocclusion, there is an anteroposterior disparity between the upper dentition and the lower dentition, which may or may not be accompanied by a skeletal discrepancy. For orthodontists, this is one of the common malocclusions encountered during clinical practice. This might be due to excess maxillary growth or retarded growth of the mandible or a combination of both. In such types of malocclusion, both the upper and lower airways are affected, the lower one most commonly. Characteristic features seen are a narrow maxillary arch, a proclined upper anterior, and mouth breathing as a developing habit. Also, the position of the condyle in the skeletal type of class II malocclusion plays a vital role in the development of temporomandibular joint disorders. Treating such disparity in a growing individual leads to better results in the long term as well as prevention of malocclusion taking a severe form. Myofunctional appliances are useful for repositioning the mandible as well as the condyle. In adults, extraction of the upper premolars is most commonly done for the correction of class II malocclusion. This provides the patient with a better esthetic appearance. In addition to this, various treatment modalities, such as splint therapy, exercise, and prolotherapy, are beneficial for pain relief and temporomandibular disorder (TMD) correction. This article deals with the characteristics, development, etiology, and comprehensive treatment options of class II malocclusion and its co-relation with the upper and lower airway along with the severity of temporomandibular joint disorders. Repositioning of the condyle in the glenoid fossae is the key to the correction of this disorder.

Diffuse large B-cell lymphoma (DLBCL) is the most prevalent subtype of non-Hodgkin\'s lymphoma (NHL). This subtype can be present in various extranodal sites, including the brain, bones, intestines, kidneys, adrenal glands, and other soft tissues. As demonstrated in this case, one rare site of DLBCL is the nasal septum, which presents as a rapidly enlarging mass resistant to antibiotics and steroids. The definitive diagnosis for this case involved biopsy, but further workup, such as computed tomography (CT) and fluorescence in situ hybridization (FISH), helped support the diagnosis of DLBCL. While determining the stage of the lymphoma, treatment with R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) was initiated immediately. This case demonstrates a rare presentation of DLBCL in the nasal septum and describes the significance of urgent examination as well as treatment.

OBJECTIVE: Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients.
METHODS: We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis.
RESULTS: 249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention.
CONCLUSIONS: TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention.
METHODS: