Reconstructive Surgery of the Laryngotracheal Junction. / Rekonstruktive Chirurgie des laryngo-trachealen Übergangs.

The laryngotracheal junction is an anatomical region with special pathophysiological features. This review presents clinical pictures and malformations that manifest pre-dilectively at this localisation in children and adolescents as well as in adults. The diagnostic procedure is discussed. The possibilities of surgical reconstruction are presented depending on the pathology and age of the patient.

Long-term Airway Outcomes and Interventions in Children With Oesophageal Atresia With Tracheoesophageal Fistula: A 20-year Single Centre Observational Study.

BACKGROUND: Airway anomalies, symptoms and interventions are commonly reported in children with oesophageal atresia with tracheoesophageal fistula (OA/TOF). The purpose of this study was to assess the incidence of these airway pathologies and those requiring interventions in the long-term. METHODS: A retrospective case note review of all patients admitted to the Neonatal Unit at the Royal Hospital for Children, Glasgow between January 2000 and December 2015 diagnosed with OA/TOF. Included patients had a minimum of 5 years follow-up. RESULTS: 121 patients were identified. 118 proceeded to OA/TOF repair. 115 patients had long-term follow-up data. Ninety-five (83%) children had one or more airway symptom recorded. Thirty-six (31%) neonates underwent airway endoscopy at the time of their initial OA/TOF repair. Forty-six (40%) children underwent airway endoscopy at a later date due to airway symptoms. Airway pathologies identified included airway malacia, thirty-two (28%), subglottic stenosis, eleven (10%), tracheal pouch, twenty-five (22%), laryngeal cleft, seven (6%) and recurrent fistula, five (4%). Airway interventions included endoscopic division of tracheal pouch, ten (9%), tracheostomy, seven (6%), aortopexy, six (5%), repair of recurrent fistula, five (4%), endoscopic repair of laryngeal cleft, three (3%) and four (3%) required open airway reconstruction for subglottic stenosis. One child (1%) remains tracheostomy dependent. CONCLUSIONS: Long-term airway pathologies are common in children with OA/TOF. Many of these are remediable with surgical intervention. Clinicians should be cognisant of this and refer to Airway Services appropriately.

Superior laryngeal artery originating from the lingual artery.

In most cases, the superior laryngeal artery (SLA) branches from the superior thyroid artery, which, in turn, leaves the external carotid artery. Few dissection studies found previously that the SLA could originate from the lingual artery. We report here probably the first evidence of such a rare anatomical variation found unilaterally in a retrospectively evaluated by computed tomography angiography adult male case. The left SLA left a suprahyoid coil of the lingual artery and continued over the greater hyoid horn to enter the larynx through the thyrohyoid membrane. On both sides, thyroid foramina were found, but only the right one used for the entry of the right SLA. Therefore, the rare SLA origin from the lingual artery can be documented on computed tomography angiograms, which could help during preoperative evaluations and prevent unwanted surgical complications.

Laryngotracheoesophageal Cleft Type IV in a Preterm Neonate. A Case Report and Literature Review.

We present a case of a preterm neonate with a type IV laryngo-tracheo-oesophageal cleft, an uncommon congenital malformation, resulting from the failure of separation of the trachea and the oesophagus during fetal development, often associated with other deformities as well. Data in the literature shows that the long-term morbidity from the entity has declined over the last decades, even though prognosis remains unfavourable for types III and IV. This report emphasizes the complex issues neonatologists are faced with, when treating neonates with this rare disorder in the first days of life, what will raise suspicion of this rare medical entity, and that direct laryngoscopy/bronchoscopy finally depicts the exact extension of the medical condition. At the same time extensive evaluation for coexisting congenital anomalies should be performed. For all the above reasons, these neonates should be treated in specialized tertiary pediatric centers for multidisciplinary prompt management, which may improve, the outcome.

Does the History of Tracheoesophageal Fistula Repair Alter Outcomes of Laryngeal Cleft Repair?

OBJECTIVE: Tracheoesophageal fistula and esophageal atresia (TEA) and laryngeal cleft (LC) can coexist in some patients. The surgery-specific success rate of LC repair in children with associated TEA has not been well described. The aim of the study is to determine if the history of TEA alters the LC repair outcomes. STUDY DESIGN: Case series with chart review. SETTING: Single-institution academic medical center. METHOD: A retrospective review was conducted of patients with LC with and without TEA repair between January 2001 and November 2020. Data collected and analyzed included demographics and clinical characteristics, LC type, and LC with TEA timing of repairs. RESULTS: An overall 282 patients met the inclusion criteria of LC repair: LC (n = 242, 85.8%) and LC + TEA (n = 40, 14.2%). Revision repair was required in 43 patients (15.2%) with 8 (2.8%) needing a second revision repair. The first LC revision rate in the LC group was 36/242 (14.9%) as compared with 7/40 (17.5%) in the LC + TEA group (P = .67). The second LC revision rate in the LC and LC + TEA groups was 7 (2.9%) and 1 (2.4%), respectively. The median time to revision was 5.1 months (interquartile range, 3.45-10.6) in the LC group as compared with 29.2 months (interquartile range, 4.8-44.2) in the LC + TEA group (P = .06). CONCLUSION: The incidence of TEA and LC was 14.2% in our study. Based on our findings, history of TEA repair is not associated with a higher revision rate vs LC alone. The history of TEA repair did not alter the outcomes of LC repair.

Transfixing Anterior Laryngeal Cleft in a Malformed Stillborn at Term: Case Report.

Introduction: Laryngeal clefts (LC) are upper respiratory malformations predominately found in the posterior laryngeal wall. The frequency is 1:10,000, more frequently affect males, and can be syndromic features. There is no report of a transfixing anterior laryngeal cleft. Case report: This diabetic mother at full-term pregnancy delivered a stillborn macrosomic 4780 g dysmorphic stillborn male with left renal agenesis, aortic coarctation, and anterior laryngeal cleft. Conclusion: Anterior laryngeal clefts can occur, and in this case, occurred in association with renal agenesis and maternal diabetes.

Upper Esophageal Sphincter Dysfunction in Children with Type 1 Laryngeal Cleft after Failed Primary Cleft Repair.

Changes in pharyngeal and upper-esophageal-sphincter (UES) motor dynamics contribute to swallowing dysfunction. Children with type 1 laryngeal clefts can present with swallowing dysfunction and associated symptoms which may persist even after the initial endoscopic intervention. This study sought to characterize pharyngeal and esophageal motor function in children with type 1 laryngeal clefts who had persistent presenting symptoms after their initial therapeutic intervention. We retrospectively analyzed high-resolution esophageal manometry studies of children ≤ 18 years old with type 1 laryngeal clefts who had an esophageal manometry study performed for persistent symptoms after an initial repair. A total of 16 children were found to have significantly increased UES resting pressure, UES pre- and post-swallow maximum pressures, and duration of UES contraction during swallows in comparison to nine age-matched controls of children without pharyngeal anatomical abnormalities. There was no difference between UES residual pressures or pharyngeal dynamics between the two groups. UES resting and residual pressures did not correlate with VFFS in penetration and aspiration scores of children with type 1 laryngeal clefts status post repair. Our study is the first to identify specific changes in UES motor function in patients with type 1 laryngeal cleft post initial repair.

Neonatal Laryngotracheal Anomalies.

Respiratory distress in the neonate is one of the most common reasons for referral to a tertiary NICU, accounting for more than 20% of admissions. (1) The cause of respiratory distress can range from parenchymal lung disease to anomalies of any portion of the neonatal airway including the nose, pharynx, larynx, trachea, or bronchi. This review will focus on airway anomalies at or immediately below the level of the larynx. Although rare, those with such congenital or acquired laryngotracheal anomalies often require urgent evaluation and surgical intervention. This review describes 1) the pathophysiology associated with congenital and acquired laryngotracheal deformities in the neonate, 2) the clinical presentation and diagnostic evaluation of these anomalies, and 3) the current medical and surgical strategies available in the NICU and after discharge.

[Outcomes of surgical management of typeâ ¢ laryngotracheal clefts: anterior laryngofissure approach and posterior cartilage graft laryngotracheoplasty].

Objective: Our aim of this study is to describe the outcomes of a series of patients who underwent cleft repair and posterior cartilage grafts laryngotracheoplasty (LTP) from anterior midline cervical approach for type Ⅲ laryngotracheoesophageal clefts (LETC). Methods: A review of patients with type Ⅲ LETC between May 2017 and December 2021 was performed. Demographic features including gender, age at surgery, weight, airway support, feeding status, and airway and other comorbidities were collected preoperatively. Patients were evaluated in breathing, swallowing and phonation postoperatively. The developmental status and morbidities were recorded. Results: Five patients who underwent cleft repair and posterior cartilage grafts LTP from anterior midline cervical approach were included. All patients survived and thrived postoperatively. At last follow-up, 3 patients were able to successfully extubate with acceptable voice, and 2 patients were tracheostomied. Four patients were able to be fed orally without aspiration, and one patient needed to be fed by thick food. Conclusion: The combination of cleft repair and posterior cartilage grafts LTP from anterior midline cervical approach is an effective and safe treatment for type Ⅲ LETC.

Poor correlation between airway fluoroscopy and rigid bronchoscopic evaluation in paediatric tracheomalacia.

INTRODUCTION: Tracheomalacia is a common congenital condition causing stridor in young children. However, the evaluation of these children remains poorly standardised across institutions. METHODS: We conducted a retrospective review of all children undergoing an elective laryngotracheobronchoscopy at a single tertiary paediatric institution between March 2010 and December 2018. Emergency bronchoscopies and children with tracheostomies were excluded. 1163 children undergoing an elective bronchoscopy were included in this study, and 545 children also had an airway fluoroscopy. RESULTS: The median age at bronchoscopy was 17 months, and the majority of children were male. Tracheomalacia was diagnosed in 21.6% of children at bronchoscopy, of these 48.5% had tracheomalacia diagnosed on a previous airway fluoroscopy. Overall, airway fluoroscopy had a low sensitivity (62.3%) and a low specificity (67.5%) for diagnosis of tracheomalacia when compared with bronchoscopy. Increasing severity of tracheomalacia on airway screen significantly predicted a diagnosis of tracheomalacia on bronchoscopy. CONCLUSIONS: Airway fluoroscopy has a low sensitivity and specificity in diagnosis of tracheomalacia and should be used judiciously rather than as a screening tool for children with stridor. However, this imaging technique may be beneficial in investigating children with severe symptoms who have had other conditions such as laryngomalacia excluded.

Early detection of laryngeal cleft in infants by novel technique of flexible endoscopy with sustained pharyngeal inflation.

OBJECTIVE: We report a novel technique of flexible endoscopy with noninvasive ventilation (NIV) and sustained pharyngeal inflation (FE-NIV-SPI) in assessing aeroesophageal tracts (AET) to facilitate early detection of laryngeal clefts in infants. METHODS: Medical charts and flexible endoscopy videos of the children who were diagnosed with laryngeal cleft in a tertiary care hospital between January 2000 and December 2020 were retrospectively reviewed and analyzed. The FE-NIV-SPI technique had been applied to all these children. RESULTS: Totally, 12 infants with laryngeal cleft were identified. This equates to a prevalence of 0.28% in all the children who underwent flexible endoscopy at our institution. Their mean age was 5.0 ± 4.9 months and mean body weight was 4.7 ± 2.3 kg. Nine (75%) infants were referred in without laryngeal cleft diagnosis, which was missed by 11 prior bronchoscopy and 5 computer tomography examinations. With the FE-NIV-SPI technique, the pharyngolaryngeal space could be pneumatically dilated permitting a detailed assessment. All laryngeal cleft types and coexisting AET lesions were visualized at the first FE-NIV-SPI examination with a mean time of 4.2 ± 0.9 min; they were eight Type I, two Type II, and one Type III. Ten (83.3%) infants had coexisting airway malacia. CONCLUSION: Routine use of FE-NIV-SPI technique can help in early detection of laryngeal clefts and other associated AET lesions. Further multicenter collaborative investigations are essential to verify the early detection of this rare and occult lesion of the laryngeal cleft with this technique.

Brachycephalic Obstructive Airway Syndrome.

Dogs presenting with brachycephalic obstructive airway syndrome suffer from multilevel obstruction of the airway as well as secondary structural collapse. Stenotic nares, aberrant turbinates, nasopharyngeal collapse, soft palate hyperplasia, macroglossia, tonsillar hypertrophy, laryngeal collapse, and left bronchial collapse are described as the most common associated anomalies. Rhinoplasty and palatoplasty as well as newer surgical techniques and prudent preoperative and postoperative care strategies have resulted in significant improvement even in middle-aged dogs.

The changing face of paediatric airway endoscopic surgery: An 8-year single surgeon review.

INTRODUCTION: As a recently established division, we sought to reflect on the development of our paediatric airway surgery service, and prospectively examine the diagnoses that underwent microlaryngobronchoscopy (MLB) to help quantify the evolving population demographics of paediatric airway disorders. MATERIAL AND METHODS: This was a prospective longitudinal study conducted of all paediatric MLBs performed by a single surgeon in a tertiary paediatric ENT centre between 2012 and 2019. RESULTS: A total of 1040 MLBs were performed in 498 patients at the paediatric ENT centre of the Royal London Hospital. Median age at first procedure was 19 months. Median follow-up was 48 months. Primary diagnoses were laryngomalacia (21%), subglottic stenosis (SGS - 18%), laryngeal cleft (13%), and normal anatomy (28.3%). Repeat procedures were needed in 39.1% patients, who underwent a median of 2 repeat procedures. SGS (57.7%) constituted majority of the repeat category, followed by laryngeal cleft (12.36%), laryngomalacia (10.15%), unilateral/bilateral vocal cord palsy(4.24%) and laryngeal papilloma(4.24%). Laryngeal papilloma constituted largest number of procedures per patient (Median = 4, IQR = 5.75), followed by subglottic web and SGS. Mean length of stay(LOS) was 0.67 ± 0.96 days(d), with laryngeal cleft cases recording longest mean LOS. There was a steady increase in proportion of day-surgeries across study period [6.9% (2012) vs 59%(2019)]. CONCLUSION: SGS constitutes the major bulk of paediatric airway surgery, reflective of increasing number of premature births and prolonged intubation among neonates. Day-case MLB is a safe and feasible option in selected patients. This long-term data provides useful information to accurately prognosticate patients regarding potential number of repeat procedures for each diagnosis.

Injection laryngoplasty for laryngeal cleft type I in an 8-week-old infant.

A laryngeal cleft is a rare anatomical deformity which is increasingly treated with injection laryngoplasty. Since diagnosis of laryngeal cleft type I is often made between 2 and 5 years of age, this treatment is rarely performed on very young children. In this case, we describe how injection laryngoplasty is performed safely on an 8-week-old child, and we illustrate its added value for the diagnostic process and for temporary symptom relief.

CHAOS: síndrome de obstrucción congénita de las vías aéreas superiores / CHAOS: congenital high airway obstruction syndrome

Resumen El síndrome de obstrucción congénita de vías áreas superiores (CHAOS) es una condición que se caracteriza por la existencia de una obstrucción en las vías áreas altas en el feto, la cual puede ser parcial o completa. Comúnmente es una situación incompatible con la vida, por lo que su diagnóstico prenatal es importante considerando el pronóstico y los diferentes manejos prenatales y posnatales que existen. Presentamos un caso de CHAOS diagnosticado en la semana 21, con una breve revisión de la literatura sobre su diagnóstico, pronóstico y alternativas terapéuticas.

Abstract Congenital high airway obstruction syndrome (CHAOS) is a condition characterized by the existence of an obstruction of the fetal upper airways, which may be partial or complete. It is commonly incompatible with life, so its prenatal diagnosis is important due to the prognosis and the recently described pre and postnatal management options. We present a case of CHAOS in a pregnancy of 21 weeks with a brief review of the current literature about its diagnosis, prognosis and therapeutic alternatives.