Congenital Subglottic Stenosis Research Articles

One-stage thyroid cartilage laryngotracheal reconstruction for children less than one year old with congenital subglottic stenosis.

To evaluate one-stage thyroid cartilage laryngotracheal reconstruction in children less than one year of age with congenital subglottic stenosis. Congenital subglottic stenosis children less than one year old who underwent one-stage thyroid cartilage laryngotracheal reconstruction between 2016 and 2020 in our department were retrospectively reviewed. Their clinical characteristics, treatments and prognoses were assessed. Eleven congenital subglottic stenosis children (6-11 months) were included: seven with Myer-Cotton grade II, and four with Myer-Cotton grade III. Their tracheal diameters were corrected to normal size using thyroid cartilage, and they were intubated under sedation for two weeks after surgery. Moreover, all of them received anti-infection and anti-reflux therapies during hospitalisation. No breathing difficulty, aspiration, hoarseness or laryngitis was observed during the follow-up period (10-30 months), and their growth and development were age appropriate. The one-stage thyroid cartilage laryngotracheal reconstruction is a good treatment option for congenital subglottic stenosis children less than one year old with Myer-Cotton grade II-III.

Successful Treatment Of A Child With Severe Congenital Subglottic Stenosis With Balloon Dilatation.

Successful Treatment Of A Child With Severe Congenital Subglottic Stenosis With Balloon Dilatation.

Efficacy of Cricotracheal Stenosis Resection (CTSR) Among Children with Congenital vs. Acquired Sub-glottic Stenosis.

Sub-glottic Stenosis (SGS) treatment in children is challenging because there is no standard algorithm to follow; however, the use of endoscopic techniques in SGS treatment has emerged over the last decades and has advanced. The aim of this study was to assess the efficacy of Cricotracheal Stenosis Resection (CTSR) among children with congenital vs. acquired SGS. In this retrospective study, we reviewed the charts of 22 patients who underwent endoscopic intervention as the primary modality of treatment for SGS at King Abdulaziz Medical City from January 1, 2011 to October 31, 2019. Successful treatment was defined as: resolution of symptoms, restoration of a normal patent airway with no stenosis, and decannulation. Out of 22 patients, 14 cases were acquired and 8 were congenital SGS. Most of the patients had grade 3 stenosis before surgery 15 (68%), followed by grade 1 stenosis among 4 (18.2%) and grade 2 stenosis was present in 3 (13.6%) patients. Postoperatively, 17 (77.3%) patients improved to grade zero, whereas grade 3 stenosis was not reported in any patient. The Mc-Nemar's test showed significant improvement between pre- and post-operative stenosis grade with test value = 22, and P value = 0.003. This technique was successful among 18 (82%) patients with P value ≤ 0.01. Irrespective of the differences in the age of patients, length, and character of stenosis among congenital and acquired groups, the endoscopic CTSR technique proved to be successful in both groups. We achieved a success rate of 86% in the acquired series, and 75% in the congenital series, which is a very promising result.

Immediate extubation after single-stage laryngotracheal reconstruction for subglottic stenosis in children

PurposeTo evaluate the feasibility and clarify the appropriate indications for extubation immediately after single-stage laryngotracheal reconstruction (SS-LTR) in pediatric subglottic stenosis (SGS).MethodsA retrospective study was performed from July 2017 to July 2022. All patients underwent SS-LTR with anterior costal cartilage graft. Information such as demographics, comorbidities, history of intubation or tracheostomy, Classification and grading of airway stenosis, the operation-specific decannulation rate and overall decannulation rate were analyzed.ResultsTwenty-two patients with simple SGS were identified. The median age at SS-LTR was 19 months (IQR = 18.5 months). Fourteen patients (63.6%) were intubated prior to the presentation of symptoms. Fourteen patients (63.6%) required preoperative tracheostomy to maintain a secure airway. Eight patients (36.4%) had congenital SGS, 10 patients (45.5%) had acquired SGS, and 4 patients (18.2%) had mixed SGS. Three patients had Grade II stenosis. Nineteen patients had Grade III stenosis. Comorbidities were found in 10 patients (45.5%). Major comorbidities were pneumonia. Congenital airway anomalies were found in 6 patients (27.3%). After anesthesia, all 22 patients were successfully extubated and returned to the general ward. Twenty patients had a satisfactory airway after SS-LTR. Two patients required reintubation or tracheostomy after operation. Operation-specific decannulation rate was 90.9%. The overall decannulation rate is 100%.ConclusionSS-LTR with anterior costal cartilage graft is an effective method to treat simple SGS ranging from Grades I to III in children. Extubation immediately after surgery is safe and feasible.

Endoscopic anterior cricoid split with dilation

Endoscopic anterior cricoid split may be performed as an adjunct to balloon dilation in select patients with subglottic stenosis. It offers the possibility for the treatment of more significant, mature subglottic stenosis, as well as congenital subglottic stenosis with cartilaginous anomaly endoscopically whereas previously these patients could only successfully be treated with an open procedure. Here the indications, preoperative workup, operative technique and post-operative considerations are outlined. In certain situations, an endoscopic anterior-posterior cricoid split or open approach may be better suited. Endoscopic anterior cricoid split may be performed as an adjunct to balloon dilation in select patients with subglottic stenosis. It offers the possibility for the treatment of more significant, mature subglottic stenosis, as well as congenital subglottic stenosis with cartilaginous anomaly endoscopically whereas previously these patients could only successfully be treated with an open procedure. Here the indications, preoperative workup, operative technique and post-operative considerations are outlined. In certain situations, an endoscopic anterior-posterior cricoid split or open approach may be better suited.

3D bioprinting in airway reconstructive surgery: A pilot study

ObjectivesOpen surgery is a reliable choice for congenital subglottic stenosis, that represents the third most common congenital anomaly of the larynx. One of the procedures performed is anterior laryngotracheal reconstruction (LTR) with anterior rib graft. The objective of this preliminary study was to evaluate the potential of 3D printing technology for the realization of laryngo-tracheal scaffold in Polycaprolactone (PCL) implanted in vivo in ovine animal model. MethodsA 3D computer model of a laryngeal graft and a tracheal graft was designed and printed with PCL through 3D additive manufacturing technology. The scaffolds were seeded with autologous mesenchymal stem cells and cultured in vitro for up to 14 days. Anterior graft LTR with 3D printed scaffolds was performed on 5 sheep. The animals underwent endoscopic examinations at the first, 3rd, 6th, and 12th weeks after surgery and before sacrifice. The integration of the material was evaluated by the pathologist. ResultsTwo animals showed a favourable postoperative course and were sacrificed at 6 months postoperatively. In these cases, we observed endoscopically a complete integration of the cellularized PCL scaffold into the peri-implant tissues, and the pathologist found the growth of respiratory epithelium on the scaffold's inner surface. Other two animals showed a difficult post-operative recovery characterized by respiratory distress resulting in early sacrifice on postoperative days 31 and 33. In these animals we found a poor integration of the grafts into the tracheal structure, and a better integration of the laryngeal scaffold. The last animal developed a wound abscess and was sacrificed 80 days after surgery. We observed, in this case, a poor scaffold integration and an acute inflammatory reaction. ConclusionsFrom the preliminary data obtained we found that the excessive stiffness of the material, along with the anatomical features of the sheep, is a major limitation of this study. It will be necessary in the future to create a new biocompatible, more flexible and elastic graft, to achieve greater integration into surrounding tissues. Bioconstructed grafts could simplify surgery for the treatment of laryngo-tracheal stenosis, particularly in the treatment of long tracheal stenoses, which have, at the moment, very complex surgical options. Level of evidenceNA.

Updated clinical and glycomic features of mannosyl-oligosaccharide glucosidase deficiency: Two case reports

BACKGROUNDMannosyl-oligosaccharide glucosidase (MOGS) deficiency is an extremely rare type of congenital disorder of glycosylation (CDG), with only 12 reported cases. Its clinical, genetic, and glycomic features are still expanding. Our aim is to update the novel clinical and glycosylation features of 2 previously reported patients with MOGS-CDG.CASE SUMMARYWe collected comprehensive clinical information, and conducted the immunoglobulin G1 glycosylation assay using nano-electrospray ionization source quadruple time-of-flight mass spectrometry. Novel dysmorphic features included an enlarged tongue, forwardly rotated earlobes, a birth mark, overlapped toes, and abnormal fat distribution. Novel imaging findings included pericardial effusion, a deep interarytenoid groove, mild congenital subglottic stenosis, and laryngomalacia. Novel laboratory findings included peripheral leukocytosis with neutrophil predominance, elevated C-reactive protein and creatine kinase, dyslipidemia, coagulopathy, complement 3 and complement 4 deficiencies, decreased proportions of T lymphocytes and natural killer cells, and increased serum interleukin 6. Glycosylation studies showed a significant increase of hypermannosylated glycopeptides (Glc3Man7GlcNAc2/N2H10 and Man5GlcNAc2/N2H5) and hypersialylated glycopeptides. A compensatory glycosylation pathway leading to an increase in Man5GlcNAc2/N2H5 was indicated with the glycosylation profile. CONCLUSIONWe confirmed abnormal glycomics in 1 patient, expanding the clinical and glycomic spectrum of MOGS-CDG. We also postulated a compensatory glycosylation pathway, leading to a possible serum biomarker for future diagnosis.

Long-term results of slide laryngotracheoplasty for congenital subglottic stenosis in newborns and infants

ObjectivesSlide laryngotracheoplasty is an effective, single-step procedure without tracheostomy and stenting for treating high-grade congenital subglottic stenosis in neonates and infants. Long-term outcomes were evaluated to assess the reliability of the procedure performed in this age of rapid development of the laryngeal structures. MethodsWe report five children who underwent slide laryngotracheoplasty before the age of 4 months, each with >3 years follow-up. Increases of length and bodyweight were systematically assessed. Breathing, swallowing, voice, and overall satisfaction was assessed by a quality of life questionnaire. Voice quality was objectively evaluated by measuring shimmer, jitter, fundamental frequency, and the harmonics-to-noise ratio. ResultsAll patients had a stable and adequate airway during follow-up without any additional open airway surgery. The patients' voices were physiological, and the intervention had no negative impact on speech development. Swallowing function was optimally retained, and the patients’ bodyweight gain and length were satisfactory. During at least 3 years of observation, the anastomosis remained stable and grew dynamically with the patient. ConclusionsSlide laryngotracheoplasty (as a single-step procedure) provides an adequate airway without tracheostomy, grafting, or stenting with good long-term functional results in selected neonates and infants with congenital subglottic stenosis.

НЕОДИМОВЫЙ ЛАЗЕР В ЛЕЧЕНИИ ЛАРИНГОТРАХЕОСТЕНОЗА ПОСЛЕ ТРАХЕОСТОМИИ У ДЕТЕЙ МЛАДЕНЧЕСКОГО ВОЗРАСТА

Objective. To evaluate the effect of neodymium laser application for successful decannulation in infants with a congenital and acquired tracheal stenosis and tracheostomy. Methods. 6 children were being treated in the 1<sup>st</sup> City Clinical Hospital of Minsk in 2011-2014. All children underwent tracheostomy with a prolonged tracheal intubation and subsequent development of tracheostenosis. The children were examined using X-ray, esophagogastroscopy (PentaxEG-16K10), and bronchoscopy (Olympus MAF TYPE GM). Tracheal stenosis was treated using a medical multifunctional laser complex Multiline (“Linline Medical systems” Ltd, Belarus) equipped with a high-energy neodymium laser emitter. Children with excessive growth of granulations in thelumenof thetrachea underwent vaporization of granulations by means of a bulbous probe with a neodymium laser emitter (wavelength of 1340 nm, power: 15 W, 2 sec. exposure). In cicatricial tracheal stenosis, incisions were made with a laser scalpel (the wavelength of 1064 nm, power of 20 W). Results. Effective decannulation was observed in 5 children, including 4 children who underwent3-5 treatment sessions. In 1 child with the congenital subglottic larynx stenosis, to perform the decannulation became possible after 2 years and 24 sessions of laser recanalizationofstenosis and ablation of granulations. One child, after 9 treatment sessions, dropped out of observation, the result has not been defined. Conclusion. Tracheostomy in children promotes the formation of granulations in the tracheal lumen with the formation of stenosis, which subsequently hinders a successful decannulation. The neodymium laser application with the endoscopic access provides the visual direct approach to the focus of impact with the aim of laser-induced vaporization of soft tissues and recanalization of the trachealmass in children with short tracheal stenosis leads to successful decannulation. What this paper adds Firstly the neodymium laser for the treatment of congenital and acquired tracheal stenosis in children after tracheotomy has been used. The method has been developed for restoring the tracheal lumen by laser-inducedvaporization of excessive tracheal granulation and making scalpel incisions in the scar area. It is shown the neodymium laser application with the endoscopic access provides the visual direct approach to the focus of impact. Laser-induced vaporization of soft tissues and recanalization of the tracheal lumen in children with short tracheal stenosis leads to successful decannulation.

Modified Infant Feeding Tube as an Indigenous Endotracheal Tube in Unanticipated Congenital Subglottic Stenosis

Introduction: In neonates with an unanticipated difficult airway, there is an increased probability of failed intubation even with highly trained hands while performing direct laryngoscopy. Aims: We describe our experience of using a modified infant feeding tube (IFT) as an indigenous endotracheal tube (ETT) in a series of paediatric surgical patients with congenital subglottic stenosis (SGS). Material and Methods: A retrospective study was performed in our institute from January 2018 to December 2019. ETT with modified 6 and 8 French (Fr) sized IFT’s were used. A stylet obtained from the VP shunt system was used to aid in intubation. Results: Out of 12,500 admissions in our department, there were 5 pediatric cases with SGS which were managed using modified IFT. Four were neonates and 1 infant (M:F = 2:3). Modified IFT(s) were used after failed intubation with ETT of size 2.5 mm in 4 patients (non-availability of ETT of size 2 mm), while in one neonate, even 2 mm ETT could not be negotiated due to SGS. In all the 5 children, successful intubation was finally performed with modified 8 French (Fr) IFT in 3, and with modified 6 Fr IFT in two cases. The surgical procedure was completed in 4 neonates out of 5 cases, while in one infant it was deferred due to the clinical condition of the patient and significant SGS. Conclusion: All 5 patients with SGS were managed without performing a tracheostomy. Modified IFT(s) is an effective alternative if smaller sized ETT(s) cannot be negotiated or unavailable in difficult neonatal and paediatric airway. This modified IFT as ETT has to be an important part of the emergency airway tray. It should not be considered as a replacement for routine use of standard ETT.

Partial cricotracheal resection for congenital subglottic stenosis

Partial cricotracheal resection for congenital subglottic stenosis

Subglottic Stenosis in Children: Preliminary Experience from a Tertiary Care Hospital.

Introduction This retrospective study describes our experience in the evaluation and management of infants with subglottic stenosis. Materials and Methods The study included 10 patients aged between 1 wk and 18 months with 6 cases having congenital subglottic stenosis and 4 cases having acquired subglottic stenosis. Results 6 patients had grade I, 3 patients had grade II, and 1 patient had grade III subglottic stenosis. Tracheostomy was required in 4 patients at the time of presentation. 7 patients were treated successfully with Bougie dilation followed by topical application of mitomycin, whereas 1 patient who failed to serial dilation needed open reconstructive procedure. Laser excision of the anterior subglottic web was performed in one patient. Another patient with underlying cerebral palsy could not be operated upon and was managed with tracheostomy. Conclusion Subglottic stenosis may be effectively man-aged with endoscopic surgical techniques, although the number of such sittings required varies with the type and severity of stenosis. Open surgical procedures need to be individualised as per the needs of the patient only after all the other endoscopic possibilities have been exhausted.

Malformed cricoid cartilage causing congenital subglottic stenosis: A rare case report

Subglottic stenosis (SGS) may be congenital or acquired and is described as narrowing of the airway beneath the vocal cords up to the lower border of the cricoid cartilage. The acquired SGS is usually due to airway trauma following prolonged intubations while congenital SGS is caused by a developmental defect or may occur as an association with other congenital anomalies. Manifestation of congenital SGS in infants ranges from asymptomatic to frank stridor and the anaesthetic management of these infants is challenging. We report one such case which emphasises the nature of such rare lesions-silent yet life threatening.

Laser-Assisted Endoscopic Cricotracheal Stenosis Resection (CTSR) in Paediatric Congenital Cartilaginous Subglottic Stenosis.

Subglottic stenosis (SGS) in children can be a congenital condition or acquired through injury such as from prolonged intubation. Surgeons face challenges in choosing the best SGS treatment for a particular patient because of variability in the success rate of each technique. Conventional open surgical resection and reconstruction have been proven effective but, in recent years, endoscopic surgery has become more prevalent as it eliminates the incision and reduces the surgery time and subsequent hospital stay. The purpose of this retrospective case study was to report on an endoscopic technique using a CO2 laser for cricotracheal stenosis resection (CTSR) for high-grade congenital SGS. From forty-five paediatric patients who underwent endoscopic intervention as a primary modality of treatment for high-grade SGS in a tertiary referral centre, a total of eight patients who met the inclusion criteria have been included in our study. This small patient series is the first to use a CO2 laser alone as a single excision tool to eliminate complex congenital SGS and restore airway patency. The procedure's goal was to return the airway to an early stage of postintubation injury prior to scar formation; therefore, surgical sessions at intervals of 2–3 weeks were performed to ensure natural epithelization, to remove any granulation tissue, and manage fibrosis. Successful treatment was defined as a resolution of symptoms, restoration of a normal patent airway with no stenosis, and decannulation. The success rate was 75%. Two outcomes need to be highlighted. First, the CO2 laser should be reconsidered as an excision tool for congenital SGS because of its low risk of exacerbating preexisting stenosis. It allows the surgeon to restore and augment the airway without the need for open surgery or dilatation. Second, the shorter interval between procedures is crucial for controlling the healing process and making sure that it is proceeding properly.

Pediatric airway management in undiagnosed congenital subglottic stenosis patients

Pediatric unanticipated difficult airway in cases of subglottic stenosis (SGS) is always a challenge. We present two cases of SGS which were managed according to the pediatric difficult intubation protocols where supraglottic device was used as secondary intubation plan successfully. Airway management in these cases depends on the urgency of the surgery; if the surgery is nonemergent, the case can be postponed and both surgical and nonsurgical modalities of treatment can be offered; however, in wake of emergency surgery, surgical airway is indicated. Strategies of the pediatric airway management have evolved over the years, but the risk and morbidity associated with the adverse airway events loom larger.

Slide laryngotracheoplasty for congenital subglottic stenosis in newborns and infants.

Subglottic stenosis is the most common laryngeal anomaly necessitating tracheostomy in early childhood. Crico- and laryngotracheal resection and laryngotracheal reconstruction-usually with autologous cartilage graft implantation-are the most effective treatments. These surgical techniques are obviously challenging in neonatal age and infancy. However, a reconstructive surgery performed at early age may prevent the sequel of complications. The authors present their novel surgical method for congenital subglottic stenosis. Seven infants had inspiratory stridor; two of them had to be intubated and one required tracheostomy. Laryngotracheoscopy, CT or MRI revealed subglottic stenosis: Cotton-Myer grade II in one, and grade III in six cases. Slide laryngotracheoplasty was performed before 5 months of age (10-130 days), with a follow-up period of average 36 months (4-80 months). Phoniatry and quality of life questionnaire were used for evaluation of postoperative results. Slide laryngotracheoplasty in the neonatal age made the temporary tracheostomy unnecessary. All babies remained intubated for 3 to 10 days with an uncuffed tracheal tube. After extubation, no dyspnea or swallowing disorder occurred. A subjective quality of life questionnaire, laryngotracheoscopy, clinical growth charts showed satisfactory functional results. Single-stage slide laryngotracheoplasty might be a favorable solution for subglottic stenosis, even in early childhood. In one step, the airway can be maintained without stenting and tracheostomy. 4 Laryngoscope, 130:E199-E205, 2020.

Difficult intubation: A real neonatal resuscitation nightmare

Abstract: Subglottic stenosis can be congenital or acquired and defined as the presence of an abnormally small subglottic lumen (<3.5 mm in the newborn period). Patients with congenital subglottic stenosis will present with mild to severe airway obstruction at birth.

Bilateral Cricoarytenoid Joint Ankylosis with a Perplexing Etiology

Bilateral Cricoarytenoid Joint Ankylosis with a Perplexing Etiology

Congenital laryngeal webs and congenital subglottic laryngostenosis. Surgical approach

Objective: To analyze surgical approaches in various options of congenital laryngeal webs and congenital subglottic stenosis of larynx in consideration of the suggested clinical-morphological classification. Method: Retrospective study of medical records of the patients with congenital subglottic stenosis of larynx examined and treated at Saint Petersburg State Pediatric Medical University. Results: At the period of 1998 to 2017, otorhinolaryngology clinic SPbGPMU observed 30 children with various types of congenital subglottic laryngeal stenosis and congenital laryngeal webs at the age of 24 hours to 5 years.

Asymptomatic congenital subglottic stenosis in a neonate - infant feeding tube as a "Guardian angel!"

Asymptomatic congenital subglottic stenosis in a neonate - infant feeding tube as a "Guardian angel!"