Posterior Laryngeal Cleft Research Articles

Repair of posterior laryngeal cleft: a 10-year experience in a tertiary referral hospital.

This study aimed to present experience with surgical treatment of laryngeal cleft cases through both open and endoscopic approaches. A retrospective evaluation of all patients diagnosed as having a laryngeal cleft in a tertiary hospital over 10 years was performed. Pre-operative data, conservative and surgical management of cases, and outcomes were collected, tabulated and analysed. This study included 43 patients aged from 2 to 44 months with a median of 9.19 months. Concerning management technique, 12 patients had conservative treatment and the remaining 31 underwent a surgical procedure (of them, 20 patients underwent endoscopic intervention and 11 had the open surgical technique). In the open group, we used either tibial periosteum (six cases) or harvested costal cartilage (five cases). Surgical management in the form of endoscopic Coblation-assisted or an open approach is indicated in severe cases or mild cases not responding to conservative management.

К вопросу диагностики задней расщелины гортани у новорожденного с множественными врожденными пороками развития

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.

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.

False Cord Retractor: An important Tool in diagnosing Posterior Laryngeal Cleft

ABSTRACT When the tracheoesophageal septum fails to develop or fuse, a laryngeal cleft is formed. It may or may not be associated with a tracheoesophageal fistula. Diagnosing a laryngeal cleft is not straightforward as was found to be in this case. Here, the cleft was masquerading as a laryngeal polyp, which resulted in misdiagnoses and delay in the management. A false cord retractor was of significant help in diagnosing the cleft. We recommend the use of this instrument during evaluation of laryngobronchoscopy in all patients suspected to have a laryngeal cleft. How to cite this article Bradoo R, Dave VJ, Joshi A, Shah K. False Cord Retractor: An important Tool in diagnosing Posterior Laryngeal Cleft. Int J Phonosurg Laryngol 2017;7(1):20-22.

Laryngeal clefts

Laryngeal clefts

Interarytenoid sodium carboxymethylcellulose gel injection for management of pediatric aspiration.

This study aimed to investigate the role of interarytenoid injection laryngoplasty (IL) for the management of pediatric aspiration. Medical records of 30 patients, 9 female, with radiographically confirmed chronic aspiration who underwent intraoperative IL were retrospectively reviewed. Clinical improvement was defined as successful advancement of feeds to thinner consistencies. Clinical improvement was observed in 57% of patients. Six children with type 1 posterior laryngeal cleft (PLC-1) were not significantly more likely to show improvement compared to the children without PLC-1. Type 1 posterior laryngeal cleft was associated with older age and higher prevalence of neurodevelopmental risk factors relative to absence of PLC-1. Patients with PLC-1 were more likely than noncleft patients to show recurrence of symptoms after initial improvement with IL. Five patients underwent endoscopic repair. Repair was successful in 3 patients who improved after IL but not in 2 patients who did not improve after IL. Chronic aspiration can improve after IL even in patients with normal anatomy. Injection laryngoplasty can be performed to improve selection of PLC-1 patients for definitive endoscopic repair. Further prospective research, with a randomized control group, is needed to understand whether interarytenoid incompetence plays a role in some patients with chronic aspiration, who do not have a PLC-1.

Distal Airway Lesions in Mandibular Distraction Patients

Objective: 1) Determine prevalence and type of synchronous airway lesions (SALs) in patients undergoing mandibular distraction osteogenesis (MDO) at Children’s Memorial Hospital. 2) To determine whether these SALs affect surgical success of MDO in relieving airway obstruction. Method: Restrospective review of patients with micrognathia undergoing MDO and direct laryngoscopy and bronchoscopy (DLB) over a period of 11 years (2001-2011) at a tertiary care pediatric hospital. Charts were reviewed for demographics, prevalence/type of SALs, success rate for airway improvement for MDO, and coexisting medical and craniofacial conditions. Results: Eighteen patients were found who had undergone both MDO and DLB. The incidence of at least 1 SAL was 77.8%. Types of SALs encountered include laryngeal edema (61.1%), tracheobronchitis (50%), laryngomalacia (38.9%), tracheomalacia (16.7%), bronchomalacia (16.7%), subglottic stenosis (11.1%), pulsatile airway compression (11.1%), posterior laryngeal cleft (0%), and subglottic cysts (0%). 94.4% of MDO patients received tracheostomy, and 88.9% had airway improvement following MDO. Craniofacial anomalies include micrognathia (100%), cleft palate (61.1%), midface hypoplasia (33.3%), craniosynostosis (5.6%), choanal stenosis/atresia (5.6%), pyriform aperture stenosis (5.6%), and macroglossia (0%). Baseline medical characteristics/syndromes include Pierre Robin sequence (61.1%), Nager syndrome (11.1%), Stickler syndrome (11.1%), asthma (11.1%), Goldenhar syndrome (11.1%), arthrogryposis (5.6%), Mobius syndrome (5.6%), and Cornelia de Lange syndrome (5.6%). Conclusion: There is a high rate of SALs in patients undergoing MDO. The most common SALs are laryngeal edema, tracheobronchitis, and laryngomalacia. MDO is highly successful in relieving upper airway obstruction in micrognathic patients despite the high incidence of SALs. DLB should be considered in all patients who require MDO.

Diagnosis and Management of Type I Posterior Laryngeal Clefts

We review the diagnosis and management of type I posterior laryngeal clefts (PLCs). We performed a retrospective study at a tertiary-care children's hospital of children who were diagnosed with a PLC between January 2003 and August 2008. We studied concurrent airway anomalies, comorbidities, presenting symptoms, age at the time of aspiration resolution, and rate of aspiration resolution. Sixty-seven children with PLCs were identified (41 boys and 26 girls). Fifty-nine had type I clefts, 6 had type II, and 2 had type III. Of the 59 type I cases, 15 (25.4%) were surgically repaired by endoscopy. Eleven of these 15 children (73.3%) have had symptomatic improvement since the surgery, and 7 of those 11 (63.6%) are tolerating thin liquids by mouth. Two of the 15 (13.3%) displayed no improvement with surgery, and 2 of the 15 (13.3%) were lost to follow-up. Forty-four of the 59 type I clefts (74.6%) were managed nonsurgically. Twenty of these 44 children (45.5%) did not present with aspiration. Twenty-four of the 44 (54.5%) presented with aspiration, and 16 of the 24 (66.7%) are now tolerating thin liquids by mouth. Seven of these 24 patients (29.2%) are still aspirating, and 1 has died. The average time to resolution of aspiration was 7.8 months for the surgical group and 13.6 months for the nonsurgical group (p = 0.19). In the surgical group, the average age at resolution of aspiration for patients who received their diagnosis at 0 to 6 months of age was 21.5 months; that for those with a diagnosis at 6 to 12 months was 27.3 months; and that for those with a diagnosis at older than 12 months was 27.3 months (p = 0.31). In the nonsurgical group, the average age at resolution of aspiration for patients who received their diagnosis at 0 to 12 months of age was 15.8 months; that for those with a diagnosis at 12 to 24 months was 27.3 months; and that for those with a diagnosis at older than 24 months was 77.3 months (p = 0.0015). We found that (1) the reported incidence of type I PLCs is increasing; (2) type I PLCs can often present without clinical aspiration; (3) aspiration caused by type I PLCs can be managed medically or surgically; and (4) operative intervention is advantageous for patients who have severe symptoms or who have persistent aspiration after 2 years of age.

A 2.3 Mb deletion of 17q24.2–q24.3 associated with ‘Carney Complex plus’

We present a 12-year-old with a de novo interstitial deletion of approximately 2.3 Mb in chromosome band 17q24.2–q24.3, which was identified by array CGH. The most characteristic features in this case are posterior laryngeal cleft and the presence of numerous freckles and lentigines in childhood. Growth restriction, microcephaly and moderate mental retardation are also prominent features but are frequently seen with other chromosomal anomalies. The microdeletion causes haploinsufficiency of PRKAR1A (protein kinase, cAMP-dependent, regulatory 1α), which is known to cause Carney Complex but this diagnosis alone does not account for all of her problems and she therefore has ‘Carney Complex plus’. This report illustrates the practical benefits associated with a clear cytogenetic diagnosis, as regular endocrinological and cardiac screening is required.

Clinical Aspects of Type 1 Posterior Laryngeal Clefts: Literature Review and a Report of 31 Patients

The clinical relevance of posterior laryngeal clefts (PLCs) of type 1 has only recently been highlighted in the medical literature. We present a review of the literature and a report of a contributive series of 31 type 1 PLC patients. We performed a MEDLINE search of studies published between 1966 and September 2005 and a retrospective cohort study in children aged 0 to 16 years. In the reviewed articles as well as in the presented study, a high degree of comorbidity in patients with PLC type 1 is reported. The majority of patients (90%) with severe problems with oral feedings were treated conservatively by means of nasogastric tube feeding. Increased awareness improves the chances of an accurate diagnosis of type 1 PLC in patients with congenital stridor. Type 1 PLC may be present in all patients with congenital stridor and laryngomalacia, particularly when stridor is accompanied by symptoms such as feeding difficulties and choking.

Malformations congénitales du larynx

Clinical manifestations of laryngeal malformations in children include stridor, dyspnea, hoarseness and sometimes swallowing disorders. Laryngomalacia is the most frequent aetiology, however laryngeal palsy, subglottic stenosis and sometimes subglottic haemangioma or posterior laryngeal cleft can be involved. Endoscopy is the gold standard for the diagnosis of laryngeal malformations. Treatment includes medical and/or surgical techniques selected according to the aetiology.

Diagnosis and management of type 1 laryngeal cleft

Posterior laryngeal clefts have been reported as exceptionally rare congenital anomalies. We demonstrate that type 1 posterior laryngeal clefts are more common than previously described, by reporting a series of 12 type 1 posterior laryngeal clefts diagnosed at our institution over a 12-month period. Typically, type 1 posterior laryngeal clefts are managed conservatively. In our series, 75% of the clefts were treated successfully with endoscopic repair, following failure of conservative management. This suggests surgical repair may be warranted in a greater number of type 1 posterior laryngeal clefts in an attempt to prevent associated morbidity, secondary to aspiration, pneumonia and respiratory distress. We highlight the importance of educating other paediatric specialities in maintaining a high index of suspicion for the presence of a posterior laryngeal cleft when treating patients with suggestive symptoms. This leads to early referral and diagnosis.

Repair of type 1-A and 1-B posterior laryngeal clefts

Posterior laryngeal clefts are part of the continuum of defects that manifest with laryngotracheoesophageal clefts. Repair of these defects is mandatory if the patient is in acute airway distress or suffers unremitting symptoms after conservative treatment. Repair of type-1 A clefts has been performed endoscopically; we describe our method of diagnosis and correction and present a brief case report on endoscopic repair of a type 1-B cleft.

Anterior submucous laryngeal cleft

Upper airway obstruction in the newborn is frequently due to congenital laryngeal anomalies. One of these, the posterior laryngeal cleft, is often associated with subglottic stenosis and respiratory difficulty. This discussion of the anterior laryngeal cleft reports findings in an infant who required intubation immediately after birth and survived only 3 days. The anterior cricoid cleft was associated with severe congenital anomalies including congenital tracheal stenosis.

Asymptomatic laryngeal malformations are common in patients with Pallister-Hall syndrome.

Pallister-Hall syndrome (PHS) comprises hypothalamic hamartoma, polydactyly, pituitary dysfunction, laryngotracheal cleft, imperforate anus, and other anomalies. Some patients with PHS have a bifid epiglottis, a rare malformation. Greig cephalopolysyndactyly syndrome (GCPS) comprises polydactyly with craniofacial malformations without the PHS malformations. Both disorders are caused by mutations in the GLI3 gene. Laryngoscopy on 26 subjects with PHS showed that 15 had a bifid or cleft epiglottis (58%) and none of 14 subjects with GCPS had a cleft epiglottis. The malformed epiglottis was asymptomatic in all of the prospectively evaluated subjects. One additional PHS subject was found to have bifid epiglottis and a posterior laryngeal cleft on autopsy. We conclude that bifid epiglottis is common in PHS but not GCPS. Posterior laryngeal clefts are an uncommon manifestation of PHS and are identified only in severely affected patients. The diagnosis of a bifid epiglottis should prompt a thorough search for other sometimes asymptomatic anomalies of PHS to provide better medical care and recurrence risk assessment for affected individuals and families.

Posterior laryngeal clefts: preliminary report of a new surgical procedure using tibial periosteum as an interposition graft.

To present the preliminary results of a new surgical procedure for posterior laryngeal cleft repair. Retrospective study in an academic tertiary care center. The study included three male patients (age at surgery, 2, 13, and 14 mo). One presented with severe aspiration and cyanotic attacks, the two others with aspiration and recurrent chest infections. The types of laryngeal clefts included complete cleft of the cricoid with varying degrees of tracheal involvement but not further than the first six tracheal rings. Associated malformations included one VATER syndrome, one esophageal atresia, and one tracheoesophageal fistula. Surgery was performed under general anesthesia with nasotracheal intubation. A vertical anterior laryngofissure was performed. The mucosal margins of the clefts were incised and then repaired in two layers with polyglactin sutures. The original feature of this procedure was the interposition of a small piece of tibial periosteum between the two layers. This fascia graft is known to be strong and resistant in cleft palate surgery. Clinical and endoscopic follow-up was used for evaluation of results. The three patients had successful laryngeal repair at a mean follow-up of 6 months (range, 4-14 mo). The anterior laryngofissure provides a good surgical access to the cleft. The interposition of tibial periosteum allows durability of the cleft repair. A longer follow-up is needed to confirm these preliminary results. A computed tomography scan study and a study on the rabbit are planned in order to evaluate the outcome of these periosteal grafts.

Type I posterior laryngeal clefts.

Posterior laryngeal clefts (PLCs) are described in the literature as rare laryngeal abnormalities. The authors believe type I clefts are much more common than previously reported. In two busy pediatric tertiary care centers, such clefts are the second most common congenital laryngeal finding at rigid endoscopy, second only to laryngomalacia. PLCs frequently present with symptomatology that can be attributed to other common disease processes and are often undiagnosed unless the surgeon maintains a high index of suspicion and specifically examines the posterior glottis by palpation during microlaryngoscopy. This report presents a series of 41 patients with type I PLCs, reviews their subtle and often confusing presenting signs and symptoms, and describes a simple yet reliable method of diagnosis.

Delayed diagnosis of a laryngotracheoesophageal cleft

Laryngotracheoesophageal (LTE) clefts are rare anomalies of the airway. Surgical correction has been performed in all cases reported in the literature for clefts diagnosed during life. Posterior laryngeal clefts (PLC) are defined as minor (type I) to severe (type IV), with types III and IV being further described as LTE clefts. Type I clefts have been observed with an increased frequency of up to 6% of all pediatric direct laryngoscopies performed at a tertiary pediatric referral hospital. These can be managed medically and seldom require surgical correction. A case is presented of a boy whose initial diagnosis of LTE cleft (PLC type III) was made at age 7 years. His presenting complaints were chronic airway symptoms without airway distress. He developed only two episodes of pneumonia. Surgical correction was not performed. This young athlete has been followed for more than 6 years without significant problems. This appears to be the only case found in the literature of the successful management of a LTE cleft that has not required surgical correction. Photographic documentation and suggestions on endoscopic diagnosis are presented.

Management of posterior laryngeal and laryngotracheoesophageal 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.

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.