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Orphanet Journal of Rare Diseases Dec 2011A laryngo-tracheo-esophageal cleft (LC) is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx,... (Review)
Review
A laryngo-tracheo-esophageal cleft (LC) is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx, possibly extending downward between the trachea and the esophagus. The estimated annual incidence of LC is 1/10,000 to 1/20,000 live births, accounting for 0.2% to 1.5% of congenital malformations of the larynx. These incidence rates may however be underestimated due to difficulty in diagnosing minor forms and a high mortality rate in severe forms. A slightly higher incidence has been reported in boys than in girls. No specific geographic distribution has been found. Depending on the severity of the malformation, patients may present with stridor, hoarse cry, swallowing difficulties, aspirations, cough, dyspnea and cyanosis through to early respiratory distress. Five types of laryngo-tracheo-esophageal cleft have been described based on the downward extension of the cleft, which typically correlates with the severity of symptoms: Type 0 laryngo-tracheo-esophageal cleft to Type 4 laryngo-tracheo-esophageal cleft. LC is often associated with other congenital abnormalities/anomalies (16% to 68%), mainly involving the gastro-intestinal tract, which include laryngomalacia, tracheo-bronchial dyskinesia, tracheo-bronchomalacia (mostly in types 3 and 4), and gastro-esophageal reflux disease (GERD). The syndromes most frequently associated with an LC are Opitz/BBB syndrome, Pallister Hall syndrome, VACTERL/VATER association, and CHARGE syndrome. Laryngeal clefts result from failure of fusion of the posterior cricoid lamina and abnormal development of the tracheo-esophageal septum. The causes of the embryological developmental anomalies leading to LC are not known but are thought to be multifactorial. LC appears to be mostly sporadic although some familial cases with suspected autosomal dominant transmission have been reported. The age of diagnosis depends mainly on the severity of the clinical symptoms and therefore on the extent of the LC. Diagnosis is made either based on clinical manifestations or on investigations, such as endoscopy, X-ray, CT scan, performed for other conditions. Differential diagnoses include tracheo-bronchial fistula, gastro-esophageal reflux disease and neurological swallowing disorders, as well as laryngomalacia and laryngeal palsy. Prenatal diagnosis of LC has never been reported, although associated anomalies may be detected on fetal ultrasonography. Once the cleft is diagnosed, it is essential to determine its length to orient the management and treatment approach. Management involves maintenance of satisfactory ventilation, prevention of secondary pulmonary complications as a result of repeated aspirations, and adequate feeding. Endotracheal intubation may be required for respiratory distress in severe cases. Treatment requires endoscopic or external surgery to close the cleft. Surgery should be performed as early as possible to avoid complications related to aspiration and gastric reflux, except in type 0 and type 1 cases in which conservative measures must first be attempted. The prognosis is variable depending on the severity of the LC and associated malformations. Early diagnosis and appropriate treatment and management help to reduce mortality and morbidity.
Topics: Abnormalities, Multiple; Animals; Child; Child, Preschool; Congenital Abnormalities; Endoscopy; Esophagus; Female; Humans; Infant; Infant, Newborn; Larynx; Male; Prognosis; Rats; Trachea
PubMed: 22151899
DOI: 10.1186/1750-1172-6-81 -
Combined laryngeal cleft injection laryngoplasty and salivary botulinum toxin for saliva aspiration.Laryngoscope Investigative... Aug 2022Children with type I laryngeal clefts and sialorrhea can have posterior drooling, aspiration of oral secretions, and respiratory complications. Laryngeal cleft injection...
OBJECTIVES
Children with type I laryngeal clefts and sialorrhea can have posterior drooling, aspiration of oral secretions, and respiratory complications. Laryngeal cleft injection laryngoplasty (LCIL) and salivary botulinum injections (Sal-Bot) have been used separately for short-term treatment of type I laryngeal clefts and sialorrhea. Our goal was to evaluate combined LCIL and Sal-Bot and create recommendations for further treatment based on response to initial treatment.
METHODS
Retrospective chart review of nine patients who underwent direct laryngoscopy and bronchoscopy with combined LCIL and Sal-Bot from 2012 to 2019. Charts were reviewed for patient characteristics, response to treatment, and pre and post-op hospitalizations. Subsequent procedures were performed depending on efficacy of initial treatments.
RESULTS
Nine patients were identified. All had pre-existing neurologic conditions, gastrostomy tubes, and a history of coughing and choking on secretions. Only one patient was able to feed orally (purees). 1 U/kg of botulinum toxin per gland was injected into each parotid and submandibular gland. The average units of botulinum toxin injected was 67 U. The mean laryngeal cleft injection volume was 0.35 cc. Subsequent treatment was based on timing of symptomatic improvement and individual patient factors. Five patients had respiratory-related hospitalizations in year preceding the procedures (median 1.5, range 1-10). Three (60%) had a reduction in respiratory-related hospitalization 1 year post procedure (median 1, range 1-3). One patient died during the follow up period due to continued chronic respiratory failure.
CONCLUSIONS
This is the first study of combined laryngeal cleft injection laryngoplasty and botulinum toxin injections for patients with posterior laryngeal penetration and aspiration of oropharyngeal secretions. We highlight strategies for choosing subsequent procedures based on response to initial treatment.
LEVEL OF EVIDENCE
4.
PubMed: 36000049
DOI: 10.1002/lio2.823 -
Anaesthesia Sep 1994Agenesis of the trachea is a rare anomaly. The main signs are respiratory distress and cyanosis, inability to vocalize and impossible tracheal intubation. In most cases...
Agenesis of the trachea is a rare anomaly. The main signs are respiratory distress and cyanosis, inability to vocalize and impossible tracheal intubation. In most cases concomitant congenital anomalies of the heart, digestive tract or genitourinary tract are present. Endoscopy and X ray studies will confirm the diagnosis. There is no long-term surgical solution because no suitable material for a tracheal prosthesis is available at present, therefore the condition is ultimately fatal. We report a case of tracheal agenesis. After the diagnosis was established the baby's lungs were ventilated for several hours via an oesophageal tube and two broncho-oesophageal fistulae, but she finally died from untreatable respiratory acidosis. Autopsy revealed a Floyd's type III tracheal agenesis and a laryngeal cleft.
Topics: Acidosis, Respiratory; Bronchial Fistula; Esophageal Fistula; Female; Humans; Infant, Newborn; Respiratory Distress Syndrome, Newborn; Trachea
PubMed: 7978136
DOI: 10.1111/j.1365-2044.1994.tb04453.x -
Laryngoscope Investigative... Apr 2022(1) To highlight the important causes of chronic and recurrent cough in children. (2) To discuss multidisciplinary approach to management of chronic/recurrent pediatric... (Review)
Review
OBJECTIVES
(1) To highlight the important causes of chronic and recurrent cough in children. (2) To discuss multidisciplinary approach to management of chronic/recurrent pediatric cough.
METHODS
Review of scholarly articles, guidelines, expert panels via PubMed and Google Scholar.
CONCLUSION
Chronic cough (CC) in children is mainly attributed to persistent bacterial bronchitis, asthma, nonspecific cough, and gastroesophageal reflux disease (GERD) symptoms. A multi-disciplinary approach is cost-effective and aids with earlier diagnosis and appropriate treatment. Congenital or acquired narrowing of the subglottis is the leading ENT cause for recurrent croup (RC) in children. Laryngeal cleft-type 1 is commonly seen in children with recurrent aspiration and CC. Children are usually referred to pulmonologists for wet cough not responding to treatment. Eosinophilic esophagitis (EoE) and GERD should be considered in the differential diagnosis of CC in children with both respiratory symptoms and failure to thrive.Level of Evidence: 2a.
PubMed: 35434349
DOI: 10.1002/lio2.778 -
Frontiers in Pediatrics 2023The aim of this study was to describe a novel surgical technique of endoscopic percutaneous repair in pediatric patients with type 1, type 2 and type 3 laryngeal cleft...
OBJECTIVE
The aim of this study was to describe a novel surgical technique of endoscopic percutaneous repair in pediatric patients with type 1, type 2 and type 3 laryngeal cleft (LC).
METHODS
A retrospective study involving 12 patients with LC was performed at a tertiary pediatric hospital between February 2021 and June 2022. Endoscopic percutaneous repair was performed in all the patients. Information such as demographics, comorbidities, history of tracheostomy and the open approach for the repair, type of cleft and complications were analyzed.
RESULTS
Twelve patients were diagnosed with LC. The median age of the patients at the time of surgery was 8.50 months (interquartile range, 49.50 months). Seven patients had tracheomalacia, four patients had subglottic stenosis, three patients had laryngomalacia. No surgical complications occurred in the 10 patients who underwent the primary procedure. For two patients who underwent a secondary procedure, endoscopic percutaneous repair failed again to heal the cleft. During the follow-up period after surgery, none of the patients had stridor, recurrent pneumonia, feeding difficulties, or dyspnea. Follow-up modified barium swallow postoperatively demonstrated no aspiration in 10 patients. Only the 2 patients with a secondary procedure had intermittent cough while taking large gulps of water. The cure rate of endoscopic percutaneous repairer was 83.3% (95% confidence interval: 73.9%-92.8%).
CONCLUSION
Endoscopic percutaneous repair should be considered as an alternative to the open transcervical approach and the traditional endoscopic approach for type 1, type 2 and type 3 LC.
PubMed: 36911017
DOI: 10.3389/fped.2023.1113894 -
Indian Journal of Otolaryngology and... Sep 2022To emphasize the need for high clinical suspicion in the diagnosis of Laryngeal cleft in paediatric population, to catalogue the pattern of presentation, time to...
To emphasize the need for high clinical suspicion in the diagnosis of Laryngeal cleft in paediatric population, to catalogue the pattern of presentation, time to treatment and the evolution of surgical techniques for Laryngeal cleft repair at our center. A retrospective review of laryngeal cleft cases which presented over a period of 8 years (May 2012-May 2020), from a tertiary care center, was done. Data includes-patient demographics, preliminary investigations, diagnostic methods, type of cleft, surgical steps and post-operative follow up. Extensive literature search was done and we could not find similar studies from South East Asia and the Indian subcontinents. Of the 10 patients 7 were managed surgically and 3 conservatively. There was an equal distribution of type 1 (n = 5) and 2 (n = 5) clefts. 80% cases were males and 9 out of 10 patients had associated congenital anomalies. 80% cases had symptom resolution (75% were managed surgically and 25% managed medically). Surgical intervention should be based on the extent of anatomical defect and the functional impairment caused by cleft such as respiratory problems, persistence of feeding issues despite maximal medical management and feeding therapy. Early surgical management of type I and II clefts have satisfactory outcomes.
PubMed: 36213473
DOI: 10.1007/s12070-021-02479-y -
Indian Journal of Otolaryngology and... Oct 2022Laryngeal cleft is a rare pathology and needs a high index of suspicion for diagnosis. Various classifications are used but the Inglis classification which describes 4...
Laryngeal cleft is a rare pathology and needs a high index of suspicion for diagnosis. Various classifications are used but the Inglis classification which describes 4 grades of cleft is most widely accepted. Grade 3 and 4 clefts are very rare and are usually associated with other congenital abnormalities. Grade 1 and 2 clefts are more common and can be easily corrected with good outcomes. We are presenting our experience with 9 cases of low grade (Grade 1 and 2) laryngeal cleft which were managed successfully in our department. Out of the 9 cases 4 failed conservative management and required a trans-oral repair. All patients improved with management, except one who intermittently developed another episode of pneumonia 2 months' post-surgery. However satisfactory cleft obliteration was confirmed on endoscopy and the patient has been under close follow up ever since. Majority of low grade laryngeal clefts can be managed with dietary modifications and feeding rehabilitation. Those who do not respond usually require surgical repair with near complete resolution of symptoms.
PubMed: 36452777
DOI: 10.1007/s12070-020-02184-2 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Dec 2023This paper focuses on the diagnosis and treatment of disorders of laryngeal airway in children, including congenital anomalies, infection, and tumor of the larynx to...
This paper focuses on the diagnosis and treatment of disorders of laryngeal airway in children, including congenital anomalies, infection, and tumor of the larynx to provide a new technology for managing these diseases. Based on the characteristic of them, the pediatric upper airway is dedicated to the clinical evaluation of airway obstruction and the assessment of the compromised pediatric airway, including clinical evaluation of symptoms, diagnostic endoscopy, and imaging examination. Information on endoscopic techniques used for dealing with different degrees of pediatric airway comprised is provided, also this techniques could diagnose what kinds of airway disorder. For example, determining the a particular laryngeal cleft at the initial otolaryngology encounter, flexible laryngoscopy should be performed. In order to define the extent of any identified cleft, the rigid bronchoscopy should be completed to evaluate for classification of laryngeal cleft including typeⅠ, Ⅱ, Ⅲa, Ⅲb, Ⅳa, Ⅳb under general anesthesia. The decision to pursue any therapy for the disorders of laryngeal airway in children should be based on the severity of the patient's symptoms, endoscopic and imaging examination. There are two medical treatments including nonsurgical therapy and surgical therapy. For example the laryngeal cleft, approximately half of type 1 and select type 2 patients can be managed entirely with nonsurgical therapy. Medical management is multifaceted. Under recommendations from the feeding team, a modified diet with thickened feeds and possibly altered feeding position should be initiated. Endoscopic surgical repair is the current gold standard for definitive repair of type 1, the majority of type 2, and selected type 3 clefts. Finally, information on new techniques used into the future for dealing with the diagnosis and treatment of disorders of laryngeal airway in children in this paper.
Topics: Child; Humans; Infant; Larynx; Laryngoscopy; Endoscopy; Bronchoscopy; Congenital Abnormalities
PubMed: 38114307
DOI: 10.13201/j.issn.2096-7993.2023.12.001 -
JAMA Otolaryngology-- Head & Neck... Jul 2017Referral to a neurologist and imaging play important roles in the management of laryngeal cleft. Swallowing involves a complex series of neuromuscular interactions, and...
IMPORTANCE
Referral to a neurologist and imaging play important roles in the management of laryngeal cleft. Swallowing involves a complex series of neuromuscular interactions, and aspiration can result from anatomical causes (eg, laryngeal cleft), neuromuscular disorders, or some combination thereof. To date, no protocols or guidelines exist to identify which patients with laryngeal cleft should undergo neuroimaging studies and/or consultation with a neurologist.
OBJECTIVE
To establish guidelines for neurologic evaluation and imaging techniques to identify or rule out neuromuscular dysfunction in children with laryngeal cleft.
DESIGN
Retrospective review of the medical records of 242 patients who were diagnosed with laryngeal cleft at a tertiary children's hospital between March 1, 1998, and July 6, 2015. Based on this review, an algorithm to guide management of laryngeal cleft is proposed.
MAIN OUTCOMES AND MEASURES
Data extracted from patient medical records included the type of laryngeal cleft, details of neurologic referral, results of neuroimaging studies, and objective swallow study outcomes.
RESULTS
Of the 242 patients, 142 were male and 100 were female. Mean age at the time of data analysis was 8.7 years (range, 10 months to 25 years), and there were 164 type I clefts, 64 type II, 13 type III, and 1 type IV. In all, 86 patients (35.5%) were referred to a neurologist; among these, 33 (38.4%) had examination findings indicative of neuromuscular dysfunction or dyscoordination (eg, hypotonia, spasticity, or weakness). Abnormal findings were identified in 32 of 50 patients (64.0%) who underwent brain imaging. Neurosurgical intervention was necessary in 3 patients diagnosed with Chiari malformation and in 1 patient with an intraventricular tumor detected on neuroimaging.
CONCLUSIONS AND RELEVANCE
A substantial proportion of patients with laryngeal cleft have coexistent neuromuscular dysfunction as a likely contributing factor to dysphagia and aspiration. Collaboration with a neurologist and appropriate neuroimaging may provide diagnostic and prognostic information in this subset of patients. At times, imaging will identify critical congenital malformations that require surgical treatment.
Topics: Adolescent; Algorithms; Child; Child, Preschool; Congenital Abnormalities; Female; Humans; Infant; Larynx; Male; Neuroimaging; Neurologic Examination; Referral and Consultation; Retrospective Studies; Young Adult
PubMed: 28384788
DOI: 10.1001/jamaoto.2016.4735 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Dec 2023At present, there is no unified gold standard for the treatment of laryngeal clefts. Type Ⅰ laryngeal clefts with mild symptoms can be treated conservatively, such as... (Review)
Review
At present, there is no unified gold standard for the treatment of laryngeal clefts. Type Ⅰ laryngeal clefts with mild symptoms can be treated conservatively, such as thick diet feeding and using proton pump inhibitor to control reflux, while Ⅱ-Ⅳ laryngeal clefts mostly requires surgical intervention.There are many different surgical methods for the treatment of laryngeal clefts, including injection laryngoplasty, endoscopic surgical repair of laryngeal clefts and open laryngoplasty through anterior cervical approach. How to choose a more suitable surgical plan for children is a problem worth discussing. This article will review the literature on the surgical treatment of laryngeal clefts both domestically and internationally, and summarize the current situation and challenges of surgical treatment of laryngeal clefts.
Topics: Child; Humans; Congenital Abnormalities; Endoscopy; Laryngoplasty; Laryngoscopy; Larynx
PubMed: 38114316
DOI: 10.13201/j.issn.2096-7993.2023.12.010