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1.
Ultrasound J ; 16(1): 35, 2024 Jul 12.
Article in English | MEDLINE | ID: mdl-38995421

ABSTRACT

BACKGROUND: Tracheal necrosis post endotracheal intubation is a rare life-threatening disease that can compromise airway patency. We demonstrated a novel usage of upper airway ultrasonography (USG) to diagnose tracheal necrosis. CASE PRESENTATION: A middle-aged smoking male presented with productive cough, noisy breathing and exertional dyspnea for 2 weeks. He was intubated one month prior due to a traumatic brain injury. Upper airway USG findings showed irregular air-mucosal interface (AMI) and comet tail artefacts over the 1st and 2nd tracheal ring. A direct laryngoscopy in the operating room showed thick mucopus inferior to the vocal cords, with necrotic tracheal cartilages and debris obstructing the airway. He was successfully treated with parenteral antibiotics, wound debridement and tracheostomy. CONCLUSION: Our case highlights the first documented USG findings of tracheal necrosis. Upper airway USG serves as a potential diagnostic modality in managing the condition.

2.
Ther Clin Risk Manag ; 20: 405-412, 2024.
Article in English | MEDLINE | ID: mdl-38974311

ABSTRACT

Purpose: Intravenous sedation (IVS) with propofol (PPF) is commonly performed in dental treatment, particular in patients with dentophobia, with gag reflex, or undergoing implant surgeries, as PPF has the advantages of rapid induction and recovery. However, PPF and other intravenous sedatives may cause respiratory depression. Thus, IVS with PPF requires oxygen administration. But airway burn may occur when high-concentration oxygen is stored in the oral cavity and catches fire. For these reasons, the present study aimed to elucidate the changes in oxygen concentration (OC) under IVS with PPF and oxygen administration. Patients and methods: Nineteen healthy male volunteers participated in the study. None of them had missing teeth, nasal congestion, or temporomandibular joint dysfunction. They were sedated with a continuous PPF infusion dose of 6 mg/kg/hr for 25 min, followed by administration of 3 L/min oxygen via a nasal cannula. The OC was measured at two sites, namely, the median maxillary anterior teeth (MMAT) and median maxillary soft palate (MMSP), before PPF infusion (baseline) and 14, 15-18 (Term 1), 19, and 20-23 (Term 2) min after the start of infusion. Results: Compared with the values at baseline, the OC in the MMSP significantly increased at each time point, whereas the OC in the MMAT significantly increased at Term 2. Furthermore, in the comparison of the OC before and after the use of a mouth prop, the OC exhibited an upward trend, but no statistically significant differences were observed between the two time points in the MMAT and MMSP. In IVS with PPF and oxygen administration, the OC in the pharynx increases as the sedative level deepens. Conclusion: Oxygen administration should be temporarily discontinued, and suction should be performed to decrease the OC in the oral cavity when sparking procedures during IVS with PPF and oxygen administration are performed.

3.
Cureus ; 16(6): e62898, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39040758

ABSTRACT

Background Upper airway obstruction (UAO) is a significant clinical concern due to its potential to lead to serious health issues, including obstructive sleep apnea (OSA) and cardiovascular diseases. Traditional diagnostic methods, such as spirometry, are often invasive and complex. This study aims to validate a portable ventilatory airway screening (PVAS) device as a non-invasive, cost-effective alternative for measuring upper airway breathing pressure and respiratory flow. Objectives To validate the accuracy of the PVAS device in measuring upper airway breathing pressure and respiratory flow by comparing its readings with those obtained from standard spirometry tests. Methods This cross-sectional analytical study involved 40 growing individuals aged 10-14 years, divided into two groups based on cephalometric analysis: Skeletal Class I (20 patients) and Skeletal Class II with retrognathic mandible (20 patients). Breathing pressure, volume, and velocity measurements were recorded using both the PVAS device and spirometry, and their accuracy was compared. Results The PVAS device showed high concordance with spirometry results, demonstrating significant accuracy in measuring breathing pressure, volume, and velocity. Skeletal Class II individuals exhibited significantly higher breathing pressure and reduced respiratory flow compared to Class I individuals, as measured by the PVAS device. Conclusion The PVAS device is a valid and accurate tool for non-invasive measurement of upper airway breathing pressure and respiratory flow. Its ease of use and reliability make it a valuable tool for clinical practice, particularly in the early diagnosis and management of airway obstructions.

4.
Indian J Otolaryngol Head Neck Surg ; 76(3): 2411-2416, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38883486

ABSTRACT

Tracheostomy is a surgical procedure in an emergency setting to relieve the upper airway obstruction by creating an opening in the anterior part of trachea. It can also be done electively to wean off from a ventilator, during an elective surgery and clearance of pulmonary secretions. This study was a retrospective analysis of microbiological profile, antibiotic sensitivity & resistance pattern in patients with a tracheostomized wound. A retrospective review of the microbiological profiles of all adult patients who underwent a tracheostomy was conducted between May 2022 and May 2023 at our hospital. Based on the tracheostomy indications, patients were allocated under obstructed and non-obstructed group. Any patient with at least one positive sample was followed up quarterly for a year. The first culture result obtained was recorded at least one month following the last antibiotic dose in each quarter. Out of the 65 tracheal aspirate results obtained from 58 patients (mean age, 57.5 ± 16.48 years), the most common procedure and indications were surgical tracheostomy (72.4%) and non-obstructed causes (74.1%), respectively. Moreover, 47.7% of the culture results indicated Pseudomonas aeruginosa, which showed significantly different proportions across the quarters (p = 0.006). Among obstructed patients, P. aeruginosa was the most common (35%), followed by methicillin-resistant Staphylococcus aureus (MRSA; 23.5%). The colonization was predominantly by gram negative bacteria Acinetobacter species, P. aeruginosa & Klebsiella pneumoniae and fungal species like Candida albicans followed by Aspergillus niger and non-Albicans candida.

5.
Cleft Palate Craniofac J ; : 10556656241261846, 2024 Jun 17.
Article in English | MEDLINE | ID: mdl-38881285

ABSTRACT

OBJECTIVE: Narrowing of the palatal cleft is often observed in infants with Robin sequence (RS) treated with the Stanford Orthodontic Airway Plate treatment (SOAP) even though SOAP is utilized primarily to establish airway patency. The current study quantified dimensional changes of the cleft palate (CP) in infants with RS treated with SOAP. DESIGN: A retrospective chart review. PATIENTS: Infants with RS and CP who completed SOAP and had maxillary arch models at both pre- and post-treatment time points at a single tertiary referral hospital between September 2019 and July 2023. SETTING AND OUTCOME MEASURE: Maxillary arch models were measured and analyzed using Bivariate statistical analysis. RESULTS: Seventeen infants were included in the study. The median age (min, max) was 6.7 weeks (1.1, 21.9) at pre-treatment and 26.6 weeks (18.7, 37.0) at post-treatment. The median Obstructive Apnea Hypopnea Index was 36.2 events/hour (8.1, 103.1) at pre-treatment and 4.1 events/hour (1.9, 8.6) at post-treatment. The pre-treatment width of CP decreased by an average (± standard diviation) of 6.37 mm (± 3.55, p < 0.001) at post-treatment. The ratio of the posterior cleft width to the total maxillary arch width decreased from 40% (± 9.1) at pre-treatment to 22% (± 11) at post-treatment (p < 0.001). CONCLUSION: The dimensions of CP reduced significantly during SOAP in infants with RS and CP treated for their severe upper airway obstruction. The findings highlight a potential benefit of SOAP that may contribute favorably to the palate repair surgery.

6.
Radiol Oncol ; 2024 Jun 12.
Article in English | MEDLINE | ID: mdl-38861690

ABSTRACT

BACKGROUND: The aim of the study was to provide insight into the influence of the COVID-19 on the frequency and characteristics of urgent and emergent tracheostomies (TS), comparing data collected both before and during the pandemic. Our two hypotheses were that during COVID-19, more TS were performed in the emergent setting and that during COVID-19 more TS were performed under general anaesthesia. PATIENTS AND METHODS: The research was retrospective. The study period included the two years before and after the COVID-19 outbreak in Slovenia. Forty-one patients in each period met the inclusion criteria. Their medical charts were reviewed. The anamnestic, clinical, surgical and anaesthesiological data were collected. The two groups of patients from corresponding time periods were statistically compared. RESULTS: Predominantly men required the surgical resolution of acute upper airway obstruction (76% of patients). The causes for acute respiratory distress included head and neck cancer (62%), infections (20%), vocal cord paralysis (16%), and stenosis (2%). There were no statistically significant differences either in the (emergent/urgent) setting of TS or in the type of anaesthesia used. Both hypotheses were rejected. A statistically significant rise in use of the C-MAC laryngoscope during COVID-19 (from 3% to 15%) was reported. CONCLUSIONS: The outbreak of COVID-19 did not have a statistically significant effect on the frequency of performing emergent and urgent tracheostomies nor on the use of general or local anaesthesia. It did, however, require a change of intubation technique. Consequently, a significant rise in the use of the C-MAC laryngoscope was noted.

8.
J Clin Med ; 13(9)2024 Apr 30.
Article in English | MEDLINE | ID: mdl-38731172

ABSTRACT

Mechanical insufflation-exsufflation (MI-E) is essential for secretion clearance, especially in neuromuscular disorders. For the best outcomes, initiation of MI-E should be started at the correct time with regular evaluation to the response to treatment. Typically, cough peak flow has been used to evaluate cough effectiveness with and without MI-E. This review highlights the limitations of this and discussed other tools to evaluate MI-E efficacy in this rapidly developing field. Such tools include the interpretation of parameters (like pressure, flow and volumes) that derive from the MI-E device and external methods to evaluate upper airway closure. In this review we pinpoint the differences between different devices in the market and discuss new tools to better titrate MI-E and detect pathological responses of the upper airway. We discuss the importance of point of care ultrasound (POCUS), transnasal fiberoptic laryngoscopy and wave form analysis in this setting. To improve clinical practice newer generation MI-E devices should allow real-time evaluation of waveforms and standardize some of the derived parameters.

9.
Int Med Case Rep J ; 17: 409-415, 2024.
Article in English | MEDLINE | ID: mdl-38715733

ABSTRACT

Background: Thyroid gland abscess is a rare pathology with life-threatening complications when there is a delay in diagnosis. However, physicians should be aware of and consider this differential in patients with anterior neck swelling having acute onset compressive symptoms to ensure early diagnosis and management. Case Presentation: A 62-year-old female patient presenting with worsening of painful anterior neck swelling with associated fever, shortness of breath, and difficulty swallowing. The patient was found to have a thyroid abscess causing upper airway obstruction, against a background of follicular nodular disease found on clinical examination, cytology and fluid analysis from aspirate, biopsy, ultrasonography, and computed tomography. The patient was managed with endotracheal intubation and was subsequently discharged after recovery with antibiotic therapy, incision and drainage, and thyroid lobectomy. Conclusion: Thyroid abscess is an uncommon, critical clinical condition with high morbidity and mortality. Thyroid gland abscess should be considered while evaluating patients presenting with acute onset anterior neck swelling. Satisfactory clinical outcomes could be achieved with early diagnosis and proper management.

10.
Cureus ; 16(4): e58735, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38779228

ABSTRACT

Infectious mononucleosis (IM) is a clinical disease caused by the Epstein-Barr virus (EBV). Common presenting symptoms include sore throat, lymph node enlargement, fever, and malaise. Although severe upper airway obstruction is uncommon, it is a potentially fatal complication that requires immediate intervention. We describe the case of an 18-year-old Hispanic man who presented with a progressive sore throat and difficulty speaking, requiring endotracheal intubation for airway protection. CT images showed diffuse swelling of Waldeyer's tonsillar ring, multiple enlarged lymphadenopathies, and splenomegaly. Acute EBV infection was confirmed considering clinical presentation and using the heterophile antibody, anti-nuclear and anti-viral capsid antigens, and quantitative PCR. The patient was managed with ventilatory support, empirical antibiotic therapy, and systemic corticosteroids, achieving a positive outcome. Our case illustrates the use of corticosteroids in managing severe upper airway obstruction complicating IM.

11.
Wilderness Environ Med ; : 10806032241252109, 2024 May 10.
Article in English | MEDLINE | ID: mdl-38725406

ABSTRACT

Snakebites in unusual anatomical locations may lead to life-threatening consequences. Merrem's hump-nosed viper (Hypnale hypnale) is a medically important snake in Sri Lanka and India that causes many bites and envenomings. Their bites occur almost exclusively on upper and lower limbs and commonly result in local effects, with some patients developing systemic envenoming. No antivenom is available for treating envenoming by H. hypnale. We report an unusual case of H. hypnale bite on the tongue of a 10-month-old infant resulting in rapid local swelling of the tongue and floor of the mouth, requiring prompt intervention to prevent life-threatening upper airway obstruction. Early tracheostomy prevented upper airway obstruction and, along with supportive steroid therapy and antibiotics, led to a complete resolution of the local effects of the infant without permanent disability, despite the unavailability of antivenom.

12.
JDR Clin Trans Res ; : 23800844241246198, 2024 May 10.
Article in English | MEDLINE | ID: mdl-38733110

ABSTRACT

KNOWLEDGE TRANSFER STATEMENT: Obstructive sleep apnea has been proven to have a great negative impact on patients, and the relationship between sleep apnea and dental caries is still inconclusive. Our study shows that patients with sleep apnea and those older than 45 y have a significant risk of dental caries.

13.
Otolaryngol Head Neck Surg ; 171(2): 554-559, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38668374

ABSTRACT

OBJECTIVES: (1) To investigate the prevalence and severity of drooling among healthy young children referred for adenotonsillectomy; (2) to evaluate the effect of adenotonsillectomy on drooling. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. METHODS: Healthy typically developed children aged 18 to 48 months referred to adenotonsillectomy for upper airway obstruction (UAO) were recruited. Age-matched children recruited from the community served as controls. Drooling frequency and severity were assessed at baseline and 2 months following surgery based on 2 subjective scales: the Drooling Infants and Preschoolers Scale (DRIPS) and Thomas-Stonell and Greenberg Saliva Severity Scale (TSGS). RESULTS: Eighty-seven children aged 18 to 48 months were included in the study. Forty-three children referred to adenotonsillectomy (study group) and 44 age-matched controls. There were significant differences in almost all of the DRIPS items between children in the presurgery group compared to controls. Drooling severity and frequency were greater in the former compared to the latter (TGF-s severity: 1.4 ± 1.0 vs 0.6 ± 0.8, P = .001; TGF frequency: 1.3 ± 0.9 vs 0.5 ± 0.6, P < .001). After surgery, the scores for all DRIPS and TSGS items decreased significantly and were comparable to the control group. CONCLUSIONS: The frequency and severity of drooling among otherwise young children referred for adenotonsillectomy were greater than those for healthy controls. Following surgery, both the frequency and severity significantly improved and became comparable to those of controls. These findings suggest that a major improvement in drooling is one of the benefits of a surgical intervention in a child with UAO.


Subject(s)
Adenoidectomy , Severity of Illness Index , Sialorrhea , Tonsillectomy , Humans , Tonsillectomy/methods , Child, Preschool , Sialorrhea/surgery , Sialorrhea/etiology , Male , Female , Prospective Studies , Infant , Prevalence , Treatment Outcome , Case-Control Studies
14.
Cureus ; 16(3): e56017, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38606260

ABSTRACT

Congenital nasal pyriform aperture stenosis (CNPAS) is a very rare cause of neonatal respiratory distress and is often missed because of its rarity. It arises from the overgrowth of the nasal process of the maxilla. Maxillofacial CT scan findings of pyriform aperture width <11 mm in a full-term baby, median central incisor, triangular-shaped palate, and median palatal ridge confirm the diagnosis. We describe here a case of CNPAS admitted with respiratory distress that increased further on feeding. An infant feeding tube of size 6 was not negotiable through the nostrils. Resistance was appreciated at the inlet of the nostril. Maxillofacial CT showed pyriform aperture stenosis of 3.4 mm, suggesting CNPAS. The child could not be weaned off a high-flow nasal cannula despite conservative management with decongestants, steroids spray, dilatation, and stenting for 20 days. Subsequently, surgical widening of the nasal aperture by a sublabial approach was done. The child was discharged on the 10th postoperative day on full oral feeds. It is important to suspect CNPAS in neonates with respiratory distress where other common causes have been ruled out, as it can be treated by surgery in cases refractory to conservative management.

15.
J Vet Intern Med ; 38(3): 1449-1457, 2024.
Article in English | MEDLINE | ID: mdl-38561963

ABSTRACT

BACKGROUND: Aerophagia (ingestion of air), is a functional aerodigestive disorder in people. Criteria for diagnosis of aerophagia in dogs are >1/3 of bolus volume containing air or ingested air resulting in gastric distention (>1/3 of end gastric volume). Aerophagia is highlighted during eating and drinking. Videofluoroscopic swallow studies (VFSS) document aerophagia in dogs, but the incidence, clinical signs (CS), and associated disorders are unknown. OBJECTIVES: Identify the incidence of aerophagia, compare CS between dogs with and without aerophagia, and identify associated and predisposing disorders using VFSS. ANIMALS: A total of 120 client-owned dogs. METHODS: Sequential VFSS and associated medical records from dogs presenting to veterinary teaching hospitals at Auburn University and the University of Missouri were retrospectively reviewed. Statistical comparisons were made using Mann-Whitney and chi-squared tests, odds ratios (OR), and multiple logistic regression (P < .05). RESULTS: The incidence (95% confidence interval [CI]) of aerophagia was 40% (31.7-48.9). Dogs with mixed CS (gastrointestinal [GI] and respiratory; P < .001, 58.3%) were more likely to have aerophagia than dogs with exclusively respiratory CS (25%). Aerophagia was significantly more common in brachycephalic dogs (P = .01; 45.8% vs 13.8%), dogs with nonbrachycephalic upper airway obstruction (P < .001; 33.3% vs 4.1%), pathologic penetration and aspiration (P-A) scores (P = .04; 41.6% vs 23.6%), and gagging (P < .001; 25% vs 11.7%). Mixed CS (P = .01), brachycephaly (P < .001), and upper airway obstruction (P < .001) were independent predictors of aerophagia. CONCLUSIONS AND CLINICAL IMPORTANCE: Aerophagia was common, particularly in dogs with mixed CS. Brachycephalic dogs and dogs with upper airway obstruction are predisposed. Aspiration risk was high, emphasizing overlapping upper aerodigestive pathways.


Subject(s)
Dog Diseases , Animals , Dogs , Dog Diseases/diagnostic imaging , Dog Diseases/epidemiology , Female , Male , Retrospective Studies , Fluoroscopy/veterinary , Incidence , Aerophagy/veterinary , Video Recording , Deglutition/physiology
16.
Article in Chinese | MEDLINE | ID: mdl-38433691

ABSTRACT

Objective:To analyze the characteristics of otorhinolaryngological clinical manifestations in children with Mucopolysaccharide(MPS) type Ⅰ and type II in order to improve the knowledge of otorhinolaryngologists about this disease. Methods:Clinical data related to 55 children with MPS type Ⅰ and type II were retrospectively analyzed to investigate the clinical manifestations of MPS in ENT. Results:All 40 patients(72.72%) with MPS had at least one ENT symptom during the course of the disease, with 95% of them having an ENT symptom prior to the diagnosis of MPS; upper airway obstruction was the most common ENT symptom(34, 85.00%), followed by recurrent upper respiratory tract infections(23, 57.50%), and lastly, hearing loss(11, 27.50%); all 26 patients had undergone at least one surgical procedure, of which 15(57.69%) had undergone ENT surgery, and all of these patients underwent ENT surgery before diagnosis. The most common ENT surgery was adenoidectomy. Conclusion:Early clinical manifestations of MPS patients are atypical, but the early and prevalent appearance of otolaryngologic symptoms and increased awareness of the disease among otolaryngologists has a positive impact on the prognosis of MPS.


Subject(s)
Deafness , Nose Diseases , Child , Humans , Retrospective Studies , Adenoidectomy , Glycosaminoglycans
17.
Ear Nose Throat J ; : 1455613241238620, 2024 Mar 11.
Article in English | MEDLINE | ID: mdl-38462908

ABSTRACT

Objectives: Acute upper airway obstruction is a critical medical condition that presents considerable challenges to anesthesiologists. This study aims to provide a formal report on the incidence and outcomes of perioperative complications, as well as the factors that influence unstable intraoperative hemodynamics. Methods: This retrospective study reviewed patients aged 18 years and older who underwent emergency tracheostomy between January 2013 and October 2021. Data on perioperative complications and outcomes within the first 24 hours and 7 days after surgery were subjected to analysis. Descriptive and multivariate analyses were used to examine the results. Results: A total of 253 patients were included in the study. The mean age was 61.5 ± 12.9 years. Malignancy was detected in 78.3% of the patients. General anesthesia was administered to 43.9% of the patients. The incidence of intraoperative complications was 51.8%, with hypotension and hypertension occurring in 30.4% and 22.5% of the cases, respectively. Desaturation and cardiac arrest were observed in 4.7% and 0.8% of the patients, respectively. The mortality rate at 7 days after surgery was 1.6%. Multivariate analysis revealed that monitored anesthesia care [adjusted odds ratio (OR) = 1.80; 95% confidence interval (CI): 1.08-3.00] and hypertensive patients (adjusted OR = 1.70, 95% CI: 1.01-2.86) were associated with unstable intraoperative hemodynamics. Conclusions: Cardiovascular instability represented the majority of the complications observed. Monitoring anesthesia care and hypertension were significant prognostic factors for unstable intraoperative hemodynamics. We recommend extensive communication between ear, nose, and throat surgeons and anesthesiologists to determine the appropriate choice of anesthesia.

18.
Cleft Palate Craniofac J ; : 10556656241233239, 2024 Feb 19.
Article in English | MEDLINE | ID: mdl-38373407

ABSTRACT

OBJECTIVE: To identify weight gain trends of infants with Robin sequence (RS) treated by the Stanford Orthodontic Airway Plate treatment (SOAP). DESIGN: Retrospective longitudinal cohort study. SETTING: Single tertiary referral hospital. PATIENTS: Eleven infants with RS treated with SOAP. INTERVENTIONS: Nonsurgical SOAP. MAIN OUTCOME MEASURES: Body weight, Weight-for-age (WFA) Z-scores, and WFA percentiles at birth (T0), SOAP delivery (T1), SOAP graduation (T2), and 12-months old (T3). RESULTS: Between T0 and T1, the weight increased but the WFA percentile decreased from 36.5% to 15.1%, and the Z-score worsened from -0.43 to -1.44. From T1 to T2, the percentile improved to 22.55% and the Z-score to -0.94. From T2 to T3, the percentile and the Z-scores further improved to 36.59% and -0.48, respectively. CONCLUSIONS: SOAP provided infants experiencing severe respiratory distress and oral feeding difficulty with an opportunity to gain weight commensurate with the WHO healthy norms without surgical intervention.

19.
Cleft Palate Craniofac J ; : 10556656241229892, 2024 Feb 01.
Article in English | MEDLINE | ID: mdl-38303142

ABSTRACT

OBJECTIVE: The only findings consistent among infants with Robin sequence (RS) are the presence of micrognathia, glossoptosis, and upper airway obstruction (UAO). Feeding and growth dysfunction are typical. The etiopathogenesis of these findings, however, is highly variable, ranging from sporadic to syndromic causes, with widely disparate levels of severity. This heterogeneity has created inconsistency within RS literature and debate about appropriate workup and treatment. Despite several attempts at stratification, no system has been broadly adopted. DESIGN: We recently presented a novel classification that is summarized by the acronym MicroNAPS. Each of 5 elements is scored: Micrognathia, Nutrition, Airway, Palate, Syndrome/comorbidities, and element scores are summarized into a "stage". RESULTS: Testing of this system in a sample of 100 infants from our center found it to be clinically relevant and to predict important management decisions and outcomes. CONCLUSIONS: We herein present an interactive website (www.prscalculator.com) and printable reference card for simple application of MicroNAPS, and we advocate for this classification system to be adopted for clinical care and research.

20.
Int J Pediatr Otorhinolaryngol ; 177: 111841, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38181460

ABSTRACT

BACKGROUND: Congenital laryngotracheal stenosis (CLS) is a rare cause of stridor among newborns. Evidence has shown that several family members can be affected by CLS. Knowledge of the pathophysiology of familial congenital laryngotracheal stenosis (FCLS) will enable more effective therapeutic strategies. OBJECTIVE: To determine the clinical course and outcome of familial congenital laryngotracheal stenosis (FCLS). METHODS: A literature search was conducted over a period of one month (September 2023) by searching several databases to identify studies published from inception to 31st August 2023. RESULTS: Of 256 papers identified, five articles met the inclusion criteria. A total of 17 patients with slight female predominance (59 %) were identified. Familial congenital tracheal stenosis was reported in female twins (100 %). A variety of clinical presentations were listed. An endoscopic airway study was performed on all patients. 64.8 % of the included children were managed surgically. Genetic studies performed on 41 % of children could not locate genetic abnormalities. CONCLUSION: Consanguinity, twin births, and female gender could be predisposing factors for FCLS, although the quality of evidence is low due to the rarity of the condition.

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