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

OBJECTIVES: Slide 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. METHODS: We 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. RESULTS: All 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. CONCLUSIONS: Slide 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.

Slide laryngotracheoplasty for congenital subglottic stenosis in newborns and infants.

OBJECTIVES: 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. METHODS: 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. 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. CONCLUSIONS: 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. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:E199-E205, 2020.

Serial intralesional triamcinolone acetonide injections for acquired subglottic stenosis in premature infants.

PURPOSE: Long-term intubation of premature infants sometimes induces acquired subglottic stenosis (SGS), causing glottic or supraglottic problems. These kinds of SGS often require tracheostomy and subsequently make decannulation difficult. The aim of our study was to clarify the efficacy of repeated intralesional steroid injections to the stenosis. METHODS: Six children with acquired SGS, who were treated with triamcinolone acetonide injections to the subglottic space just below the vocal folds between September 2015 and December 2017 were retrospectively reviewed. RESULTS: The patients' mean age was 4.3 (range 1.3-4.4) years, the mean gestational age at birth was 25 (23-28) weeks, and the mean birth weight was 591 (456-734) g. The degree of SGS was grade II in one patient and III in five patients, with both tracheostoma and glottic or supraglottic abnormalities. They generally underwent ten procedures, every 3-4 weeks. In most cases, the patency of the injected space improved by 25-220%, and the symptoms were relieved. One patient achieved decannulation, and another one underwent laryngotracheal reconstruction and decannulation. Two patients started using a speech cannula. There were no severe complications. CONCLUSION: Serial intralesional steroid injections are likely to be effective in improving the patency of acquired SGS.

Congenital and iatrogenic laryngeal and vocal abnormalities in patients with 22q11.2 deletion.

BACKGROUND: Voice abnormalities often go unrecognized in patients with 22q11.2 deletion because speech abnormalities become the focus of evaluation. OBJECTIVE: To analyze voice and vocal fold abnormalities in patients with 22q11.2 deletion by examining voice, not speech. METHODS: This is a case series with chart review from 2009 to 2016. Records of both a velocardiofacial syndrome (VCF) clinic and pediatric otolaryngology clinic at a tertiary pediatric hospital were reviewed. All patients with confirmed 22q11.2 deletion were identified. Cardiac, otolaryngological, speech, and voice characteristics were recorded along with surgical history. Main outcomes included voice characteristics based on speech therapy assessment, along with vocal fold or laryngeal abnormalities. Co-morbidities and surgical history were also recorded. RESULTS: 109 patients were identified with 22q11.2 deletion by genetic testing. Eighteen percent (n = 20) displayed a vocal fold or laryngeal abnormality, either congenital or iatrogenic. The most common congenital abnormalities were laryngeal web (n = 10) and subglottic stenosis (n = 7). The most common iatrogenic abnormality was paralyzed left vocal fold (n = 4). Perceptual analysis by speech therapy showed 65% (n = 71) with a voice within normal limits while 17% (n = 19) were not within normal limits. Of the 20 patients with a vocal fold abnormality, 15% (n = 3) presented a voice within normal limits, while 65% (n = 13) were not within normal limits. Of the 19 patients with a voice outside normal limits, 68% (n = 13) had a diagnosed vocal fold abnormality. Sixteen percent (n = 3) of these patients had an abnormal voice as a result of other issues. CONCLUSION: Out of 109 patients with 22q11.2 deletion, 18% displayed a laryngeal abnormality and 17% had abnormal vocal quality. This suggests that voice and vocal fold abnormalities are important to consider in this population. Distinguishing between speech and voice abnormalities in patients with 22q11.2 deletion can help ensure appropriate intervention.

[Laryngotracheal stenosis in children].

Laryngotracheal stenosis includes congenital laryngotracheal stenosis and secondary laryngotracheal stenosis. Congenital laryngotracheal anomalies are rare, accounting for 0.3%-1.0% of all laryngotracheal stenosis(LTS). Despite the management of pediatric LTS over the past 30 years had been considerable improved, primary surgery still failed in a significant number of complex LTS cases. In some severe LTS cases combined with glottic stenosis, the primary surgery failed around 30%, even with the latest available techniques. Children with LTS usually need multiple operations, and need the tracheostomy tube for months or even years. Therefore, the etiology, clinical features, diagnosis and treatment of laryngotracheal stenosis are summarized in this paper.

Rare Upper Airway Anomalies.

A broad spectrum of congenital upper airway anomalies can occur as a result of errors during embryologic development. In this review, we will describe the clinical presentation, diagnosis, and management strategies for a few select, rare congenital malformations of this system. The diagnostic tools used in workup of these disorders range from prenatal tests to radiological imaging, swallowing evaluations, indirect or direct laryngoscopy, and rigid bronchoscopy. While these congenital defects can occur in isolation, they are often associated with disorders of other organ systems or may present as part of a syndrome. Therefore workup and treatment planning for patients with these disorders often involves a team of multiple specialists, including paediatricians, otolaryngologists, pulmonologists, speech pathologists, gastroenterologists, and geneticists.

Congenital laryngeal anomalies.

INTRODUCTION: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. OBJECTIVES: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. METHODS: Revision article, in which the main aspects concerning airway management of infants will be analyzed. CONCLUSIONS: It is critical for clinicians to understand issues relevant to the airway management of infants.

Congenital laryngeal anomalies / Anomalias congênitas da laringe

Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants. .

Introdução: As anomalias congênitas da laringe estão em risco de uma via aérea instável, sendo essencial que o clínico tenha uma boa compreensão dos problemas relevantes para o manuseio das vias aéreas de bebês. Objetivos: Familiarizar os clínicos com os problemas relevantes para o manuseio das vias aéreas de bebês e apresentar uma descrição sucinta do diagnóstico e tratamento de uma série de anomalias congênitas da laringe. Método: Artigo de revisão, no qual serão tratados os principais aspectos relacionados ao manuseio das vias aéreas de bebês. Conclusões: É essencial que o clínico tenha um bom conhecimento dos problemas relevantes ao manuseio das vias aéreas de bebês. .

Tracheotomy after laryngotracheoplasty: risk factors over 10 years. [Corrected].

BACKGROUND: Subglottic stenosis (SGS) is the most common congenital and/or acquired laryngotracheal anomaly requiring tracheotomy in infants. We sought to determine factors associated with a greater likelihood of tracheotomy in symptomatic infants with SGS who underwent laryngotracheoplasty (LTP). METHODS: Retrospective case series with chart review of patients undergoing single-stage LTP for SGS over a 10-year period (2001-2010) in a tertiary-care pediatric hospital. RESULTS: Twenty-two children (15 boys, 7 girls), with a mean gestational age of 32.5weeks, underwent LTP with and without interpositional grafting, at a median age of 89days. Ten patients (43%) required postoperative tracheotomy. Of patients weighing <2.5kg, 7 of 8 eventually required tracheotomy, while none weighing >5kg needed tracheotomy (p=0.003). The average length of stay for patients with a tracheotomy was 125days, while those without tracheotomy required only 58days (p=0.011). The grade of SGS (p=0.809), gender (p=0.968), age at surgery (p=0.178), and gestational age (p=0.117) were not significantly associated with the need for tracheotomy. Weight at surgery was significantly correlated with the likelihood of needing tracheotomy (p=0.003). CONCLUSIONS: Patients who weighed less than 2.5kg at the time of LTP procedures were more likely to require a postoperative tracheotomy. Children who required tracheotomy had longer lengths of hospital stay.

Management specificities of congenital laryngeal stenosis: external and endoscopic approaches.

OBJECTIVES/HYPOTHESIS: This study aimed to report our experience in the management of congenital laryngeal stenosis and to compare our series and results to published data in the literature. STUDY DESIGN: Retrospective case series. Tertiary referral center for rare pediatric head and neck malformations. METHODS: Medical charts of patients diagnosed with congenital laryngeal stenosis in our institution were reviewed over a 15-year period, from 1996 to 2011. Surgical treatment consisted of an endoscopic procedure, open laryngeal surgery (OLS), or a combination of both. RESULTS: Sixteen patients met the inclusion criteria for the study and were divided in two groups: the endoscopic laryngoplasty (EL) group, with patients who underwent the endoscopic procedure as first-line treatment, and the OLS group, whose patients underwent open laryngoplasty with cartilage graft as first-line treatment. Each group contained eight patients with grade II to IV congenital stenosis. All patients, except one in the EL group, achieved a good result (<50% residual stenosis) at the end of the follow-up. CONCLUSIONS: This case series suggests that EL, with incision of the subglottic laryngeal cartilages with cold steel instruments and balloon dilation, is a safe and effective treatment for congenital laryngeal stenosis grade II to IV. This procedure could be considered as an alternative option to OLS, even as a first-line procedure. An endoscopic procedure does not preclude the possibility for an open laryngeal procedure in case of failure. A prolonged follow-up is mandatory. LEVEL OF EVIDENCE: 4.

[Congenital laryngeal stridor - an interdisciplinary problem]. / Wrodzony stridor krtaniowy­ problem interdyscyplinarny.

UNLABELLED: The most common causes of laryngeal stridor are laryngomalacia (60%), vocal cord paralysis (VCP) (10 %) and subglottic laryngeal stenosis. Majority of cases of VCP are idiopathic, less frequently it is the effect of abnormalities in central nervous system (Arnold - Chiari syndrome, hydrocephalus, neonatal hypoxia). Differential diagnosis should also include anomalies of aortic arch and its branches (vascular rings). The authors present two cases of neonatal congenital laryngeal stridor. In the first case the girl presented with VCP of unknown etiology. The perinatal period was normal, ultrasound of central nervous system and neurologic examination revealed no abnormalities. Due to sustained VCP in control laryngeal ultrasound examinations, tracheostomy was performed in the third month of life. In the second case, stridor was caused by laryngomalacia and subglottic laryngeal stenosis of first grade according to Meyer-Cotton scale (larynx lumen diameter <4 mm). The diagnosis was established by laryngotracheobronchoscopy. In both children angiotomography was performed and vascular ring was diagnosed (aberrant right subclavian artery). Vascular anomaly was suspected in barium X-ray. In both cases echocardiographic examination did not visualize the fourth vessel of the aortic arch. Chest X- rays were normal. Both children had no symptoms caused by vascular ring. CONCLUSION: Diagnosis of congenital laryngeal stridor is an indication for complete evaluation to establish the cause of the airway obstruction. The differential diagnosis should include laryngomalacia, vocal cord paralysis, subglottic laryngeal stenosis, congenital anomalies of large vessels and abnormalities of the central nervous system.

Impact and workload implications on service provision with establishment of a neonatal complex airway service in Scotland.

The objective of this study was to assess if any changes have occurred in the utilization of neonatal services with referral for neonatal airway assessment and how this is related to the establishment of a National Complex Airway Service. A retrospective case-note review was performed for neonates referred for airway assessment from 2004-2010 inclusive. Seventy-nine neonates were referred from throughout Scotland; 10 in 2004-2006, 24 in 2007-2008 and 45 in 2009-2010. The mean gestational age was 35 weeks; 39% were preterm. Stridor was the most common reason for referral (46%). The most common diagnosis was airway malacia (38%). Fifty-three procedures were performed on the ward. In total, 64 microlaryngobronchoscopies were performed; 45 diagnostic and 19 interventional. The most common intervention was supraglottoplasty for airway malacia. Thirty-five separate airway procedures were undertaken. Additional investigations were frequently requested and co-morbidities were common. Since the establishment of the Scottish National Complex Airway Service in 2006, referrals for neonatal airway assessment have increased significantly. The reasons for this include a greater awareness of the service, improved treatment options and increased preterm survival. These neonates frequently have associated co-morbidities and require a repertoire of specialist input. This increase has significant implications for further service provision.

CHAOS in the mirror: Ballantyne (mirror) syndrome related to congenital high upper airway obstruction syndrome.

In the mirror syndrome, maternal edema mirrors fetal edema. The pathogenesis is unknown. The most common etiologic associations are rhesus isoimmunization, twin-twin transfusion syndrome, and viral infections. Less than 10% of reported cases are associated with congenital anomalies. We report a case due to congenital laryngeal stenosis, which also caused congenital high airway obstruction syndrome (CHAOS), characterized by pulmonary hyperplasia and edema or anasarca, related to airway abnormality. The fetal manifestations of the mirror syndrome and CHAOS overlap, but occurrence of the two in the same patient does not seem to have been reported.

Balloon dilation laryngoplasty for subglottic stenosis in children: eight years' experience.

OBJECTIVE: To evaluate outcomes of balloon dilation laryngoplasty for laryngeal stenosis in children. DESIGN: Retrospective study. SETTING: Academic tertiary care department of pediatric otolaryngology. PATIENTS: All children treated with laryngeal balloon dilation (primarily or secondarily following laryngeal surgery) from 2002 to 2010. MAIN OUTCOME MEASURES: Stenosis severity, measured using the Cotton and Myer classification. RESULTS: A total of 44 children ranging in age from 1 month to 10 years (14 [32%] with grade II stenosis, 25 [59%] with grade III stenosis, and 4 [9%] with grade IV stenosis) were included. Twelve children [27%] had congenital laryngeal stenoses, and the in other 32 [7 3%], stenosis was acquired. A total of 52 balloon dilation laryngoplasties were performed, and 37 (71%) were deemed successful. Twenty of the 31 patients undergoing primary dilation (65%) had successful outcomes, and in the other 11 [35%], outcomes were unsuccessful (4 had grade II stenosis and 7 had grade III stenosis) and required either laryngotracheal reconstruction or tracheotomy. Twenty-one balloon dilations were performed as a secondary procedure after recent open surgery; 17 of the procedures (81%) were successful, and thus surgical revision was avoided. CONCLUSION: Balloon dilation laryngoplasty is an efficient and safe technique for the treatment of both primary and secondary pediatric laryngotracheal stenosis.

[Ex utero intrapartum treatment - Management of neonatal congenital high airway obstruction syndrome: Case report]. / Tratamiento extrauterino intraparto - Manejo del recién nacido con síndrome de obstrucción congénita de la vía aérea superior: Informe de un caso.

UNLABELLED: In recent years, the ex utero intrapartum treatment (EXIT), that involves extrauterine fetal intubation prior to delivery, has become relevant for the reduction in morbidity and mortality of neonates affected by congenital high airway obstruction syndrome (CHAOS). CLINICAL CASE: We report the case of the mother of an unborn child at pregnancy week 22, who was diagnosed a congenital pulmonary malformation that precluded intrapartum fetal circulatory deficit and resulted in the conduction of an EXIT technique, with the aim of ensuring fetal blood gas exchange at the time of delivery. CONCLUSIONS: A timely practice of the EXIT technique resulted, by monitoring both maternal and fetal factors that might affect fetoplacental circulation, in the birth of a child whose immediate and long-term outcomes were successful allowing the child live a normal life.

[Etiology of acute upper respiratory tract obstruction in infants: analysis of 12 cases].

OBJECTIVE: To study the etiology of acute upper respiratory tract obstruction in infants. METHODS: The medical data of 12 infants with acute upper respiratory tract obstruction were retrospectively reviewed. The patients received the examinations of laryngoscopy and CT scans for larynx and lungs. RESULTS: All of the 12 infants presented with laryngeal stridor. Eight infants (67%) were diagnosed as congenital simple laryngeal stridor before admission. Based on the clinical features, laboratory examinations, imaging examinations and laryngoscopy, 4 (33%) were definitely diagnosed with thyroglossal ductal cyst, 1(8%) with abscess-emphysema in the posterior wall of pharynx, 1(8%) with cervicallymphangioma, 2 (16%) with subglottic stenosis, and 4 (33%) with acute laryngitis. CONCLUSIONS: Acute upper respiratory tract obstruction is easily misdiagnosed in infants. Thyroglossal duct cyst is a common cause of upper respiratory tract obstruction/laryngeal stridor. It is recommend that laryngoscopy and CT scans for larynx should be performed in infants with laryngeal stridor.

Modeling flow in a compromised pediatric airway breathing air and heliox.

OBJECTIVES/HYPOTHESIS: The aim of this study was to perform computer simulations of flow within an accurate model of a pediatric airway with subglottic stenosis. It is believed that the airflow characteristics in a stenotic airway are strongly related to the sensation of dyspnea. METHODS: Computed tomography images through the respiratory tract of an infant with subglottic stenosis were used to construct the three-dimensional geometry of the airway. By using computational fluid dynamics (CFD) modeling to capture airway flow patterns during inspiration and expiration, we obtained information pertaining to flow velocity, static airway wall pressure, pressure drop across the stenosis, and wall shear stress. These simulations were performed with both air and heliox (helium-oxygen mixture). RESULTS: Unlike air, heliox maintained laminar flow through the stenosis. The calculated pressure drop over stenosis was lower for the heliox flow in contrast to the airflow case. This led to an approximately 40% decrease in airway resistance when using heliox and presumably causes a decrease in the level of effort required for breathing. CONCLUSIONS: CFD simulations offer a quantitative method of evaluating airway flow dynamics in patients with airway abnormalities. CFD modeling illustrated the flow features and quantified flow parameters within a pediatric airway with subglottic stenosis. Simulations with air and heliox conditions mirrored the known clinical benefits of heliox compared with air. We anticipate that computer simulation models will ultimately allow a better understanding of changes in flow caused by specific medical and surgical interventions in patients with conditions associated with dyspnea.

Congenital subglottic web: a rare cause of neonatal stridor.

Laryngeal web is a rare malformation caused by an anomalous embryologic development of the primitive larynx. Most of the reported cases are of the glottic type; the subglottic web is extremely rare. Laryngotracheobronchoscopy plays an essential role in the diagnostic workup of this lesion, and endolaryngeal resection is the preferred treatment whenever a structural cartilaginous subglottic stenosis is not present. The authors present the case of a neonate with a subglottic web diagnosed soon after birth. Management of this rare entity is discussed.

Modeling flow in a compromised pediatric airway breathing air and heliox.

OBJECTIVES/HYPOTHESIS: The aim of this study was to perform computer simulations of flow within an accurate model of a pediatric airway with subglottic stenosis. It is believed that the airflow characteristics in a stenotic airway are strongly related to the sensation of dyspnea. METHODOLOGY: Computed tomography images through the respiratory tract of an infant with subglottic stenosis, were used to construct the three-dimensional geometry of the airway. By using computational fluid dynamics (CFD) modeling to capture airway flow patterns during inspiration and expiration, we obtained information pertaining to flow velocity, static airway wall pressure, pressure drop across the stenosis, and wall shear stress. These simulations were performed with both air and heliox. RESULTS: Unlike air, heliox maintained laminar flow through the stenosis. The calculated pressure drop over stenosis was lower for the heliox flow, in contrast to the airflow case. This lead to an approximately 40% decrease in airway resistance when using heliox, and presumably causes a decrease in the level of effort required for breathing. CONCLUSIONS: CFD simulations offer a quantitative method of evaluating airway flow dynamics in patients with airway abnormalities. CFD modeling illustrated the flow features and quantified flow parameters within a pediatric airway with subglottic stenosis. Simulations with air and heliox conditions mirrored the known clinical benefits of heliox as compared with air. We anticipate that computer simulation models will ultimately allow a better understanding of changes in flow caused by specific medical and surgical interventions in patients with conditions associated with dyspnea.