Congenital Laryngeal Clefts Research Articles

Consensus recommendations on the evaluation and treatment of congenital laryngeal clefts

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.

A Rare Case of Congenital Laryngeal Cleft in an Adult that Showed Effective Improvement in Dysphagia with Endoscopic Repair

A Rare Case of Congenital Laryngeal Cleft in an Adult that Showed Effective Improvement in Dysphagia with Endoscopic Repair

Задние расщелины гортани

The posterior laryngeal cleft is a rare congenital malformation of the larynx, with an estimated incidence of 1 in 10,000–20,000 children born alive. Despite the apparent obviousness of the pathology, the diagnosis of the posterior cleft of the larynx often causes difficulties, which is associated with a variety of clinical symptoms, primarily due to the varying prevalence of the pathological process. Objective: To analyze the results of treatment of patients with congenital laryngeal cleft. Materials and methods: A retrospective analysis of case histories of children in the department of otolaryngology of the clinic of the St. Petersburg State Pediatric Medical University, from 2003 to 2018, diagnosed with congenital malformation of the larynx, posterior laryngeal cleft, was performed. Results: Normal respiration and nutrition through the natural pathways was achieved in 13 children with type I cleft and in 2 with type IIIa. Three patients with IIIa and IIIb types of clefts could not be rehabilitated due to severe concomitant somatic and neurological pathology, for which they continue treatment with relevant specialists at the present time. Conclusions: Rehabilitation of patients with laryngeal cleft of type IIIa and IIIb, especially in the presence of severe concomitant somatic and neurological pathology, does not always give positive results.

Not Just for Kids: A Rare Case of Congenital Laryngeal Cleft in an Adult

This case report aims to increase awareness of the diagnosis of laryngeal cleft in adult patients and discuss treatment options. Case report and review of the literature. We present a case of a 56-year-old male referred for hoarseness and chronic cough that was discovered to have a type 2 laryngeal cleft. He had chronic aspiration, recurrent pneumonia, and a hoarse voice for his entire life. In contrast to most described cases in adults, this patient was successfully treated with an endoscopic approach using absorbable suture. Laryngeal clefts are uncommon and almost always detected in childhood, making adult laryngeal clefts extremely rare. Endoscopic repair is a feasible and successful treatment option in these cases.

A case of adult congenital laryngeal cleft asymptomatic until hypopharynx cancer treatment

Laryngeal cleft is an anomaly of failed posterior closure of the larynx. Most cases are diagnosed and need treatment early in life due to respiratory and swallowing problems. We report an unusual case of a 66-year-old man with an asymptomatic laryngeal cleft until treatment for hypopharyngeal cancer. During concurrent chemoradiotherapy (CCRT), despite reduced tumor volume, he presented severe dysphagia and dyspnea, followed by severe pneumonia twice. Because CCRT had to be discontinued, a pharyngolaryngectomy was performed for the cancer treatment. The resected specimen showed total removal of the tumor and a total longitudinal cleft of the cricoid cartilage, classified as a type III laryngeal cleft by the Benjamin and Inglis’ classification. A review of computed tomography images indicated that the redundant mucosa from bilateral edges closed the separation of the posterior cricoid cartilage and narrowed the laryngeal airway during CCRT. Adult presentations of laryngeal cleft are quite rare with only ten reported cases in English literature; the present case is of the oldest patient. Undiagnosed cases with laryngeal cleft may exist asymptomatically or without severe symptoms. The awareness of this condition may increase its diagnosis as a cause of diseases such as aspiration and recurrent pneumonia even in adult patients.

The Ram Sign: Detecting Previously Undiagnosed Congenital Laryngeal Clefts in Adults

We present the clinical characteristics of a case series of adult patients with type III laryngeal clefts according to the classification of Benjamin and Inglis, in an attempt to make practitioners aware of the "ram sign," a clinical finding associated with laryngeal clefts in adults. Laryngeal clefts are uncommon defects that are nearly universally identified during infancy as a result of persistent aspiration and pneumonia. Undiagnosed laryngeal clefts in adults are extremely rare. Three type III laryngeal clefts were identified in adults (29, 48, and 60 years of age) from one clinic over an 18-month period. The existing literature features only one type III cleft, to our knowledge. The 60-year-old patient represents the oldest person in the English-language literature to have a newly diagnosed laryngeal cleft. All three cases presented with various degrees of aspiration over an extended period. The computed tomographic imaging and endoscopic findings from these three patients were reviewed. The videolaryngoscopic images demonstrated that the "ram sign"--an endoscopic finding associated with redundant soft tissue overlying the arytenoid cartilages that prolapses into the cleft, creating the appearance of ram's horns--was a consistent and striking feature in all three patients. Although rare, laryngeal clefts may represent an underdiagnosed entity in the adult population. With increased awareness, they may be identified more frequently as a treatable cause of aspiration and recurrent pneumonia.

Injection Augmentation of Type 1 Laryngeal Clefts

To describe a series of children diagnosed with type I congenital laryngeal clefts (LC-I) and treated, due to various presentations, with endoscopic injection augmentation (IA). Case series with chart review. Tertiary care academic children's hospital in Edmonton, Canada. All pediatric patients diagnosed with LC-I and treated with IA in a single tertiary care practice. The children were identified from a prospectively collected database. Only those who were treated with IA and had a minimum follow-up of 3 months were included. The authors collected demographics, diagnoses, surgical procedures, number of IA procedures, clinical outcomes, and complications. Over a period of 8 years, 43 patients were diagnosed with LC-I. Eighteen had undergone IA over the past 4 years. Mean age at IA was 37.11 ± 32.68 months with a male-to-female ratio of 1.25:1. The indications were swallowing dysfunction (13), atypical croup (2), chronic cough (1), cyanotic spells (1), and asthma (1). Seven patients required repeated injections (mean, 2.57 injections). A total of 13 patients responded with resolution of symptoms in question. A single postoperative complication was recorded. IA is a brief, simple management option that succeeds in a number of children with LC-I. It is minimally morbid and supplements other conservative approaches to treat the condition.

Injection Augmentation for Type I Laryngeal Clefts

Objective: Describe a series of children diagnosed with type I congenital laryngeal clefts (LC-I) and treated due to various presentations with endoscopic injection augmentation (IA). Method: This is a retrospective study. The children were identified from a prospectively collected database. Only those who were treated with IA (using hyaluronic acid derivatives) and had a minimum follow-up of 3-months were included. We collected demographics, diagnoses, surgical procedures, number of IA, clinical outcomes, and complications. Results: Over a period of 8 years, 32 patients were diagnosed with LC-I. Fifteen had undergone IA over the past 3 years. Mean age at IA was 28.67 ± 17.54 months with a male-to-female ratio of 8:7. The indications were swallowing dysfunction (13) and atypical croup (2). Seven patients required repeated injections (mean of 2.57 injections). A total of 10 patients responded with resolution of symptoms in question. No record of complications was found. Conclusion: IA is a brief, simple management option that succeeds in a number of children with LC-I. It is minimally morbid and supplements other conservative approaches to treat the condition.

Laryngotracheoesophageal Cleft; Neonatal Presentation and Diagnostic Challenges

Congenital laryngeal clefts are rare developmental disorders of the upper airway accounting for 0.3% to 0.5% of all congenital anomalies of the larynx. The study is reporting the research team's first experience with this anomaly at the Armed Force Hospital Southern Region to emphasize the difficulties and the challenges in the diagnosis as well as the treatment of such infants. This is a 32 week preterm female infant with antenatal ultrasound diagnosis of isolated esophageal atresia. After delivery the team faced a great difficulty in intubation and passing nasogastric tube. A large NGT size 12FR passed to the stomach, and contrast study showed abnormal anteriorly located esophagus, which suggested a common esophageotracheal tract. Esophagogram and soft fibro-optic endoscopy was done by ENT consultant which confirmed the presence of long segment laryngo– rachea-esophageal cleft. The infant was transferred to tertiary center where she died from respiratory failure at the age of 6 months.

Familial occurrence of congenital laryngeal clefts.

Two sibships of double first cousins in which 6 children had congenital laryngeal anomalies are reported. 3 of the children had proven congenital posterior laryngeal clefts, 1 had subglottic stenosis with a deformed cricoid cartilage, and it is likely that the other 2 also had posterior laryngeal clefts. It is suggested that the anomaly is inherited as an autosomal dominant.

Laryngeal and Laryngotracheoesophageal Clefts: Role of Early Surgical Repair

An uncommon congenital anomaly of the developing aerodigestive tract is the congenital laryngeal cleft and its extension, the congenital laryngotracheoesophageal (LTE) cleft. Because of the subtle findings sometimes seen with clefts confined to the larynx, the diagnosis may be difficult. The potential problems of airway obstruction and repeated aspiration frequently associated with laryngeal and LTE clefts mandate early surgical repair. Over the past 3 years, 14 patients with congenital laryngeal or LTE clefts have been managed at Children's Hospital Medical Center, Cincinnati, Ohio. This report details our experience and philosophy regarding the diagnosis and management of the condition, and presents the histopathologic findings of a case of complete LTE cleft in which surgical repair was delayed and death resulted.