Predictors of response to endoscopic management of subglottic/tracheal stenosis in patients without tracheostomy.

INTRODUCTION: Subglottic and tracheal stenosis (SGTS) in adults is an acquired or idiopathic condition that can lead to dyspnea, and even life-threatening airway obstruction. Endoscopic techniques have advanced and largely eclipsed open surgery, with open surgery now reserved for refractory cases (Hseu et al., 2013; Feinstein et al., 2017). Currently, there is no accepted guideline for the endoscopic treatment of SGTS. Thus, the aim of the present study is to examine the impact of various clinical and pathological characteristics on outcomes to endoscopic treatment in a cohort of SGTS patients. DISCLOSURE: None of the authors have any financial or personal relationship that could cause a conflict of interest regarding this article. METHODS: Retrospective chart review was performed for 41 patients presenting with SGS without a tracheostomy over a 4-year-period (2018-2022), within a single tertiary care center. Quantitative outcomes including number of dilation procedures undergone and need for open procedures were examined. The qualitative variables included a history of pulmonary disease, prior tracheostomy/tracheal resection, presence of tracheomalacia, granulation tissue, excessive dynamic airway collapse (EDAC), and etiology of idiopathic subglottic stenosis. RESULTS: The presence of granulation tissue seen on tracheoscopy was associated with a higher number (4+) of dilation procedures (p = 0.01). A history of pulmonary disease (p = 0.037), the presence of tracheomalacia (p = 0.039), and the presence of granulation tissue (0.003) were all associated with a need for open procedures. CONCLUSION: Patients with the presence of granulation tissue, tracheomalacia, and a history of pulmonary disease were more associated with more severe disease requiring either a higher number of endoscopic procedures or need for open procedures.

The voice and swallowing profile of adults with laryngotracheal stenosis before and after reconstructive surgery: A prospective, descriptive observational study.

OBJECTIVES: Airway reconstruction for laryngo tracheal stenosis (LTS) improves dyspnoea. There is little evidence relating to impact upon voice and swallowing. We explored voice and swallowing outcomes in adults with LTS before and after reconstructive surgery. DESIGN: Outcome measures were collected pre-reconstructive surgery, two-weeks post-surgery and up to 4-6 months post-surgery. SETTING: Tertiary referral centre. PARTICIPANTS: With ethical approval, twenty consecutive adult (≥18 years) LTS patients undergoing airway reconstruction were prospectively recruited. MAIN OUTCOME MEASURES: These included physiological values (maximum phonation time (MPT) and fundamental frequency; penetration aspiration score, residue score), clinician-reported (GRBAS, functional oral intake score, 100ml Water Swallow Test) and patient-reported outcomes (Voice Handicap Index-10, Reflux Symptoms Index, Eating Assessment Tool, Dysphagia Handicap Index). RESULTS: The observational study identified patient-reported and clinician-reported voice and swallow difficulties pre- and post-surgery; median and interquartile range are reported at each time point: Voice Handicap Index-10 23 (8-31); 20.5 (9-33.5), 24.5 (12.5-29); Dysphagia Handicap Index 9 (0-37); 13 (7-44); 15 (4-34); GRBAS grade 1(1-2); 2 (1-2.5); 2(1-2); 100ml Water Swallow Test volume score 16.7 (11.1-20); 14.3 (12.5-16.7); 16.7 (14.3-20.0); 100ml Water Swallow Test capacity score 16.3 ± 9.0; 11.0 ± 4.1; 12.5 ± 2.6. CONCLUSIONS: We present the first prospective data on voice and swallowing outcomes in adults with LTS before and after reconstructive surgery. The variability of the outcomes was higher than expected but importantly, for many the voice and swallow outcomes were not within normal limits before surgery. The clinical value of the study demonstrates the need for individual assessment and management of LTS patients' voice and swallowing.

Endoscopic management of adult subglottic stenosis: an alternative to open surgery.

PURPOSE: Various surgical techniques could be used to treat subglottic stenosis (SGS). The aim of this study is to present our experience in endoscopic management of SGS and show the impact of symptoms' evaluation, clinical examination and spirometry in the therapeutic decision. METHODS: Endoscopic treatment was performed in patients referred for SGS and consisted of CO2 scar lysis associated with balloon dilation and concomitant steroids' injection for patients with grade II or higher on the mMRC (modified Medical Research Council) dyspnea scale associated with a DI (Dyspnea Index) score higher than 10/40 and objective stenosis equal or higher than grade II. The preoperative DI score, EDI (expiratory disproportion index) and voice parameters were compared to postoperative results. The mean interval between endoscopic procedures (IEP) was calculated and we looked for the evolution of the IEP during repeated procedures. RESULTS: Nineteen patients were included. 35 dilations were performed. The mean IEP was 86 weeks. There was a significant decrease of the postoperative DI scores by 18.6 points ± 11 (SD). An important difference of 20.1 ± 13.5 (SD) was identified between the pre and postoperative EDI. Minimal changes occured in voice parameters. CONCLUSIONS: Endoscopic treatment with CO2 scar lysis associated with balloon dilation and concomitant steroids' injection is a safe, reliable and minimally invasive endoscopic procedure to treat SGS. Decision to treat has to be on an individual basis taking into account subjective symptoms including Dyspnea Index score and objective laryngoscopic findings and spirometry.

How can we identify subglottic stenosis in patients with suspected obstructive disease?

PURPOSE: Subglottic stenosis, a rare condition of the upper airway, is frequently misdiagnosed as obstructive lung disease. The aim of this study was to investigate whether subglottic stenosis could be identified and distinguished from asthma and chronic obstructive pulmonary disease (COPD) using spirometry or the dyspnea index (DI). METHODS: The study population included 43 patients with asthma, 31 patients with COPD and 50 patients with subglottic stenosis planned to undergo endoscopic intervention. All patients completed the DI and underwent dynamic spirometry registering both inspiratory and expiratory volumes and flows, including the expiratory disproportion index (EDI), the ratio of forced expiratory volume in 1 s to peak expiratory flow. One-way analysis of variance assessed the discrepancy of the variables among the study groups, and receiver operating curve (ROC) analysis determined the measurement with the best discriminatory power providing a cutoff value, maximizing both sensitivity and specificity. RESULTS: The only statistically significant variables differing between all three groups were the EDI and the DI. The EDI showed an excellent area under the ROC curve (0.99, p < 0.001) with a cutoff value of 0.39 (98% sensitivity, 96% specificity), followed by DI (0.87, p < 0.001) with a cutoff score of > 25 (83% sensitivity and 78% specificity). CONCLUSION: In patients with dyspnea of unknown cause, an increase in EDI should arouse a suspicion of extrathoracic airway obstruction, advocating for further evaluation with laryngotracheoscopy.

Not Just Dyspnoea: Swallowing as a Concern for Adults with Laryngotracheal Stenosis Undergoing Airway Reconstruction.

Acquired laryngotracheal stenosis (LTS) is a rare condition causing dyspnea and stridor. Patients often require multiple surgical procedures with no guarantee of a definitive outcome. Difficulty swallowing is a recognised problem associated with LTS and the reconstructive surgeries required to manage the condition. The breathlessness patient's experience impacts on swallowing, and the vulnerable structures of the larynx are implicated during complex surgeries. This leads to dysphagia post-surgery, with some patients experiencing more chronic symptoms depending on the biomechanical impact of the surgery, or a pre-existing dysphagia. Despite this there is limited observational research about the dysphagia associated with LTS, with no exploration of the patient experience. Our aim was to investigate patient experience of living with LTS focussing on dysphagia in order to guide clinical practice. A qualitative study was completed using focus groups and semi-structured interviews with 24 patients who have had reconstructive surgery for LTS. Thematic analysis was used to identify three over-arching themes: The Physical Journey, The Emotional Journey and The Medical Journey. Key sub-themes included the importance of self-management and control, presence of symptoms, benefits of therapy, living with a life-long condition, fear and anxiety, autonomy, medicalisation of normal processes and the dichotomy between staff expertise and complacency. Swallowing was connected to all themes. The results are reviewed with consideration of the wider literature of lived experience particularly in relation to other chronic conditions and those that carry a high symptom burden such as head and neck cancer. Future clinical and research recommendations have been made. Akin to other clinical groups, adults with LTS are keen that management of their swallowing is person-centred and holistic.

Subglottic cysts in preterm infants: superficially located cysts versus deeply buried cysts in the mucosa.

PURPOSE: To analyze the reason for the diversity of the clinical course of subglottic cysts and discuss their pre- and coexistence with subglottic stenosis. METHODS: The medical records of patients who were treated for subglottic cysts between 2003 and 2020 were retrospectively reviewed and direct laryngoscopy videos were analyzed to assess the healing patterns of their disease. RESULTS: Of the 15 patients, 10 had a history of intubation in the neonatal period. In 11 patients, the cysts were transparent and well defined, and no recurrence of subglottic cysts occurred after the initial surgery. In four patients, the cysts were located deep in the mucosa and did not have the typical appearance of a cyst, but rather of a stenotic segment; all of them had a history of intubation and three of them required laryngotracheal reconstruction. CONCLUSION: Transparent, thin-walled superficial subglottic cysts with healthy surrounding mucosa can easily be treated with endoscopic marsupialization; however, the treatment of deep subglottic cysts can be challenging. The coexistence of subglottic cysts and subglottic stenosis is not rare. We point out the need for considering the possibility of a missed deep submucosal cyst in a seemingly refractory case of pediatric subglottic stenosis with atypical endoscopic findings and with a background history of prior intubation.

Voice-related quality of life: Relation to objective spirometry measures in subglottic stenosis.

OBJECTIVES: The current study seeks to identify the correlation between in-office spirometry data and voice-related quality of life in patients with subglottic stenosis (SGS). METHODS: Patients with SGS of any etiology were included when in-office spirometric data was available in addition to voice-related patient-reported outcomes (PROM) data in the form of the Voice Handicap Index-10 (VHI-10) and/or the Voice-Related Quality of Life (V-RQOL) survey. Overall survey scores and individual question responses were assessed for degree of correlation to spirometric data. RESULTS: Twenty-nine patients were included in the final analysis. Overall mean total VHI-10 scores totaled 7.15 (SD 9.11), while mean overall V-RQOL scores totaled 78.41 (SD 16.45). Both PEF and PIF rates correlated to total scores on the VHI and V-RQOL surveys. This correlation was stronger with PIF than with PEF, and with the V-RQOL than with the VHI. Questions related to breathlessness most closely correlated with spirometric data. CONCLUSION: Voice-related QOL is impacted in patients with SGS in a predictable way. Breathlessness while speaking may be more impactful than inability to produce speech in this population.

Developmental delay, intellectual disability, short stature, subglottic stenosis, hearing impairment, onychodysplasia of the index fingers, and distinctive facial features: A newly reported autosomal recessive syndrome.

We report on a multiply consanguineous Syrian family where two siblings, a boy and a girl, presented with a compilation of symptoms including developmental delay, severe intellectual disability, absent speech, hearing impairment, short stature, subglottic stenosis, increased length of the palpebral fissures, onychodysplasia of index fingers, scoliosis, genu valgum, and malpositioned toes. Two other individuals from the extended family with similar clinical features are also described. Array-CGH did not reveal any pathological copy number variation. Exome sequencing failed to find any causal variants. Differential diagnoses and the possibility that we might be reporting a hitherto unknown syndrome are discussed.

An Adolescent With Progressive Shortness of Breath.

Shortness of breath and wheezing are common presenting signs for children in the emergency department. In adolescence, it is often due to asthma or lower respiratory tract infections. We present a rare pediatric case of an adolescent with biphasic stridor and progressive exercise-induced shortness of breath who was found to have severe idiopathic subglottic stenosis.

Vocal fold fixation due to proximal stenosis progression complicating idiopathic subglottic stenosis.

PURPOSE: This study examined the incidence and risk factors for vocal fold fixation due to proximal progression of idiopathic subglottic stenosis (ISS) over the course of serial treatments. METHODS: Records of 22 consecutive patients with ISS treated between 2004 and 2016 were retrospectively reviewed. Patient, stenosis, and treatment details were recorded. Cox regression was used to identify independent predictors of vocal fold fixation. RESULTS: All patients were female and mean age at diagnosis was 46 ± 7 years. In five patients, the stenosis was within 15 mm of the glottis at first treatment. Vocal fold fixation due to proximal stenosis progression occurred in seven (32%) patients. It led to permanent hoarseness due to unilateral vocal fold fixation in two patients and caused airway compromise due to bilateral vocal fixation in two other patients. No airway-related deaths occurred and no patient required a tracheostomy. Stenosis incision using coblation or potassium titanyl phosphate laser, and an initial glottis-to-stenosis (GtS) distance < 15 mm significantly increased the risk of proximal stenosis progression on univariable analysis. CONCLUSION: Vocal fold fixation due to proximal stenosis progression is a significant complication of idiopathic subglottic stenosis and causes permanent voice and/or airway sequelae. It should be actively looked for and documented every time a patient is assessed. If a reduction in the GtS distance is observed, definitive surgery should be promptly considered before proximal stenosis progression compromises the efficacy and safety of definitive treatment or, worse, causes vocal fold fixation.

Rigid dilatation of pediatric laryngotracheal stenosis as an adequate alternative to balloon dilatation.

INTRODUCTION: Endoscopic balloon dilation (EBD) is the mainstay of endoscopic therapy for laryngotracheal stenosis (LTS), although there is no evidence that it achieves better results than traditional rigid laryngeal dilators. Rigid bougie dilators are less expensive and easier to use, and confer the advantage of providing tactile information about the stenosis to the surgeon. We analyzed the outcome of endoscopic rigid bougie dilatation of LTS in a large series of children and compared it to the reported results of EBD in the same setting. PATIENTS AND METHODS: All cases of pediatric LTS treated by endoscopic rigid dilatation in a tertiary referral center between 2006 and 2015 were retrospectively studied. They were divided into a primary dilatation group (PDG) and a post-reconstruction dilatation group (PRG). The PDG children had no history of reconstructive airway surgery, and dilatation was the major treatment approach. The PRG children underwent dilatations after airway reconstruction surgery as part of routine postoperative management. A successful primary outcome was defined as improvement of dyspnea and achievement of a functional airway without reconstructive laryngotracheal surgery or need for a tracheostomy at final follow-up. RESULTS: Sixty-two children (68 cases, mean age 5.1 years, range 0.7-17.2) underwent 156 endoscopic rigid dilatations. Successful outcome was achieved in 48 cases (70.6%), 73.0% in the PDG and 67.7% in the PRG. There were no procedure-related adverse events. CONCLUSIONS: Endoscopic rigid dilatation is a relatively inexpensive and efficacious tool in endoscopic management of pediatric LTS. Its success rates are in the same range as those of EBD.

Cross-sectional follow-up of voice outcomes in children who have a history of airway reconstruction surgery.

OBJECTIVES: This study reports vocal function in a cross-section of children with subglottic stenosis. Each child had a history of laryngotracheal reconstruction and/or cricotracheal resection surgery. Vocal function was measured using laryngoscopy, acoustic analysis, perceptual evaluation and impact of voice on quality of life. DESIGN: All patients aged >5 years with history of laryngotracheal reconstruction and/or cricotracheal resection surgery at the Scottish National Complex Airways service were invited to participate. SETTING: Data were gathered in the Royal Hospital for Children in Glasgow in a single outpatient appointment. PARTICIPANTS: Twelve of 56 former patients (aged 5-27) provided a voice sample and eleven consented to awake laryngoscopy. All consented for detailed evaluation of their medical records. MAIN OUTCOME MEASURES: Acoustic analysis of fundamental frequency and pitch perturbation was conducted on sustained vowel [a]. Perceptual evaluation was conducted by 4 trained listeners on a series of spoken sentences. Impact on quality of life was measured using the paediatric voice-related quality of life questionnaire. Laryngeal function was descriptively evaluated. RESULTS: Four children had normal voice acoustically, perceptually and in relation to voice-related quality of life. One of these had vocal fold nodules unrelated to surgical history. Two other children had "near normal" vocal function, defined where most voice measurements fell within the normal range. CONCLUSIONS: Normal or "near normal" voice is a possible outcome for children who have had this surgery. Where there is an ongoing complex medical condition, voice outcome may be poorer.

Common causes of congenital stridor in infants.

Stridor is a high-pitched respiratory sound that signals upper airway obstruction. It can be encountered by clinicians in a variety of clinical settings and requires a team-based, interdisciplinary approach. Early recognition is crucial, as the differential diagnosis can be broad, and causes range from benign to life-threatening. This article reviews the most commonly encountered causes of chronic congenital stridor in infants, focusing on the diagnostic approach, pathophysiology, clinical presentation, and management strategies.

Granulomatosis with polyangiitis-associated acute subglottic stenosis in a 13-year-old boy: a case report.

We present a case of a child with granulomatosis with polyangiitis, admitted with acute respiratory distress attributed to subglottic stenosis. The anesthetic management and potential complications are described.

Effects of Balloon Dilation for Idiopathic Laryngotracheal Stenosis on Voice Production.

OBJECTIVE: To evaluate the effects of balloon dilation for idiopathic laryngotracheal stenosis on voice production. METHODS: Retrospective review of 10 female patients with idiopathic laryngotracheal stenosis undergoing balloon dilation. Voice outcomes were evaluated by comparing pre- and posttreatment patient-reported, perceptual, aerodynamic, and acoustic parameters. Complete data sets were not available for all subjects; sample size for each parameter is reported with the corresponding result. RESULTS: Total Voice Handicap Index (VHI) decreased significantly (22.9 ± 13.5 to 6.8 ± 6.5; n = 8; p = .015), as did glottal function index (7.2 ± 4.9 to 1.5 ± 2.0; n = 6; p = .022). No changes were observed in the GRBAS (grade, roughness, breathiness, asthenia, strain) scale. Changes in aerodynamic parameters were not statistically significant. Percent jitter decreased (1.32 ± 1.37 to 0.60 ± 0.29; n = 7; P = .078), and fundamental frequency range was preserved (507 ± 325 to 612 ± 281; n = 7; P = .309). CONCLUSIONS: Our sample of patients with idiopathic laryngotracheal stenosis had a mild-moderate dysphonia that improved with balloon dilation. Importantly, adverse effects on voice that can occur with open procedures were not observed. Patient perception of dysphonia improved while fundamental frequency range was maintained and aerodynamic parameters remained within or moved toward the normal range. Larger prospective studies are warranted to further evaluate changes in voice production associated with balloon dilation.

Noninvasive Treatment of Acquired Subglottic Stenosis.

A 23-day-old infant referred to the neonatal intensive care unit with difficulty breathing and inspiratory stridor increasing with exercise. Medical history included a cardiothoracic surgery for transposition of the great arteries, patent ductus arteriosus, atrial septal defect, and a history of intensive care unit from surgery. Flexible fiberoptic transnasal laryngoscopy revealed subglottic stenosis that was probably caused by prolonged intubation with a higher airway pressure. Computed tomographic scan of the neck showed a tiny stenosis without cartilage deformity and limited in subglottic region. Tracheotomy and external open surgery was found risky for sternal wound infection or mediastinitis as the neonate had newly thoracotomy. The patient had a balloon dilation under general anesthesia without intubation. Presenting symptoms of the neonate were fully improved with balloon dilation.

Optimal Management of a 2-Hour-Old Newborn With Severe Congenital Subglottic Stenosis and Multiple Congenital Heart Diseases.

INTRODUCTION: Congenital subglottic stenosis (C-SGS) is the third most common congenital anomaly of the larynx. It necessitates tracheotomy in newborns if it causes severe airway obstruction. When the negative impact of tracheostomy cannot be tolerated, as in presence of congenital heart disease requiring further surgical intervention, other alternative procedures are required. This case report is the first of its kind reporting airway expansion in a neonate in the first few hours after birth. CASE REPORT: A 38-week-fetus male was born with multiple congenital heart anomalies and C-SGS of grade III. Single-stage laryngotracheoplasty (SS-LTP) with anterior thyroid alar cartilage (TAC) grafting was performed. Our team was fully aware of all potential risks during SS-LTP procedure in such debilitated patient. DISCUSSION: Treatment of C-SGS in premature neonates is tracheostomy to avoid prolonged intubation. In some cases, tracheostomy is not a good option as in presence of congenital heart disease necessitating urgent further surgical interventions. Among all surgical procedures to augment the airway without tracheostomy, SS-LTP with placement of anterior TAC graft was our choice. CONCLUSION: This case report demonstrated that SS-LTP with anterior TAC graft can be performed in a newborn with severe C-SGS and congenital heart disease. It can alleviate the need for tracheostomy and avoid unnecessary delay for subsequent cardiac interventions. However, further study is likely needed to make a definitive statement of its safety and efficacy.

A Novel Therapy for Laryngotracheal Stenosis: Treatment With Ethosomes Containing 5-Fluorouracil.

PURPOSE: The purpose of our article was to explore the effect of ethosomes containing 5-fluorouracil (5-FU) with different sizes on laryngotracheal stenosis treatment. METHODS: The physical characteristics of ethosomes containing 5-FU were investigated, including size, shape, and entrapment percentage. The effect of ethosomes containing 5-FU was evaluated on the airway stenosis rabbit model. The formation of fibrous/scar tissue was investigated by hematoxylin and eosin (HE) staining, and the permeation depth was observed under fluorescence microscope. RESULTS: The mean sizes of 5-FU ethosomes extruded by D=50 nm and D=100 nm pore were 60±10 nm and 110±13 nm, respectively. The 5-FU entrapment percentage of ethosomes was determined to be 15% (D=60±10 nm) and 32% (D=110±13 nm). After being treated by ethosomes containing 5-FU (D=60±10 nm), the fibroblast and collagenous fiber distributed sparsely in the deep scar tissue. The permeation capability of ethosomes containing 5-FU (D=60 nm) was significantly better than ethosomes (D=110 nm). Besides, the 5-FU ethosomes resulted in less stenosis than 5-FU only. CONCLUSIONS: Topical administration of 5-FU ethosomes may be a novel candidate therapy for laryngotracheal stenosis treatment.

Safety of outpatient airway dilation for adult laryngotracheal stenosis.

OBJECTIVES: To evaluate the safety of outpatient airway dilation for adult patients with subglottic or tracheal stenosis. METHODS: The records of patients treated with airway dilation between October 2003 and September 2013 were reviewed. Outcomes of patients who underwent dilation as inpatients versus outpatients were compared. Emergency room visits, readmissions, and 3 or more primary care physician visits within 30 days postoperatively were specifically evaluated. Postoperative hemorrhage, airway edema, recurrent laryngeal nerve paralysis, reintubation, tracheostomy, tracheal rupture, pneumomediastinum, pneumothorax, acute respiratory distress, or death were also reviewed. RESULTS: One hundred fourteen dilations performed in 53 patients with airway stenosis were included. Outpatient dilation was performed in 93 (82%); 21 (18%) underwent the procedure in the inpatient setting. Complications were low among both inpatient and outpatient groups (10% vs 1%, P=.09). No complications occurred during the overnight stay of the inpatient group. CONCLUSIONS: Outpatient airway dilation is a safe and feasible procedure. It can be routinely performed on an ambulatory basis.

Voice outcomes after endoscopic treatment of laryngotracheal stenosis.

OBJECTIVE: This study aimed to assess vocal disability in patients with laryngotracheal stenosis who are managed with endoscopic surgery, comparing patients with posterior glottic stenosis (PGS) to those with subglottic or tracheal stenosis (SGS/TS). METHODS: Patients who underwent endoscopic treatment for laryngotracheal stenosis with voice outcomes data from 2005 to 2013 were studied. The mean Voice Handicap Index-10 (VHI-10) was compared over the study period. Of those with SGS/TS, the distance from the vocal folds to the proximal portion of the stenosis was obtained and compared to VHI-10. RESULTS: Forty-four patients met inclusion criteria. The mean VHI-10 for all patients was 14.6. Fifty percent were dysphonic (VHI-10>11). The mean VHI-10 for PGS patients was 22.4, and for SGS/TS patients, 10.9 (P=.004). Of those with PGS, 78.6% were dysphonic compared to only 36.7% of those with SGS/TS. Voice Handicap Index-10 improved from 14.1 for those with proximal stenosis to 4 for those with stenosis more than 2 cm distal to the vocal folds. CONCLUSION: Following endoscopic management of stenosis, those with PGS have poorer voice outcomes compared to those with SGS/TS. The majority of those with SGS/TS are not dysphonic. Vocal outcomes are greatest for those with stenosis beginning at least 2 cm distal to the vocal folds.