OBJECTIVE: To describe the clinical outcomes of patients who underwent surgical repair through an anterior approach that involved interposition a posterior cartilage for Type III or Type IV laryngotracheoesophageal cleft (LTEC).
METHODS: A chart view was performed on patients with Type III or Type IV LTEC between May 2017 and May 2022. Demographic features and surgical outcomes were collected and analyzed.
RESULTS: Seven patients were finally included. Five patients were diagnosed with Type III LTEC and two patients were diagnosed with Type IV LTEC. All but one patients survived and thrived. Four patients were able to successfully extubate with acceptable voice, and two patients were tracheostomized. Five patients were deemed safe for all consistencies food and one was safe for thickened food. After a mean follow-up of 49 months (18-83 months), neither complications nor recurrences were observed.
CONCLUSIONS: An anterior laryngofissure approach to the cleft repair with a posterior cartilage grafting is an effective and safe treatment for Type III or IV LTEC, which enables closure of LTEC and reconstruction of cricoid plate in order to avoid tracheoesophageal fistula formation or subglottic stenosis postoperatively. Severe tracheomalacia and GERD are two main causes for surgical failure.

Congenital laryngeal cleft is a rare airway malformation, mainly manifested as choking, feeding difficulties, which affects the growth and development of children. Patients with a severe laryngeal cleft may have recurrent aspiration, leading to cyanotic spells, or even death. Advances in development of endoscopic techniques have made early diagnosis possible. Depending on the degree of cleft, management may involve a variety of approaches ranging from medical management alone to open repair. Therefore, it is important for pediatric ENT doctors to diagnose and evaluate in clinical practice. This consensus statement, developed by the Pediatric otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance on diagnosis and management of laryngeal cleft, based on symptomatology, physical examinations, and laboratory tests.
摘要: 先天性喉裂是一个罕见的气道畸形，主要表现为呛咳、喂养困难等，影响患儿的生长发育；严重的喉裂可以表现为严重的呛咳误吸，导致阵发性青紫，甚至死亡。随着内镜技术的发展，使得喉裂可以被早期发现和诊断。不同类型的喉裂治疗方案差异较大，早期的诊断与评估对于儿童耳鼻喉科医生至关重要。中国医师协会儿科医师分会儿童耳鼻咽喉专业委员会联合全国多家儿童医疗中心讨论拟定诊治共识，从症状学、客观检查等方面给出喉裂的诊断治疗方案，为临床规范化诊疗提供指导性意见。.

BACKGROUND: Laryngotracheoesophageal cleft (LTEC) is a rare disease in which the larynx and trachea communicate posteriorly to the esophagus. It is often associated with other congenital malformations, particularly gastrointestinal anomalies. Herein, we report a case of LTEC associated with a gastric polypoid lesion in bronchial tissue.
METHODS: A gastric mass was detected in a male fetus since week 21 of gestation using fetal ultrasonography. Esophagogastroduodenoscopy performed after birth revealed a pedunculated polypoid lesion of the gastric fornix. The patient experienced frequent vomiting and aspiration pneumonia, which persisted after nasoduodenal tube feeding. Communication between the airway and esophagus was suspected. Laryngoscopy performed 30 days later revealed an LTEC (type III). Partial gastrectomy was performed when the patient was 93 days of age. Histopathological examination revealed tumor consisting of cartilage tissue covered with a layer of respiratory epithelium.
CONCLUSIONS: The gastric tumor associated with LTEC exhibited structures mimicking bronchial tissue. LTEC occurs because of foregut maldevelopment, and the tumorous respiratory tissue in the stomach may have been formed from the same abnormal foregut development event as LTEC.

Laryngotracheoesophageal clefts (LTECs) and tracheoesophageal fistulae (TEF) are important structural causes of aspiration requiring bronchoscopy for diagnosis. Determining which children are at greatest risk for LTEC and TEF would enable clinicians to be more selective in performing bronchoscopy.
METHODS: Medical records of children aged 0-18 years who underwent flexible and rigid bronchoscopy for evaluation of dysphagia with aspiration were collected and analyzed to identify predictors of LTEC and TEF.
RESULTS: Seventy-two children age 2 months to 9 years were identified. LTEC was identified in 19 (26%) and TEF was identified in 1 (1.3%). One-third of the cohort was born preterm (median gestational age 34 weeks). The proportion of LTEC in those born preterm was lower than that of those born full-term (12% vs. 34%, p = .03). There was no statistically significant difference in LTEC prevalence based on age, midline defects, laryngomalacia, tracheomalacia, history of TEF repair, silent aspiration, or viscosity of barium aspirated during videofluoroscopic swallowing studies. Bronchoalveolar lavage fluid cytology, lipid-laden macrophage proportions, and culture results were similar among those with and without LTEC.
CONCLUSIONS: Children with dysphagia and tracheal aspiration born full-term are three times more likely to have LTEC than those born preterm. Dysphagia in children with a history of preterm birth is more likely to be functional as opposed to structural, however, LTEC was identified in approximately 10% of these children and must be considered. These results support the role of bronchoscopy in children with dysphagia with tracheal aspiration.

Laryngeal webs and laryngotracheoesophageal clefts (LTECs) are rare malformations of the larynx. Both can have a wide range of symptoms. While webs typically affect voice and airway and LTECs typically affect swallowing and airway, this is not always the case. A high index of suspicion is required for timely and accurate diagnosis. A discussion of management and treatment options will be presented. Both endoscopic and open surgical techniques exist for both pathologies and will be reviewed.

Trachea-esophageal defects (TEDs), including esophageal atresia (EA), tracheoesophageal fistula (TEF), and laryngeal-tracheoesophageal clefts (LTEC), are a spectrum of life-threatening congenital anomalies in which the trachea and esophagus do not form properly. Up until recently, the developmental basis of these conditions and how the trachea and esophagus arise from a common fetal foregut was poorly understood. However, with significant advances in human genetics, organoids, and animal models, and integrating single cell genomics with high resolution imaging, we are revealing the molecular and cellular mechanisms that orchestrate tracheoesophageal morphogenesis and how disruption in these processes leads to birth defects. Here we review the current understanding of the genetic and developmental basis of TEDs. We suggest future opportunities for integrating developmental mechanisms elucidated from animals and organoids with human genetics and clinical data to gain insight into the genotype-phenotype basis of these heterogeneous birth defects. Finally, we envision how this will enhance diagnosis, improve treatment, and perhaps one day, lead to new tissue replacement therapy.

Long segment laryngotracheoesophageal clefts (LTECs) are very rare large-airway malformations. Over the last 40 years mortality rates declined substantially due to improved intensive care and surgical procedures. Nevertheless, long-term morbidity, comorbidity, and clinical outcomes have rarely been assessed systematically.
In this retrospective case series, the clinical presentation, comorbidities, treatment, and clinical outcomes of all children with long-segment LTEC that were seen at our department in the last 15 years were collected and analyzed systematically.
Nine children were diagnosed with long segment LTEC (four children with LTEC type III and five patients with LTEC type IV). All children had additional tracheobronchial, gastrointestinal, or cardiac malformations. Tracheostomy for long-time ventilation and jejunostomy for adequate nutrition was necessary in all cases. During follow-up one child died from multiorgan failure due to sepsis at the age of 43 days. The clinical course of the other eight children (median follow-up time 5.2 years) was stable. Relapses of the cleft, recurrent aspirations, and respiratory tract infections led to repeated hospital admissions.
Long-segment LTECs are consistently associated with additional malformations, which substantially influence long-term morbidity. For optimal management, a multidisciplinary approach is essential.

A laryngotracheoesophageal cleft (LC) is a rare congenital anomaly of the upper aerodigestive tract resulting from the absence of fusion of the posterior cricoid lamina, which affects an abnormal communication between the larynx, trachea and esophagus. The genetic etiology of LC remains elusive. The involvement of genetic factors in the development of LC is suggested by reports of familial occurrence, and the increased prevalence of component features among first-degree relatives of affected individuals and murine knockout models. No consistent pattern of inheritance has been found in nonsyndromic patients, except for cases associated with described syndromes. Once the syndrome related to the laryngeal cleft is considered, an active search for the cleft must be initiated. The genetic evaluation of patients with LCs should be guided by the type and location of the malformation, specific medical history and a detailed physical examination. The application of genetic approaches, such as microarrays and exome sequencing might lead to elucidating the etiology of LCs.

OBJECTIVE: A type IV laryngotracheoesophageal cleft (LTEC) is a very rare congenital malformation. Type IV LTEC that extends to the carina have poor prognosis and are difficult to manage. We present our experience with surgical repair in such a case using extracorporeal membranous oxygenation (ECMO).
METHODS: A male infant, who was diagnosed with Goldenhar syndrome, showed severe dyspnea and dysphagia. Laryngoscopy indicated the presence of LTEC. The patient was transferred to our institute for radical operation 26 days after birth. Prior to surgery, a balloon catheter was inserted in the cardiac region of stomach through the lower esophagus to block air leakage, to maintain positive pressure ventilation. We also performed observations with a rigid bronchoscope to assess extent of the cleft, and diagnosed the patient with type IV LTEC. After bronchoscopy, we could intubate the tracheal tube just above the carina. Under ECMO, repair of the cleft was performed by an anterior approach via median sternotomy.
RESULTS: The patient was intubated via nasotracheal tube and paralysis was maintained for 2 weeks, using a muscle relaxant for the first 3 days. Two weeks after surgery, rigid bronchoscopy showed that the repair had been completed, and the tracheal tube was successfully extubated without tracheotomy.
CONCLUSIONS: Although insertion of a balloon catheter is a very simple method, it can separate the respiratory and digestive tracts. This method allowed for positive pressure ventilation and prevented displacement of the endotracheal tube until ECMO was established. As a result, we safely performed the operation and the post-operative course was excellent.

We report a rare case of a neonatal girl who presented with coughing and dyspnea immediately after feeds. At birth, she was noted to have an imperforate anus with a posterior fourchette fistula from which she was stooling. Initial imaging with radiography showed a normal bowel gas pattern; however, lumbar vertebral anomalies were noted. An upper GI series was performed and revealed a laryngeal cleft and malrotation. Ultrasound confirmed malrotation with an abnormal SMA-SMV relationship. Since laryngeal cleft is a rare condition and may not be known to most radiologists, its incidence is likely underestimated. It is important to note the association of laryngeal clefts with VACTERL and malrotation. In addition, it is essential not to confuse a laryngeal cleft with a tracheoesophageal fistula since the management differs.