Laryngotracheal Clefts: History, Evaluation, and Modern-Day Management.

Laryngeal clefts

To review the clinical features, associated congenital abnormalities, management, and morbidity of infants presenting with posterior laryngeal and laryngotracheal clefts. Case series. Great Ormond Street Hospital for Sick Children NHS Trust, London, England. Consecutive sample of 44 patients presenting with posterior laryngeal and laryngotracheal clefts between December 10, 1979, and January 30, 1992. Clinical features, incidence of surgery, and associated morbidity and mortality related to different types of airway cleft. The main presenting features were stridor and aspiration, which were more evident with the more extensive clefts. Twenty-five patients (56%) had associated congenital abnormalities. Fourteen patients (32%) were treated conservatively. Sixteen patients (36%) underwent primary endoscopic surgical repair. Eight patients (18%) underwent primary repair via an anterior laryngofissure; and six patients (14%) underwent primary repair via a lateral pharyngotomy. Eight patients (18%) required revision surgery, two (4%) of them on more than one occasion. Ten patients (23%) required fundoplication to control gastroesophageal reflux. Six patients (14%) died. The identification of an airway cleft requires a high index of suspicion. Morbidity and mortality are reduced by securing the airway, controlling gastroesophageal reflux, and using a multidisciplinary pediatric team. We recommend the anterior laryngofissure because of the ease of surgical access.

Laryngeal cleft (LC) associated with tracheoesophageal fistula (TEF) with or without esophageal atresia (EA) has rarely been described. The purpose of this study is to review our experience, clinical features, management, delay in diagnosis, and complications in children with these anomalies. Retrospective chart review at pediatric tertiary referral center. Patients diagnosed with LC alone or LC and TEF over a 10-year period were included. Data including demographics, type of TEF and LC, comorbidities, symptoms, management, complications and swallowing outcomes were analyzed. There were 161 pediatric patients diagnosed with LC alone and 22 with LC and TEF. In patients with LC and TEF, aspiration was the most common presenting symptom (n = 11, 50%). Seventeen patients (77%, mean age 4 years 7 months) underwent endoscopic repair and five patients (23%) with type I clefts did not require surgery. Two patients required revision surgery. For patients with LC alone, the mean age at repair was 3.70 years (4 months-19.9 years) compared to 4.69 years (8 months-17.83 years) for patients with LC and TEF (P = 0.0187). The postoperative swallowing studies from 15 patients showed no aspiration. Mean follow-up was 4 years and 6 months. The diagnosis and management of LC in patients with TEF is often delayed. If a child presents with persistent aspiration after TEF repair, a complete airway endoscopy should be performed to evaluate for vocal fold mobility and cleft. Endoscopic repair is the recommended approach for those patients requiring surgical intervention. 4.

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.

Assessment of the feeding Swallowing Impact Survey as a quality of life measure in children with laryngeal cleft before and after repair

The lipid laden macrophage index as a marker of aspiration in patients with type I and II laryngeal clefts

This case report describes five related patients presenting with signs and symptoms of chronic aspiration who were found to have laryngeal clefts requiring surgical intervention. This case highlights a possible underlying genetic cause of laryngeal cleft and need for further study in this area. A retrospective review of patients' clinical presentations, surgical intervention, procedural photographs and imaging studies was conducted. Five members of a family of 6 demonstrated symptoms consistent with chronic aspiration that became more evident during the COVID pandemic due to social isolation and perceived social pressure around coughing. One child had a history of swallowing difficulty and was found to have a laryngeal cleft on operative evaluation. This led to further evaluation of the rest of the family, with all 4 children and their father having an identifiable laryngeal cleft. All showed marked improvement after surgical repair. Genetic testing was performed and identified a familial variant of uncertain significance in the FBN1 gene (c.466A > C; p.N156H). An unidentified genetic or environmental component may contribute to laryngeal cleft. There is insufficient reporting of case of related patients to identify an inheritance pattern or specific genetic mutation. Further case reporting and study is needed in this area.

Type 1 laryngeal cleft: Establishing a functional diagnostic and management algorithm

Background: Laryngeal clefts are rare congenital anomalies of the upper aerodigestive tract with a persistent connection between the posterior laryngotracheal airway and the oesophagus. The purpose of this study was to review the clinical presentation, management and outcomes of a cohort of infants presenting with laryngeal clefts to a tertiary surgical neonatal intensive care unit in the newborn period. Methods: A single centre retrospective case review was conducted on infants with a diagnosis of types I to IV laryngeal cleft. Results: Eight infants with laryngeal clefts were identified. The median age at presentation was 1.5 days (1–118 days) and median age to diagnosis was 6.5 days. Seven out of eight neonates were male and five were born prematurely. Seven out of eight had a significant comorbidity and four infants died. Conclusions: Patients with laryngeal clefts in this study had a high morbidity and mortality despite various forms of surgical intervention.

The article describes a rare congenital anomaly — laryngeal cleft in a newborn. Laryngeal anomaly occurs with a frequency of 1 per 10–20 thousand newborns. There are 4 types depending on the lesion depth and tracheoesophageal fistula presence. Clinical manifestations are characterized by diverse nonspecific symptoms in the form of dysphagia, cough, aspiration, stridor. Condition severity is determined by the anomaly depth and degree, as well as concomitant somatic and neurological child pathology. There is no prenatal diagnosis. The gold standard of diagnostics is videolaryngoscopy, in which a doctor can study in detail the structure of the laryngeal cartilage and vocal cords from any angle, and, if necessary, take photos and videos. Medical and surgical treatment of the laryngeal cleft is quite complicated and depends on the cleft type and size, concomitant pathology, as well as the condition of the child. The article presents a clinical case of a type III laryngeal cleft in a newborn with multiple congenital anomalies, and a diagnostic search for a cleft. An infant at the age of 7 months underwent the endoscopic suturing of this defect, which allowed restoring enteral nutrition and normalizing the child nutritional status. KEYWORDS: dysphagia, laryngeal cleft, congenital anomalies, newborn, diagnosis, endoscopic correction. FOR CITATION: Petrova V.I., Dmitriev A.V., Fedina N.V., Zaplatnikov A.L. On the issue concerning diagnosis of the posterior laryngeal cleft in a newborn with multiple congenital anomalies. Russian Journal of Woman and Child Health. 2023;6(4):433–437 (in Russ.). DOI: 10.32364/2618-8430-2023-6-4-17.

Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), and laryngeal cleft are rare congenital anomalies that often occur together. Previous reports have established a link between EA/TEF and laryngeal cleft, but there have been no large case series to further characterize this relationship. To assess the prevalence of laryngeal cleft among patients with EA/TEF, identify associations between types of laryngeal cleft and EA/TEF, and identify factors associated with the timing of diagnosis for both conditions. Retrospective analysis of 270 patients. The Esophageal Atresia database was used to identify patients seen at the Esophageal and Airway Treatment (EAT) Center at Boston Children's Hospital, Boston, Massachusetts, a tertiary referral hospital, from August 1, 2009, to August 1, 2016. Patients were included if they were younger than 18 years at the time they were diagnosed with EA, TEF, or both and had undergone a procedure or examination by a surgeon from the EAT Center. Patients with acquired airway or esophageal problems were excluded. Data regarding patient demographics, primary diagnoses, dates of diagnosis, comorbidities, and airway/esophageal surgical interventions were collected and analyzed. Association between type of EA/TEF and laryngeal cleft type was evaluated. Secondary outcomes were age at diagnosis between patients who resided in Massachusetts and those who resided outside the state. Of 270 patients diagnosed with EA/TEF during the 7-year period, 138 (51.1%) were male, and the median age at diagnosis of EA/TEF was 1.0 years (IQR, 0.4-2.0) and at diagnosis of laryngeal cleft was 1.1 years (interquartile range [IQR], 0.6-2.8). Laryngeal cleft was diagnosed in 53 of the 270 patients (19.6%) (95% CI, 14.9%-24.4%). No apparent difference was found in the distribution of types of laryngeal cleft by type of EA/TEF. Among patients with EA/TEF type A or B, 9 patients (56%) had a type I cleft, 6 (38%) had a type II cleft, and 1 (6%) had a type III cleft. Among those with EA/TEF type C or D, 20 (57%) had a type I cleft, 11 (31%) had a type II cleft, and 3 (9%) had a type III cleft. Out-of-state patients were more likely to be diagnosed with EA/TEF at an older age than in-state patients (mean difference, 1.5 years; 95% CI, 0-2.9 years). Pediatric patients with EA/TEF have a much greater prevalence of laryngeal cleft than the general population. Multidisciplinary esophageal and airway programs serve as an ideal clinical setting for management of EA/TEF.

Early diagnosis and assessment in children with type 1 laryngeal cleft are essential in preventing aspiration and associated comorbidity. Appropriate use of conservative and surgical interventions in an evidence-based management strategy can improve overall outcome. To evaluate the management of care for children with type 1 laryngeal cleft in our practice and develop an updated management algorithm. We performed a review of medical records at a tertiary pediatric aerodigestive center. During a period of 7 years (July 18, 2005, to July 18, 2012), 1014 children younger than 18 years were evaluated for aspiration, choking, cough, or recurrent pneumonia. Of these, 44 children (4.3%) had a type 1 laryngeal cleft. Two were lost to follow-up; thus, 42 children were included in our final sample (28 males, 14 females). The care of 15 patients (36%) was managed conservatively, and 27 patients (64%) underwent endoscopic surgical repair of their laryngeal cleft. Assessment of our current management strategy. Success was defined as improving when a child was able to tolerate a feeding without aspirating or resolved when the child had transitioned to tolerating thin liquids. All patients received a trial of conservative therapy. Fifteen of the 42 patients (36%) had an anatomic cleft and were able to maintain the feeding regimen; thus, conservative treatment was successful in this group. The remaining 27 patients (64%) received surgical intervention. Overall operative success rate was 21 of the 27 patients (78%). The age of the child (P < .01) and comorbid conditions (P < .001) affected the outcomes of conservative measures and surgical repair. Only 6 patients did not demonstrate resolution, 5 of whom had significant comorbidities. Age, comorbidity status, severity of aspiration, and the ability to tolerate a feeding regimen should be taken into account when deciding on conservative or surgical management for children with a type 1 laryngeal cleft. A clinical pathway for conservative and surgical management is presented.

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. To establish guidelines for neurologic evaluation and imaging techniques to identify or rule out neuromuscular dysfunction in children with laryngeal cleft. 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. 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. 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. 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.

Anterior repair of posterior laryngeal and laryngotracheal clefts

The etiologies of pediatric dysphagia are complex and varied, and the impact of medical and surgical interventions for laryngeal anomalies on pediatric swallow function is still being defined. We aim to review recent literature on evaluation and management of pediatric dysphagia for children with laryngomalacia (LM) and laryngeal cleft (LC). A review of the literature over the last 5 years was completed using search engines PubMed and Google Scholar. Specific journals reviewed include “The International Journal of Pediatric Otorhinolaryngology,” “Dysphagia,” “JAMA Otolaryngology Head and Neck Surgery,” and “The Laryngoscope.” Articles were identified which discuss management of laryngomalacia and laryngeal cleft and the impact on pediatric dysphagia. Management of pediatric dysphagia for patients diagnosed with LM and LC requires multiple considerations including timing of interventions, presence of comorbidities, and coordinated care with multidisciplinary teams. Evaluation of swallow function with the appropriate test at the right time interval is critical for improved patient outcomes and patient safety. Trends toward use of evidence-based clinical pathways are encouraged and supported by recent literature.