Diagnosis and management of type 1 laryngeal cleft: systematic review

Laryngeal cleft (LC) poses a diagnostic challenge due to its rarity and non-specific clinical features. There is poor consensus regarding management as either conservative or surgical management may be appropriate for type 1 LC, given the spectrum of disease patients present with. The objective was to review the current literature and propose diagnostic and management pathways for type 1 LC. 
 Methods: The study comprised a systematic review using data collected from PubMed, Medline and Cochrane library. A quality assessment of included studies was performed using a validated tool. A meta-analysis of proportions was performed for the outcome of each treatment modality using MedCalc software. 
 Results: Of 674 potential studies, 19 were included. Complete resolution rates of conservative management, injection augmentation and endoscopic surgery were 52.3% (95% CI, 32.3–71.9%), 69.2% (95% CI, 59.1–78.1%) and 65.4% (95% CI, 49.1–80.1%) respectively. There are research survey, diagnostic and treatment algorithms, and radiological investigations presented in the literature to assist in constructing an updated management algorithm. 
  We recommend that all patients receive initial 3-month trial of conservative therapy. Endoscopic surgery may be considered if conservative management fails or in the presence of other surgical indications. Injection augmentation may be performed prior to definitive surgical repair in selected patients who have comorbidities that increase aspiration risk to evaluate the potential effect of surgery.

Related papers:

title: International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of type I laryngeal clefts.
abstract: The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition.



To provide recommendations for the diagnosis and management of type I laryngeal clefts.



Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method.



Multinational, multi-institutional, tertiary pediatric hospitals.



Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts.



This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here.

title: Laryngotracheo‐oesophageal cleft A report of three cases
abstract: The clincial presentation of cleft larynx is described in a mother, and her first‐born child, and in a third, unrelated child. The symptoms vary with the anatomical severity of the cleft, but may closely resemble those of H‐type tracheo‐oesophageal fistula. Modifications to Pettersson's classfication of the condition are suggested. These allow more precise definition of the milder cases in which surgery carries a reasonable prognosis. The accuracy of clinical diagnosis, especially at laryngoscopy, has been disappointing. The importance of probing the posterior wall of the larynx at direct laryngoscopy is emphasised, since the larynx may appear normal even when an extensive cleft is present.

title: Minor Congenital Laryngeal Clefts: Diagnosis and Classification
abstract: Cleft larynx is a rare congenital anomaly. Detection of an unsuspected minor cleft may be difficult, but the pediatric laryngologist should suspect the possibility of cleft larynx from the clinical features. Four minor clefts are reported, three cases of supraglottic interarytenoid cleft and one of partial cricoid cleft. The technique for endoscopic diagnosis and the distinctive features are described and a classification into four types is proposed.

title: Management of Type I and Type II laryngeal clefts: controversies and evidence
abstract: To summarize the pediatric Type I and Type II laryngeal cleft literature, paying special attention to recent trends, including evolution of surgical techniques, standardization of outcome assessments, and utilization of management algorithms.  There are a variety of options to choose from whenever considering Type I and Type II cleft repair, including endoscopic repair, transoral robotic surgery, and injection laryngoplasty. Conservative management including feeding therapy and treatment of comorbid medical conditions is recommended prior to repair. Validated outcome measures have arisen for swallow study interpretation and timing, as well as caregiver quality-of-life assessment. In addition, a series of medical algorithms have been proposed, which provide specific recommendations for diagnosis, treatment, and follow-up.  For clefts that fail conservative management, endoscopic repair has become the gold standard. In addition, injection laryngoplasty appears to provide both a diagnostic and therapeutic option in the management of these patients. Transoral robotic-assisted endoscopic repair appears well tolerated and feasible, although broader implementation of this technology remains limited. The development and refinement of best practice algorithms can help standardize management and improve decision-making. Furthermore, incorporating validated outcome measures, recorded and followed over time, will improve both patient care and research efforts moving forward.

title: The PRISMA Statement for Reporting Systematic Reviews and Meta-Analyses of Studies That Evaluate Health Care Interventions: Explanation and Elaboration
abstract: In order to encourage dissemination of the PRISMA explanatory paper, this article is freely accessible on the Annals of Internal Medicine, PLoS Medicine, and BMJ Web sites. The authors jointly hold the copyright of this article. For details on further use, see the PRISMA Web site . Systematic reviews and meta-analyses are essential tools for summarizing evidence accurately and reliably. They help clinicians keep up-to-date; provide evidence for policy makers to judge risks, benefits, and harms of health care behaviors and interventions; gather together and summarize related research for patients and their carers; provide a starting point for clinical practice guideline developers; provide summaries of previous research for funders wishing to support new research (1); and help editors judge the merits of publishing reports of new studies (2). Recent data suggest that at least 2,500 new systematic reviews reported in English are indexed in MEDLINE annually (3). Unfortunately, there is considerable evidence that key information is often poorly reported in systematic reviews, thus diminishing their potential usefulness (36). As is true for all research, systematic reviews should be reported fully and transparently to allow readers to assess the strengths and weaknesses of the investigation (7). That rationale led to the development of the QUOROM (QUality Of Reporting Of Meta-analysis) Statement; those detailed reporting recommendations were published in 1999 (8). In this paper we describe the updating of that guidance. Our aim is to ensure clear presentation of what was planned, done, and found in a systematic review. Terminology used to describe systematic reviews and meta-analyses has evolved over time and varies across different groups of researchers and authors (see Box 1). In this document we adopt the definitions used by the Cochrane Collaboration (9). A systematic review attempts to collate all empirical evidence that fits pre-specified eligibility criteria to answer a specific research question. It uses explicit, systematic methods that are selected to minimize bias, thus providing reliable findings from which conclusions can be drawn and decisions made. Meta-analysis is the use of statistical methods to summarize and combine the results of independent studies. Many systematic reviews contain meta-analyses, but not all. Box 1. Terminology. The QUOROM Statement and Its Evolution Into PRISMA The QUOROM Statement, developed in 1996 and published in 1999 (8), was conceived as a reporting guidance for authors reporting a meta-analysis of randomized trials. Since then, much has happened. First, knowledge about the conduct and reporting of systematic reviews has expanded considerably. For example, The Cochrane Library's Methodology Register (which includes reports of studies relevant to the methods for systematic reviews) now contains more than 11,000 entries (March 2009). Second, there have been many conceptual advances, such as outcome-level assessments of the risk of bias (10, 11), that apply to systematic reviews. Third, authors have increasingly used systematic reviews to summarize evidence other than that provided by randomized trials. However, despite advances, the quality of the conduct and reporting of systematic reviews remains well short of ideal (36). All of these issues prompted the need for an update and expansion of the QUOROM Statement. Of note, recognizing that the updated statement now addresses the above conceptual and methodological issues and may also have broader applicability than the original QUOROM Statement, we changed the name of the reporting guidance to PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses). Development of PRISMA The PRISMA Statement was developed by a group of 29 review authors, methodologists, clinicians, medical editors, and consumers (12). They attended a three-day meeting in 2005 and participated in extensive post-meeting electronic correspondence. A consensus process that was informed by evidence, whenever possible, was used to develop a 27-item checklist (Table 1; see also Table S1, for a downloadable template checklist for researchers to re-use) and a four-phase flow diagram . Items deemed essential for transparent reporting of a systematic review were included in the checklist. The flow diagram originally proposed by QUOROM was also modified to show numbers of identified records, excluded articles, and included studies. After 11 revisions the group approved the checklist, flow diagram, and this explanatory paper. Table S1. Template checklist Figure S1. Template document Table 1. Checklist of Items to Include When Reporting a Systematic Review (With or Without Meta-Analysis) Figure 1. Flow of information through the different phases of a systematic review. The PRISMA Statement itself provides further details regarding its background and development (12). This accompanying Explanation and Elaboration document explains the meaning and rationale for each checklist item. A few PRISMA Group participants volunteered to help draft specific items for this document, and four of these (DGA, AL, DM, and JT) met on several occasions to further refine the document, which was circulated and ultimately approved by the larger PRISMA Group. Scope of PRISMA PRISMA focuses on ways in which authors can ensure the transparent and complete reporting of systematic reviews and meta-analyses. It does not address directly or in a detailed manner the conduct of systematic reviews, for which other guides are available (1316). We developed the PRISMA Statement and this explanatory document to help authors report a wide array of systematic reviews to assess the benefits and harms of a health care intervention. We consider most of the checklist items relevant when reporting systematic reviews of non-randomized studies assessing the benefits and harms of interventions. However, we recognize that authors who address questions relating to etiology, diagnosis, or prognosis, for example, and who review epidemiological or diagnostic accuracy studies may need to modify or incorporate additional items for their systematic reviews.  To maximize the benefit of this document, we encourage people to read it in conjunction with the PRISMA Statement (11). We present each checklist item and follow it with a published exemplar of good reporting for that item. (We edited some examples by removing citations or Web addresses, or by spelling out abbreviations.) We then explain the pertinent issue, the rationale for including the item, and relevant evidence from the literature, whenever possible. No systematic search was carried out to identify exemplars and evidence. We also include seven Boxes that provide a more comprehensive explanation of certain thematic aspects of the methodology and conduct of systematic reviews. Although we focus on a minimal list of items to consider when reporting a systematic review, we indicate places where additional information is desirable to improve transparency of the review process. We present the items numerically from 1 to 27; however, authors need not address items in this particular order in their reports. Rather, what is important is that the information for each item is given somewhere within the report. The PRISMA Checklist Title and Abstract  Title Identify the report as a systematic review, meta-analysis, or both. Examples Recurrence rates of video-assisted thoracoscopic versus open surgery in the prevention of recurrent pneumothoraces: a systematic review of randomised and non-randomised trials (20). Mortality in randomized trials of antioxidant supplements for primary and secondary prevention: systematic review and meta-analysis (21). Explanation Authors should identify their report as a systematic review or meta-analysis. Terms such as review or overview do not describe for readers whether the review was systematic or whether a meta-analysis was performed. A recent survey found that 50% of 300 authors did not mention the terms systematic review or meta-analysis in the title or abstract of their systematic review (3). Although sensitive search strategies have been developed to identify systematic reviews (22), inclusion of the terms systematic review or meta-analysis in the title may improve indexing and identification. We advise authors to use informative titles that make key information easily accessible to readers. Ideally, a title reflecting the PICOS approach (participants, interventions, comparators, outcomes, and study design) (see Item 11 and Box 2) may help readers as it provides key information about the scope of the review. Specifying the design(s) of the studies included, as shown in the examples, may also help some readers and those searching databases. Box 2. Helping To Develop the Research Question(s): The PICOS Approach. Some journals recommend indicative titles that indicate the topic matter of the review, while others require declarative titles that give the review's main conclusion. Busy practitioners may prefer to see the conclusion of the review in the title, but declarative titles can oversimplify or exaggerate findings. Thus, many journals and methodologists prefer indicative titles as used in the examples above.  Provide a structured summary including, as applicable: background; objectives; data sources; study eligibility criteria, participants, and interventions; study appraisal and synthesis methods; results; limitations; conclusions and implications of key findings; funding for the systematic review; and systematic review registration number.  The role and dose of oral vitamin D supplementation in nonvert

title: Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies.
abstract: Criteria 1 Was the research question or objective in this paper clearly stated? 2 Was the study population clearly specified and defined? 3 Was the participation rate of eligible persons at least 50%? 4 Were all the subjects selected or recruited from the same or similar populations (including the same time period)? Were inclusion and exclusion criteria for being in the study prespecified and applied uniformly to all participants? 5 Was a sample size justification, power description, or variance and effect estimates provided? 6 For the analyses in this paper, were the exposure(s) of interest measured prior to the outcome(s) being measured? 7 Was the timeframe sufficient so that one could reasonably expect to see an association between exposure and outcome if it existed? 8 For exposures that can vary in amount or level, did the study examine different levels of the exposure as related to the outcome (e.g., categories of exposure, or exposure measured as continuous variable)? 9 Were the exposure measures (independent variables) clearly defined, valid, reliable, and implemented consistently across all study participants? 10 Was the exposure(s) assessed more than once over time? 11 Were the outcome measures (dependent variables) clearly defined, valid, reliable, and implemented consistently across all study participants? 12 Were the outcome assessors blinded to the exposure status of participants? 13 Was loss to follow-up after baseline 20% or less? 14 Were key potential confounding variables measured and adjusted statistically for their impact on the relationship between exposure(s) and outcome(s)? Quality rating Rater #1 TN Rater #2 BST

title: Swallowing function after laryngeal cleft repair: More than just fixing the cleft
abstract: To evaluate and describe the swallowing function in children after laryngeal cleft repair.

title: Postoperative Observation of Children after Endoscopic Type 1 Posterior Laryngeal Cleft Repair
abstract: To report the perioperative management and surgical outcomes in a large series of pediatric patients with endoscopically repaired type 1 posterior laryngeal cleft (PLC).   Urban, tertiary care, free-standing pediatric hospital.  Patients who underwent endoscopic carbon dioxide laser–assisted repair of type 1 posterior laryngeal clefts between January 2006 and December 2012. Medical records were reviewed.  Fifty-four patients (34 male) underwent repair of type 1 PLC. Median age was 25.5 months (range, 2-120 months). Indications for repair included aspiration (n = 39; 72%), chronic bronchitis (n = 13; 24%), and stridor with feeds (n = 2; 4%). No children remained intubated postoperatively. Thirty-three patients (61%) stayed in overnight observation (“Obs PLC”) and 21 patients (39%) stayed in the pediatric intensive care unit (“PICU PLC”) postoperatively. Between Obs PLC and PICU PLC groups, there was no significant difference in age (mean 22 vs 30 months, respectively; P = .28). Comorbidities were similar between the groups. Symptoms improved in 41 of the 54 patients (76%). No postoperative complications were noted. Two patients required revision PLC repair. The cost of admitting a patient to a lower acuity location was estimated to be 60% less per day than cost of a PICU admission.  The endoscopic surgical repair of a type 1 PLC is successful and has a low morbidity and complication rate. Patients may be safely managed in an observation unit and without postoperative intubation. This approach achieved a marked cost reduction in postoperative care.

title: Indications for Surgical Repair of Type 1 Laryngeal Cleft
abstract: Type 1 laryngeal cleft (T1LC) is a congenital deficiency in the posterior glottis, resulting in a communication between the hypopharynx and glottis. No consensus treatment paradigm exists for timing and criteria for patient selection for surgical repair. Our goal is to assess whether patient characteristics can help predict improvement after surgery.  After Institutional Review Board exemption, a retrospective chart review was performed for patients undergoing surgery to diagnose a T1LC. Charts were examined for age, presenting symptoms, comorbidities, pre/postoperative videoflouroscopic swallow study reports, and outcomes.  Ninety-seven patients with clinical suspicion for T1LC underwent direct laryngoscopy and bronchoscopy, and 63 (64%) were diagnosed with a T1LC. Twenty-two patients (63%) undergoing surgery achieved clinical or radiographic improvement. There was no difference in average age, aspiration, or penetration between clinical improvement and no improvement groups. Of 13 patients with comorbidities that increase their risk of aspiration, 12 were significantly improved. There were 5 complications, which were managed conservatively.  Our experience supports the repair of T1LC repair at time of diagnostic laryngoscopy if satisfactory improvement is not noted with conservative treatment. This should be performed without segregation for age, comorbidities, or degree of dysphagia. Our technique is performed with minimal complications and achieves satisfactory results.

title: Swallowing dysfunction among patients with laryngeal cleft: More than just aspiration?
abstract: The Modified Barium Swallow (MBS) is the most widely utilized instrumental assessment of swallowing disorders in children; however, the exact role in the evaluation of laryngeal clefts remains controversial.



This study was an IRB-approved retrospective review on patients diagnosed with laryngeal cleft from 2002 to 2014. The objective was to describe the range of swallowing dysfunction that may be present in patients with laryngeal clefts both pre- and post-intervention (conservative management versus surgery). A speech-language pathologist reviewed MBS studies and medical records to determine Penetration-Aspiration Scale (PAS) and Functional Oral Intake Scale (FOIS) scores.



One hundred seventy-five patients who underwent laryngeal cleft repair during the study period (type 1, n=111; type 2, n=54; type 3, n=9; type 4, n=1) were included. Fifty patients who were managed conservatively (type 1) were also included. Swallowing impairment was demonstrated in all phases of swallowing for all cleft types. Oral phase impairment ranged from 27-67% pre-intervention to 19-75% post-intervention, triggering impairment from 24-42% pre-intervention to 24-75% post-intervention, and pharyngeal phase impairment (laryngeal penetration and aspiration) from 57-100% pre-intervention to 40-100% post-intervention. Laryngeal penetration and aspiration on thin and thick liquids, silent aspiration, PAS, and FOIS scores are reported. Significant improvements in swallowing function (p<0.05) were documented in all of the conservatively and surgically managed sub-groups.



The MBS study is a useful tool for evaluating swallowing function in patients with laryngeal cleft and provides information beyond the lack or presence of aspiration. Understanding impairments in all phases of swallowing may be beneficial for perioperative management.

title: Modified Best-Practice Algorithm to Reduce the Number of Postoperative Videofluoroscopic Swallow Studies in Patients With Type 1 Laryngeal Cleft Repair.
abstract: There is no consensus as to the timing of videofluoroscopic swallow studies (VFSSs) in determining resolving aspiration after laryngeal cleft repair. There is a growing literature on the effect of radiation exposure in children.



To modify a previously published best-practice algorithm based on a literature review and our clinical experience to maintain the quality of care provided after successful type 1 laryngeal cleft repair, while reducing the total number of postoperative VFSSs by 10% or greater.



The previously published algorithm was modified by a multidisciplinary group at a tertiary care academic medical center (Massachusetts Eye and Ear) and was prospectively applied to 31 children who underwent type 1 laryngeal cleft repair from January 1, 2013, to February 28, 2015.



The number of VFSSs obtained in the first 7 months after surgery was compared with the peer-reviewed literature and with a retrospective cohort of 27 patients who underwent type 1 laryngeal cleft repair from January 1, 2008, to December 31, 2012.



The study cohort comprised 31 patients. Their ages ranged from 10 to 48 months, with a mean (SD) age of 23.94 (9.93) months, and 19% (6 of 31) were female. The mean (SD) number of postoperative VFSSs per patient before and after implementation of the algorithm was 1.22 (0.42) and 1.03 (0.55), respectively. The use of the algorithm reduced the number of VFSSs by 0.19 (95% CI, -0.07 to 0.45). This reduction in radiation exposure is equivalent to 1.47 chest radiographs per child per course of care. Surgical success was 87% (27 of 31) compared with our group's previously published success rate of 78% (21 of 27) (absolute difference, 0.09; 95% CI, -0.17 to 0.34).



This modified algorithm to help guide decisions on when and how often to obtain VFSSs after type 1 laryngeal cleft repair can limit patients' radiation exposure, while maintaining high surgical success rates.

title: Diagnosis and Management of Type I Posterior Laryngeal Clefts
abstract: 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.

title: Outcomes and predictors of surgical management in type 1 laryngeal cleft swallowing dysfunction
abstract: To examine the effect of and predict the success of type 1 laryngeal cleft (LC‐1) augmentation through swallowing evaluations.

title: Laser Assisted Double-Layer Endoscopic Repair of Laryngeal Clefts: Our Experience in 11 Cases
abstract: Laryngeal cleft is a rare congenital malformation of the respiratory tract leading to a high level of morbidity and mortality, recently being diagnosed with increased frequency. Management throughout the years included medical and surgical. The open surgical technique is more commonly used although it has higher risk and need longer post-operative care. Recently surgical endoscopic repair was introduced using different techniques. To evaluate the clinical features of infants and children presenting with laryngeal clefts, and review endoscopic management modality especially the technique and results of repair using double-layer (2 layers) technique. Outlining our experience with the surgical technique, complications arising from surgery as well as surgical outcome by evaluating the decannulation rate and cessation of the assisted feeding by the end of treatment. Retrospective case series study. Review of infant and children seen in our clinic with the diagnosis of laryngeal cleft from January 2012 till June 2014., and treated by CO2 laser assisted double-layer endoscopic closure. The presenting symptoms, patient demographics, diagnostic procedure, cleft type, surgical outcome and complications all were evaluated. We revised the case notes of 34 patients with a workable diagnosis of Laryngeal cleft. A total of 11 patients met our criteria and were included in the study. Clefts typing was according to modified Benjamin–Ingles classification, type 1 (n = 9) and type 2 (n = 2). All clefts were closed endoscopically by CO2 laser incision of the mucosa and two-layer endoscopic closure of the cleft without postoperative intubation or tracheotomy. They accepted oral feeding within 5 postoperative days (range 3–11 days). 5 out of 6 patients (83.3%) successfully stopped nasogastric feeding and 1 out of 2 patients successfully weaned from nasal cannula. One patient deceased few months after surgery due to other medical problems. Two patients needed redo after burst of the upper cleft stitches immediately after repair by iatrogenic cause during suctioning. The repair was stable in all patients during the second look 6 weeks after surgery with no recurrence or fistula formed. There were no clinically significant observed complications with this technique. After a mean follow up of 24 months, all children have a good voice, have no sign of residual aspiration, and less hospital admissions. Laryngeal cleft should be suspected in children presenting with recurrent pneumonia and difficulties during feeding. Endoscopic repair is a successful and safe technique for treating laryngeal clefts, and has short post-operative recovery without postoperative intubation or tracheotomy. Using the double-layer technique appeared to be promising but needs more comparative studies in the future.

title: Type 1 laryngeal cleft: establishing a functional diagnostic and management algorithm.
abstract: To report our experience with all patients diagnosed with type 1 laryngeal cleft over a period of 3 years in our referral practice and to describe a functional diagnostic and management algorithm for children with this disorder.



A prospective longitudinal study in a tertiary care referral center. Twenty pediatric patients diagnosed with type 1 laryngeal cleft in a 3-year period (5/1/2002-5/1/2005) were included in this study. The incidence, presenting symptoms, diagnostic procedures, medical and surgical interventions performed, and clinical outcomes were evaluated.



The incidence of type 1 laryngeal cleft was 7.6%. Among the 20 patients in this study, aspiration with thin liquids was the most common presenting symptom (18 patients, 90%). Three patients underwent modified barium swallow (MBS) alone, 3 patients underwent functional endoscopic evaluation of swallow (FEES) alone, and 11 patients underwent both MBS and FEES prior to intraoperative endoscopic evaluation. Four patients (20%) were successfully treated with conservative therapy. Sixteen patients (80%) required endoscopic surgical repair after failing a course of conservative measures. The success rate of surgical repair was 94% (15 out of 16 patients).



Type 1 laryngeal cleft can be challenging diagnostically. We propose a functional diagnostic and management algorithm that includes MBS, FEES, suspension laryngoscopy with bimanual interarytenoid palpation, and a trial of conservative therapy, as a way to diagnose and manage type 1 laryngeal cleft prior to consideration of surgical repair. If conservative therapy fails, then surgical intervention is indicated.

title: Outcomes and Resource Utilization of Endoscopic Mass-Closure Technique for Laryngeal Clefts
abstract: To compare resource utilization and clinical outcomes between endoscopic mass-closure and open techniques for laryngeal cleft repair.   Tertiary academic children’s hospital. Subjects and Methods Pediatric patients undergoing repair for Benjamin-Inglis type 1-3 laryngeal clefts over a 15-year period. All 20 patients undergoing endoscopic repair were included. Eight control patients undergoing open repair were selected using matching by age and cleft type. Demographic, clinical, and resource utilization data were collected.  Twenty-eight patients were included (20 endoscopic, 8 open). Mean age, rates of tracheostomy and vocal fold immobility, and distribution of cleft types were not different between the 2 groups (all P > .2). Mean operative time (P = .004) and duration of hospital stay (P < .001) were significantly shorter in the endoscopic group. All repairs were intact in both groups at final postoperative endoscopy. Rates of persistent laryngeal penetration or aspiration on swallow study were not different between groups (P = 1.000), although results were available for only 11 patients.  Endoscopic laryngeal cleft repair using a mass-closure technique provides a durable result while requiring significantly shorter operative times and hospital stays than open repair and avoiding the potential morbidity of laryngofissure. However, open repair may allow the simultaneous performance of other airway reconstructive procedures and may be a useful salvage technique when endoscopic repair fails. Postoperative swallowing results require further study.

title: The lipid laden macrophage index as a marker of aspiration in patients with type I and II laryngeal clefts.
abstract: Laryngeal clefts are uncommon congenital anomalies that may cause pulmonary aspiration, leading to considerable morbidity including recurrent pneumonias. The lipid laden macrophage index (LLMI) is a potential marker of pulmonary aspiration. The objective of this study was to assess the utility of the lipid laden macrophage index as a marker of severity of pulmonary aspiration in children with laryngeal clefts and its role in the management of these patients.



An institutional review board approved retrospective review of all patients with laryngeal cleft who had also underwent direct laryngoscopy with rigid bronchoscopy and flexible bronchoscopy with bronchoalveolar lavage. The LLMI was measured from the lavage and compared to clinical and radiological data.



Forty-four patients with laryngeal clefts (31 type I clefts and 13 type II clefts) underwent assessment with flexible bronchoscopy and bronchoalveolar lavage. The median age at first outpatient visit in our Center for Aero-digestive Disorder was 0.92 years in patients with type I clefts and 1.66 years in patients with type 2 clefts. All patients in this study had at least one modified barium swallow (MBS) performed to assess for aspiration. The mean LLMI was significantly higher in patients with type II (mean+/-SEM) 81.8+/-11.9 clefts compared to type I clefts 44.9+/-5.6.



We recommend obtaining LLMI in patients with laryngeal cleft. The lipid laden macrophage index is increased in patients with more severe laryngeal clefts, thus potentially predicting those patients whom would most benefit from early surgical intervention.

title: The presentation and management of laryngeal cleft: a 10-year experience.
abstract: To review the presentation and associated congenital abnormalities of laryngeal cleft and present guidelines for its evaluation and management.



A 10-year retrospective study (1994-2004) with institutional review board approval.



Two pediatric tertiary care medical centers.



Twenty-two pediatric patients (mean age, 21 months) with laryngeal cleft.



Surgical repair of laryngeal cleft.



Sex, age, symptoms, other associated abnormalities, method of evaluation, type of laryngeal cleft, method of surgical repair, treatment outcome, complications, and long-term follow-up.



All 22 patients underwent surgical repair for laryngeal cleft. Airway endoscopy confirmed the following types of laryngeal clefts: type 1 (n = 3), type 2 (n = 10), and type 3 (n = 9). Surgical repair techniques included an open approach with or without interposition graft (n = 16) and an endoscopic approach (n = 6).



Early diagnosis and proper repair of laryngeal cleft are essential to prevent pulmonary damage and associated morbidity. Each patient should be assessed properly, and the surgical approach should be individualized based on the symptoms, other associated findings on airway endoscopy, and type of cleft.

title: Techniques and Outcomes of Laryngeal Cleft Repair: An Update to the Great Ormond Street Hospital Series
abstract: We present an update to the Great Ormond Street Hospital series of laryngeal clefts, describing a further 35 clefts of Benjamin-Inglis types 1 through 3 treated between 1992 and 2003. Associated congenital anomalies were common. Most type 1 and smaller type 2 clefts were repaired endoscopically, whereas larger clefts were repaired through an anterior approach. Increasing use was made recently of a 3-layer repair with an interposition graft of temporalis fascia. The rates of complication, revision surgery, and death were 54%, 26%, and 6%, respectively. Most of the children are now orally fed, and 9 still have a tracheotomy.

title: Familial occurrence of congenital laryngeal clefts
abstract: 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.

title: Type I Posterior Laryngeal Clefts
abstract: 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.

title: Low inter-arytenoid height: a subclassification of type 1 laryngeal cleft diagnosis and management.
abstract: To report our experience of patients with type 1 laryngeal cleft, (including low inter-arytenoid height) who failed conservative management over a five year period. We describe the diagnostic elements of the history, examination at laryngobronchoscopy and provide a management algorithm including the use of inter-arytenoid submucosal injection of gelfoam as a temporary therapeutic as well as diagnostic tool.



A retrospective case note review over a five year period was undertaken to review all cases of type 1 laryngeal cleft who failed conservative management. Presenting symptoms, diagnostic procedures, surgical interventions and clinical outcomes were reviewed.



Seventeen patients were identified. Chronic cough was the most consistent feature in the history (100%). All patients underwent a microlaryngoscopy with binocular microlaryngeal assessment. Six patients (35%) underwent gelfoam injection; four of these went on to a formal repair. The remaining 11 all had a repair performed without injection. The success of surgical repair was 80% (12/15) however in the other three, all had improvement in symptoms.



Type 1 laryngeal cleft anomalies may extend beyond that described by Benjamin and Inglis. An appropriate history as well as binocular inspection at the time of laryngoscopy is essential. Injection augmentation offers a safe tool in the assessment and management, and endoscopic surgical repair remains the standard for definitive therapy in those that fail conservative management.

title: Neurologic Evaluation in Children With Laryngeal Cleft
abstract: 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.

title: Laryngotracheal Cleft Repair in Children With Complex Airway Anomalies.
abstract: This study provides clinicians with relevant information regarding the surgical outcomes of patients with laryngotracheal cleft in the context of additional airway anomalies.



To determine the rates of surgical success in patients who underwent laryngotracheal cleft repair in the context of additional airway anomalies, determine the revision rate for cleft repair, characterize the time to revision among patients who underwent cleft repair, and assess the functional swallowing outcomes after cleft repair.



A retrospective study was conducted at a quaternary pediatric center of 81 children diagnosed as having laryngotracheal cleft with or without concomitant airway anomalies who underwent laryngotracheal cleft repair between February 1, 2000, and February 28, 2013. Analysis was conducted from October 1, 2012, through March 30, 2013.



Surgical repair of laryngotracheal cleft.



Surgery-specific success, overall surgical success, and revision rate. We defined surgery-specific success as a repair not requiring revision and overall surgical success as absence of a cleft or TEF at the last operative examination, regardless of the number of revisions required.



Of 81 patients with laryngotracheal cleft who underwent surgical repair, 48 (59%) had at least 1 additional airway finding; 24 (30%) had tracheomalacia and 21 (26%) had subglottic stenosis. Seventeen patients required a revision of laryngotracheal cleft repair, with a median time to revision of 8.3 months (interquartile range, 4.3-25.1 months). Six patients required a second revision, with a median interval between revisions of 44.2 months (interquartile range, 28.6-53.6 months). The surgery-specific success rate was 77% (37 of 48) in patients with additional airway anomalies and 82% (27 of 33) in those with laryngotracheal clefts alone. The overall surgical success rate was 92% (44 of 48) in patients with additional airway anomalies and 97% (32 of 33) in those with clefts alone. Fourteen (17%) patients demonstrated swallowing dysfunction postoperatively despite closure of the cleft.



Although additional airway findings were common in our cohort of patients with laryngotracheal clefts, these anomalies did not affect surgery-specific or overall surgical success. The overall surgical success rate for those with and without additional airway anomalies was 92% and 97%, respectively. In view of the fact that cleft breakdown after surgical repair is not uncommon and may occur years after the initial repair, we strongly advocate long-term follow-up. Despite successful closure, a significant percentage of children with laryngotracheal cleft will have persistent swallowing dysfunction.

title: The association between laryngeal cleft and tracheoesophageal fistula: Myth or reality?
abstract: 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.

title: Laryngeal cleft: evaluation and management.
abstract: Review the latest diagnostic and treatment modalities for laryngeal and laryngotracheoesophageal clefts as they can be a major cause of respiratory and feeding morbidity in the infant and pediatric population.



Literature review of published reports.



The presentation of laryngeal cleft usually involves respiratory symptoms, such as stridor, chronic cough, aspiration, and recurrent respiratory infections. Clefts of the larynx and trachea/esophagus can occur in isolation, as part of a syndrome (Opitz-Frias, VATER/VACTERL, Pallister Hall, CHARGE), or with other associated malformations (gastrointestinal, genitourinary, cardiac, craniofacial). This publication reviews the presenting signs/symptoms, diagnostic options, prognosis, and treatment considerations based on over a decade of experience of the senior author with laryngeal clefts.



Type I laryngeal clefts can be managed medically or surgically depending on the degree of morbidity. Types II, III, and IV require endoscopic or open surgery to avoid chronic respiratory and feeding complications.

title: Type I-II laryngeal cleft: clinical course and outcome.
abstract: Laryngeal cleft (LC) is a rare congenital anomaly manifesting in a variety of symptoms, including swallowing disorders and aspirations, dyspnea, stridor and hoarseness. The mild forms (types I-II) may be underdiagnosed, leading to protracted symptomatology and morbidity.



To evaluate the diagnostic process, clinical course, management and outcome in children with type I-II laryngeal clefts.



We conducted a retrospective case analysis for the years 2005-2012 in a tertiary referral center.



Seven children were reviewed: five boys and two girls ranging in age from birth to 5 years. The most common presenting symptoms were cough, aspirations and pneumonia. Evaluation procedures included fiber-optic laryngoscopy (FOL), direct laryngoscopy (DL) and videofluoroscopy. Other pathologies were seen in three children. Six children underwent successful endoscopic surgery and one child was treated conservatively. The postoperative clinical course was uneventful in most of the cases.



Types I-II LC should be considered in the differential diagnosis of children presenting with protracted cough and aspirations. DL is crucial for establishing the diagnosis. Endoscopic surgery is safe and should be applied promptly when conservative measures fail.

title: The utility of the fiberoptic endoscopic evaluation of swallowing (FEES) in diagnosing and treating children with Type I laryngeal clefts.
abstract: This case series of three young children with type I laryngeal clefts is presented to demonstrate the utility of fiberoptic endoscopic evaluation of swallowing (FEES) in managing these patients. FEES revealed laryngeal penetration in a posterior to anterior direction in two patients and penetration from lateral to medial in the third patient. The type of laryngeal penetration helped in making the diagnosis of a type I cleft in two children and helped establish a safe feeding regiment in the third child. Patients with type I laryngeal clefts are often misdiagnosed, most likely resulting from the complex presentation of signs/symptoms and the difficulty of detecting small clefts with currently available tests. The pattern of laryngeal aspiration seen with FEES can help in diagnosis and management in this patient population.

title: The management of laryngeal clefts.
abstract: To review the clinical presentation and management of all infants and children presenting with laryngeal clefts to a tertiary pediatric ENT centre and to identify changes in practice over time.



A retrospective case note review of the management of all infants and children with a diagnosis of a laryngeal cleft identified in our Department between 01/11/2003 and 31/12/2010.



Twelve children with laryngeal clefts were identified. Six clefts were grade 1, five grade 2 and one grade 3b. All grade 1 clefts were managed conservatively. Of the grade 2 clefts, four required surgery with one being managed conservatively. Two were repaired using an open technique and two using an endoscopic technique. The grade 3b cleft was repaired endoscopically. Two cleft repairs broke down post-operatively requiring further surgery.



Conservative management remains the management of choice for lower grade clefts. Where a laryngeal cleft requires repair there has been a trend towards the endoscopic over open technique, even of more extensive clefts.

title: Chest radiographic and CT evaluation of lung abnormalities in pediatric patients with laryngeal cleft
abstract: The purpose of this study was to (1) evaluate chest radiography (CR) and computed tomography (CT) findings in pediatric patients with laryngoscopically confirmed laryngeal cleft and (2) determine whether CT provided additional information over CR in evaluating lung abnormalities in pediatric patients with laryngeal cleft.

title: Trans‐nasal flexible bronchoscopy in wheezing children: Diagnostic yield, impact on therapy, and prevalence of laryngeal cleft
abstract: Persistent or recurrent wheezing is a common indication for flexible bronchoscopy, as anatomic and infectious or inflammatory changes are highly prevalent. We sought to evaluate the prevalence of anatomic, infectious, and inflammatory disease in a cohort of children undergoing flexible bronchoscopy for wheezing or poorly controlled asthma.

title: Advances in the diagnosis, management, and treatment of neonates with laryngeal disorders.
abstract: Neonatal airway obstruction due to laryngeal pathology may cause significant morbidity and even mortality. The most frequently occurring etiologies anatomically from superiorly to inferiorly include: laryngomalacia, saccular cyst, vocal fold paralysis, anterior glottic web, laryngeal atresia, laryngeal cleft, subglottic stenosis, and subglottic hemangioma. The pathophysiology, presentation, and treatment options for each of these entities are discussed with a focus on a multidisciplinary, evidence-based approach.

title: Early surgical intervention in type I laryngeal cleft.
abstract: Diagnosis and treatment of type 1 laryngeal clefts remains a challenge. The purpose of this study is to determine if early surgical intervention in type I laryngeal clefts improves outcomes.



A retrospective case series was conducted at an academic tertiary care children's hospital. 18 children undergoing early (≤3 months from diagnosis) surgical intervention for type I laryngeal cleft repair between August of 2012 and December 2014. Data was compiled through a manual chart review.



18 children who underwent early surgical intervention for type I laryngeal cleft repair were identified for review. 14 (78%) were male and 4 (22%) were female and the average age at time of repair was 1.6 years. Most frequent presenting symptoms included dysphagia (61%) and recurrent respiratory issues (22%). Successful swallowing outcomes, defined as subjective improvement (i.e. absence of previous symptoms) per parental report in follow-up visits, +/- normal post-operative MBS (modified barium swallow) findings, was seen in 11 patients (61%). 9 patients required hospitalization for respiratory issues prior to surgical repair. Post-operatively, 4 patients still incurred an admission for respiratory reasons.



Our series shows a success rate of 61% with early surgical intervention (≤3 months from diagnosis). A decrease in post-operative hospitalizations is appreciated.

title: Injection Laryngoplasty for Type 1 Laryngeal Cleft in Children
abstract: To review the 2-year, single-institution experience with injection laryngoplasty for diagnosis and treatment of type 1 laryngeal clefts (LC-1).  Case series with chart review.  Tertiary care academic children’s hospital.  Patients at our institution who underwent injection laryngoplasty for LC-1 from January 2008 to December 2009. Outcome measures included patient demographics, surgical and anesthetic technique, effect on swallowing, and complications. Preoperative and postoperative swallowing evaluations were compared, and quality and duration of effects were calculated.  Sixteen children, 9 male and 7 female, underwent injection laryngoplasty for LC-1. Mean gestational age was 36.4 weeks (SD, 4.0 weeks; range, 27-41 weeks). Six patients had a major congenital anomaly (37.5%). Mean age at injection was 11.8 months (SD, 8.9 months; range, 2.9-33.5 months). Nine patients (56%) demonstrated complete resolution of penetration and aspiration on postoperative modified barium swallow (MBS), 4 patients (25%) had some improvement, and 3 patients (19%) showed no change. There were no complications. Mean duration of symptom improvement was 3.3 months (SD, 3.0 months; range, 0-11 months). Five patients went on to have definitive surgical LC-1 repair.  Injection laryngoplasty was found to have favorable results in this group of patients with LC-1. Most patients experienced complete resolution of aspiration on MBS, and no patients experienced complications. These findings support the further study of injection laryngoplasty for LC-1 as both a diagnostic and therapeutic technique. Longer follow-up intervals are necessary to elucidate the predictive value with regard to success of formal cleft repair.

title: Laryngeal and Laryngotracheoesophageal Clefts: Role of Early Surgical Repair
abstract: 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.

title: Endoscopic Repair of Type IA Laryngeal Clefts
abstract: Laryngotracheoesophageal clefts (LTECs) are rare anomalies of the posterior larynx and membranous trachea that may be relatively minor or may extend into the main bronchi. Minor clefts are characterized by deficiency of interarytenoid tissue as the sole anomaly, and as a consequence, the interarytenoid notch lacks height, parallels of the level of the vocal folds in the axial plane, and places patients at risk for aspiration or inspiratory stridor. When limited to the posterior supraglottis, the cleft is known as an Evans I, Benjamin I, or Armitage IA (Table I) (1-5). Henceforth, this cleft will be referred to as type IA laryngeal cleft (IA LC). All forms of LCs are uncommon, present in 11/427 (2.6%) of stridorous patients presenting to a pediatric otolaryngologist (6). In contrast to larger LTECs that typically present in dramatic fashion, the defect of IA LCs easily may be overlooked by the endoscopist. If an IA LC is not considered, symptoms such as chronic cough, recurrent pneumonia, or aspiration may be attributed incorrectly to other causes.

title: Endoscopic repair of laryngotracheoesophageal clefts.
abstract: In Japan, surgical repair of a laryngotracheoesophageal cleft (LTEC) typically consists of the anterior approach, with the lateral approach as an alternative. Endoscopic surgery to repair the tracheoesophageal septum has been reported, and this study reviewed our experience treating several cases of LTEC endoscopically.



Endoscopic repair of LTEC was performed in 7 patients (3 boys, 4 girls; age range 4 months to 2 years 10 months; mean age 11 months; mean weight at surgery 7.23 kg; weight range 3.85-12.24 kg) between 2009 and 2014. LTEC was type I in 5 patients and types II and IV in 1 patient each. The patient with type IV was first operated on by the lateral approach, and the remaining cleft, which level was type III, was repaired endoscopically. Postoperative outcomes were retrospectively studied.



Endoscopic surgery was successful in all patients. All 6 patients with types I and II LTEC were extubated easily, while in the patient with type IV LTEC, it was difficult to remove the tracheostomy cannula because of tracheomalacia. Postoperatively, tracheostomy cannulation became more stable, and the patient is gradually being weaned off the ventilator. All patients could be fed orally without difficulty postoperatively.



Endoscopic surgery provides a view from the cephalic aspect permitting the surgeon to form a normal larynx with only minimal risk of complications.

title: Endoscopic repair of laryngeal cleft type I and type II: When and why?
abstract: To evaluate the clinical features of children with type I and type II laryngeal cleft and the role of conservative monitoring versus endoscopic repair in their management.

title: An Aerodigestive Approach to Laryngeal Clefts and Dysphagia Using Injection Laryngoplasty in Young Children
abstract: Purpose of ReviewOur objective is to summarize the presentation, diagnosis, and management of mild laryngeal clefts in children. We focus on deep interarytenoid notches (DIN), a subclassification of type 1 laryngeal clefts (LC1), and review the literature and our multidisciplinary experience utilizing injection laryngoplasty (IL).Recent FindingsDIN is a mild form of LC1 and is considered a normal anatomical variant. Recent cohort studies demonstrate IL to be a safe, low-risk, and efficacious treatment of LC1, but few studies focus on DIN. We present results from two aerodigestive clinic (ADC) pilot studies at our institution, in patients 1–3 years old, with DIN-related dysphagia and aspiration (DA). Feeding, respiratory-related symptom scores, and aspiration/penetration assessed on modified barium swallow (MBS) significantly improved following combined IL and feeding therapy using a thickener weaning protocol (TWP). Subgroup analysis reveals combined IL and TWP to be particularly effective in patients with severe baseline DA.SummaryMultidisciplinary aerodigestive evaluation and management with IL and feeding therapy focused on weaning levels of thickener is emerging as a novel and effective approach for treatment of DIN-related DA in young children. Further comparative, prospective trials investigating effects of IL and feeding therapy are required to validate results.

title: Diagnosis and Determination of the Clinical Significance of Type 1A Laryngeal Clefts by Gelfoam Injection
abstract: Laryngeal clefts are rare congenital disorders, classified by multiple different schemes. Type IA (Armitage) and type I (Benjamin and Inglis) laryngeal clefts exhibit absence or hypoplasia of the interarytenoid muscles with an intact cricoid ring. Submucous or “occult” clefts occur with intact mucosa but absent underlying cartilage and/or muscle. Children with a diagnosis of posterior laryngeal cleft often have other congenital anomalies or medical disorders, including gastroesophageal reflux, tracheomalacia, syndrome complexes, and developmental delay. These associated disorders often confuse the diagnostic picture, as they may contribute to or account for the presenting symptoms of a laryngeal cleft. We propose a method to help clarify the clinical significance of the laryngeal clefts in these patients, and determine which patients would benefit from cleft repair. Eight patients with type IA laryngeal clefts are presented who were treated with a “test dose” Gelfoam injection into the interarytenoid area at the time of endoscopic diagnosis. All patients exhibited clinical improvement, and 4 patients showed improvement on the postinjection videofluoroscopic swallow study as compared to preoperative studies. One patient has gone on to surgical repair of the cleft after multiple injections. Endoscopic Gelfoam injection at the time of diagnosis can both alleviate symptoms and provide clarification of the posterior laryngeal cleft's contribution to the clinical status of the patient in these often complex cases.

title: Clinical course following endoscopic repair of type 1 laryngeal clefts.
abstract: Treatment of type I laryngeal clefts (T1LCs) remains controversial. We present our experience with 16 endoscopic T1LC repairs to evaluate the effect of patient characteristics and surgical technique on outcomes.



A retrospective study was performed. Diagnosis of T1LC was made by interarytenoid palpation during operative microlaryngoscopy. Two surgeons performed endoscopic repair using either microflap reconstruction or laser demucosalization and reapproximation. All patients received preoperative and postoperative modified barium swallow (MBS) studies. Improved MBS at 3-5 months determined success of repair. Factors contributing to success of repair were analyzed statistically.



No intraoperative complications occurred. One T1LC repair dehisced after 3 months. Overall, 11 of 16 repairs (68.8%) were successful. Mean age at repair was 23.3 months. Length of stay for microflap repair was significantly shorter than for laser reapproximation (0.89 days vs. 4.6 days, p<0.001, two-tail t-test). The difference in patient age between failures and successes (21.3 months vs. 24.2 months) was non-significant (p=0.661, two-tail t-test). Success for the nine patients receiving microflap reconstruction (77.8%) vs. the seven receiving laser reapproximation (57.1%) is comparable (p=0.596, Fisher's exact test). No correlation between comorbidities and failure was found (p>0.05, Fisher's exact test).



This series matches the largest reported series of endoscopic T1LC repairs. Success rates were lower than in previously reported studies, and comorbidities were higher. However, comorbidities did not contribute to surgical failure. No difference in outcome was seen between the two endoscopic techniques. Microflap repair may require a shorter hospital stay.