BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory stridor.
METHODS: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient\'s breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient\'s respiratory and cardiovascular function were stable.
CONCLUSIONS: This report documents the unusual finding of severe inspiratory stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.

OBJECTIVE: The objective was to measure health-related quality of life (HRQoL) of children following treatment of all-cause tracheomalacia with aortopexy.
METHODS: Children ≥5 years and parents of children <18 years who had undergone aortopexy completed the Paediatric Quality of Life Inventory (PedsQL4.0). Scores were compared to published norms.
RESULTS: Completed questionnaires were received from 35 parents (65%) and 10 children (38%). Median age at aortopexy was 9.8 months (1 month-12.7 years) and median years of follow-up was 2.6 (4 months-6.9 years). Children who completed questionnaires had a median age of 8.4 (5.7-13.4) years. Parent and child-reported total PedsQL scores were 69.61 (SD : 19.74), and 63.15 (SD : 20.40) respectively. Half of parents and 80% of children reported scores suggesting poor HRQoL outcomes. Parent-reported total, physical and psycho-social scores were lower than those of healthy children and those with acute illness but comparable to children with chronic health conditions and cardiovascular disease. Similarly, children themselves reported comparable total scores to children with chronic illness but child-reported psycho-social scores were lower in the aortopexy group than any other group. There was no association between PedsQL scores and cause of malacia, age or time since aortopexy. The presence of complex congenital comorbidities had a significant (p < 0.05) impact on HRQoL scores.
CONCLUSIONS: Following aortopexy children remain at risk of poor HRQoL, especially those with complex comorbidities. HRQoL reported by both parent and child provides important insight into the lives of children following this procedure. Further longitudinal and qualitative study are required to better understand this complex group.

Introduction: Lower airway malacia (LAM) is characterized by a reduction in the cross-sectional luminal area during quiet respiration. There is no gold standard diagnostic test; however, flexible fiberoptic bronchoscopy (FFB) is most frequently utilized. The exact prevalence and incidence of LAM are unknown. This study aimed to determine the prevalence rates of pediatric patients diagnosed with LAM, offer a detailed understanding of their demographic and clinical characteristics, and investigate distinctions between two specific types of LAM, namely, tracheomalacia (TM) and bronchomalacia (BM). Materials and Methods: Patients younger than 18 years diagnosed with LAM using FFB were included in this retrospective case series. Demographic and clinical characteristics and comorbid disorders were compared between patients with isolated BM and those with isolated TM or tracheobronchomalacia (TM/TBM). Results: Among 390 patients who underwent FFB, 65 (16.6%) were diagnosed with LAM, 16 (24.6%) with TM, and 56 (86.2%) with BM. The median age at diagnosis was 15 months. Among them, 59 (90.8%) had other comorbidities; gastrointestinal (GI) disorders were the most common (38.5%). The most common indications for bronchoscopy were recurrent/prolonged lower respiratory tract infections (LRTI) or wheezing (43.1%), while the most frequently observed respiratory physical examination finding was stridor (35.4%). Patients with TM/TBM had significantly higher frequencies of premature births, stridor, retraction, and GI disorders. Conclusion: Patients with stridor without typical laryngomalacia features or recurrent or prolonged LRTI should undergo prompt evaluation for LAM. The potential coexistence of GI disorders such as gastroesophageal reflux disease and swallowing dysfunction should also be considered.

Relapsing polychondritis is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the cartilages involved. In addition to characteristic damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are particularly severe, and can seriously alter the prognosis. Tracheobronchial lesions are assessed by means of a multimodal approach, including dynamic thoracic imaging, measurement of pulmonary function (with recent emphasis on pulse oscillometry), and mapping of tracheal lesions through flexible bronchoscopy. Diagnosis can be difficult in the absence of specific diagnostic tools, especially as there may exist a large number of differential diagnoses, particularly as regards inflammatory diseases. The prognosis has improved, due largely to upgraded interventional bronchoscopy techniques and the development of immunosuppressant drugs and targeted therapies, offering patients a number of treatment options.

BACKGROUND: Biodegradable (BD) stents made from polydioxanone have been used only in human airways. These stents combine the advantages of classical tracheal stents, and fewer side effects are expected due to their biocompatibility and their time-limited presence in airways. However, new clinical consequences have arisen. Here, the authors share their experiences with BD stents for tracheal indications, focusing on their safety and efficacy.
METHODS: This was a retrospective review of a collected database of adult patients who underwent implantation of biodegradable tracheal stents between September 2013 and December 2022 at the Department of Respiratory Medicine of the 1st Faculty of Medicine in Prague and Thomayer University Hospital. The indications included functionally significant nonmalignant tracheal stenosis and tracheomalacia. Self-expandable, biodegradable, polydioxanone tracheal stents manufactured by ELLA-CS Ltd. (Hradec Kralove, Czech Republic) were implanted during rigid bronchoscopy under general anaesthesia. All patients were followed up in the department and were provided with the necessary care. The main efficacy and safety parameters and relationships were analysed using descriptive statistics and Fisher´s exact, Wilcoxon and Kruskal‒Wallis tests.
RESULTS: A total of 65 stents were implanted in 47 adult patients. During the first two months after implantation, when adequate function was expected, the stent was found to be effective in 26 out of 39 patients who completed this period (66.7%). The clinical effectiveness reached 89.7%, as early restenoses were mostly mild and necessitated treatment in only 4 patients. The frequencies of significant mucostasis, migration and granulation tissue growth were 2.6%, 7.5% and 23.1%, respectively, during this period. Thirty-four participants completed the half-year follow-up period after the first or second stent insertion, and some were followed up beyond this period. Poor control of symptoms, the development of restenosis and the need for interventions were characteristic of this period as the stents degraded. Twenty-two patients who experienced remodelling or stabilization of the tracheal lumen achieved a stent-free state. Seven patients underwent subsequent surgical treatment.
CONCLUSIONS: BD stents are safe and provide adequate tracheal support until they begin to degrade. The use of BD stents necessitates close monitoring of patients and accurate treatment of possible restenosis.
BACKGROUND: Based on project NT14146 - Biodegradable stents in the management of the large airways (2013-2015, MZ0/NT), registered on May 1, 2013, in the Research and Development and Innovation Information System of the Czech Republic and at ClinicalTrials.gov (reg. no. NCT02620319, December 2, 2015).

BACKGROUND: The aims of this study were to update our experience with biodegradable polydioxanone (PDO) airway stents in children, focusing on effectiveness and safety, and to analyze the factors involved in the different outcomes observed.
METHODS: Retrospective study of patients managed with PDO stents from 2012 to 2023. Variables collected: demographics, comorbidities, indication, clinical baseline, stent size, location, complications, clinical outcome, and time of follow-up. Statistical analyses were performed to detect the eventual contribution of variables in the different outcomes observed.
RESULTS: Fifty-four PDO stents were placed in 26 patients (median age, 4 months). All showed severe symptoms of central airway obstruction due to tracheomalacia in nine patients, bronchomalacia five, tracheobronchomalacia 10, and tracheal stenosis two. Stent placement was uneventful in every case: 29 stents in the trachea and 25 in the main bronchi. 53.8% of patients needed successive stenting, and all exhibited comorbidities. Complete clinical resolution was observed in eight cases (30.7%), partial improvement in 13 (50%), unchanged in 4 (15.3%), and worsened in one. Age had a significant positive impact on outcome (6 vs. 3 months; p = 0.024). Additionally, smaller stents were associated with a better outcome (20 vs. 26 mm; p = 0.044). Granulation tissue was the most frequent complication (34.6%). Five patients (19.2%) died due to severe comorbidities, follow-up was complete in survivors (median, 58 months).
CONCLUSIONS: PDO stents are safe and effective when dealing with severe tracheobronchial obstruction. Stent-related granulation tissue continues to be a relevant matter of concern. This issue, together with increased degradation times, deserves further research.

BACKGROUND: Several studies mentioned parenchymal findings after SARS-CoV-2 pneumonia, but few studies have mentioned alterations in the airways. The aim of this study was to estimate the prevalence of tracheomalacia and to analyse the clinical characteristics in a cohort of patients with SARS-CoV-2.
METHODS: The study population consisted of all patients with SARS-CoV-2 admitted a hospital serving a population of 500 000 inhabitants. Patients were visited between 2 and 6 months after hospital discharge. In this visit, all patients were subjected to an exhaustive clinical questionnaire and underwent clinical examination, pulmonary function tests and chest CT.
RESULTS: From February 2020 to August 2021, 1920 patients were included in the cohort and tracheomalacia was observed in 15 (0.8%) on expiratory HRCT imaging. All patients with tracheomalacia also presented ground glass opacities in the CT scan and 12 patients had airway sequelae.
CONCLUSIONS: Tracheomalacia is an exceptional sequela of SARS-CoV-2 survivors.

BACKGROUND: Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery.
METHODS: The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children\'s Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student\'s t-test for continuous data.
RESULTS: Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms.
CONCLUSIONS: Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.

BACKGROUND: Suprastomal collapse (SSC) is considered a major late complication of paediatric tracheostomy and can be responsible for decannulation failure in up to 20% of tracheostomised children. Depending on the severity of SSC, surgery may be required. Various strategies and techniques are available, of which the treating with airway team should be aware.
OBJECTIVE: This article intends to summarise the aetiology of SSC, its classification, clinical presentation, and the gold standard diagnostic and therapeutic algorithms according to the current literature.
METHODS: A panel of experts reviewed the available literature on SSC. Published evidence on the different surgical techniques and their advantages and disadvantages was reviewed in detail, and a treatment algorithm created.
RESULTS: The gold standard diagnostic procedure for SSC is flexible transnasal laryngotracheoscopy in spontaneous breathing followed by microlaryngoscopy (MLS) under general anaesthesia. Two main types of SSC can be differentiated, which differ in terms of surgical treatment. Purely anterior SSC is usually treated by tracheoplasty using an anterior costal cartilage graft (ACCG). Simple closure of the tracheostomy or excision of SSC with a potassium-titanyl-phosphate (KTP) laser are also described as less invasive approaches. For anterolateral SSC, segmental tracheal resection with end-to-end anastomosis or tracheoplasty with ACCG represent promising treatment options. Tracheal reinforcement with absorbable microplates is also discussed in the literature. With both types of SSC and depending on severity and the age of the child, a watch-and-wait strategy should always be considered.
CONCLUSIONS: Dynamic airway endoscopy in spontaneous breathing followed by MLS in general anaesthesia should always be performed before decannulation. It is particularly important to visualise all segments of the airway during spontaneous breathing. The decision regarding the best surgical option for each child is based on the type and localisation of SSC, as well as on the patient\'s medical and surgical history and age.
UNASSIGNED: HINTERGRUND: Der suprastomale Kollaps (SSC) gilt als eine späte Komplikation der kindlichen Tracheotomie und kann bei bis zu 20 % der tracheotomierten Kinder zu einem Scheitern des Dekanülierungsversuchs führen. Je nach Ausprägung erfordert ein SSC eine chirurgische Therapie. Es stehen einige unterschiedliche Strategien und Verfahren zur Verfügung, die dem behandelnden Team bekannt sein sollten. ZIEL: Ziel der vorliegenden Arbeit war eine Zusammenfassung der Ätiologie des SSC, seiner Klassifikation, klinischen Symptomatik und des Goldstandards in Bezug auf diagnostische und therapeutische Algorithmen gemäß der aktuellen Literatur.
METHODS: Die verfügbare Literatur zum Thema des SSC wurde von erfahrenen HNO-Ärzten in Bezug auf die chirurgischen Techniken, deren Vor- und Nachteile begutachtet und ein Behandlungspfad erarbeitet.
UNASSIGNED: Der Goldstandard zur Verifizierung eines SCC ist die flexible, transnasale Laryngotracheoskopie in Spontanatmung mit anschließender Mikrolaryngoskopie (MLS) in Vollnarkose. Die allgemein gültige Klassifizierung des SSC benennt 2 Hauptformen. Diese unterscheiden sich wesentlich in Bezug auf die chirurgischen Therapieoptionen. Bei einem rein anterioren SSC stehen hauptsächlich die laryngotracheale Rekonstruktion (LTR) anterior mit Rippenknorpel zur Verfügung. Die Pexie der Tracheavorderwand durch Nahttechnik und die Exzision des Kollapses mit dem Kaliumtitanylphosphat(KTP)-Laser werden ebenfalls beschrieben. Beim anterolateralen SSC gibt es die Tracheaquerresektion oder die LTR anterior mit Rippenknorpel als erfolgversprechende chirurgische Verfahren. Die Verstärkung der malazischen Trachea mit resorbierbaren Mikroplatten wird ebenfalls in der Literatur diskutiert. Bei beiden Formen des SSC sollte je nach Alter des Kindes und Ausprägung auch immer ein abwartendes Verhalten erwogen werden.
UNASSIGNED: Vor einem Dekanülierungsversuch sollte immer eine Laryngotracheoskopie in Spontanatmung gefolgt von einer MLS in Vollnarkose erfolgen. Insbesondere auf die Visualisierung sämtlicher Abschnitte des Atemwegs in Spontanatmung ist zu achten. Für die Entscheidungsfindung der besten Therapieoption ist die genaue Diagnosestellung, aber auch das Einbeziehen vorangegangener rekonstruktiver Eingriffe, Begleiterkrankungen sowie des Alters des Kindes entscheidend.

It was a descriptive cross-sectional study to determine the frequency and risk factors of tracheomalacia in patients undergoing thyroidectomy. One hundred and forty-nine patients underwent thyroidectomy at MTI-Khyber Teaching Hospital Peshawar between 1st January 2021 and 1st March 2022. The frequency of post-thyroidectomy tracheomalacia and possible associated factors were determined. The inclusion criteria were patients of either gender, between 18 to 70 years fulfilling criteria of clinically diagnosed cases of thyroid disorders who underwent subtotal, hemi, near or total thyroidectomy for their respective thyroid diseases. Post-thyroidectomy tracheomalacia was recorded in 18 patients (12.1%): Seventeen patients were aged less than 30 (p-value 0.038) and 14 (77.8%) patients had a duration of surgery > three hours (p-value <0.001). Young female patients with multinodular goitre who had a longer duration of surgery developed post-thyroidectomy tracheomalacia more frequently. Hence, the incidence of post-thyroidectomy tracheomalacia can be markedly reduced with proper preoperative assessment and postoperative measures. Key Words: Thyroid gland, Thyroid disorders, Tracheomalacia, Thyroidectomy.