Congenital Laryngeal Anomalies Research Articles

Management of Pediatric Bilateral Vocal Fold Paralysis: A State-of-the-Art Review of Etiologies, Diagnosis, and Treatments.

This paper reviews the current literature about epidemiology, etiologies, diagnosis, and management of pediatric bilateral vocal fold paralysis (PBVFP). According to PRISMA statements, a narrative review of the current literature was conducted through the PubMed, Scopus, and Cochrane Library databases about the epidemiology, etiologies, diagnosis, and management of PBVFP. PBVCP is the second most common congenital laryngeal anomaly in the pediatric population, accounting for 10% to 20% of pediatric laryngeal conditions. PBVCP is related to idiopathic (42.2%), congenital (19.7%), and neurological (16.9%) conditions. A tracheotomy is required in 60% of cases regarding stridor and dyspnea, which are the most prevalent symptoms. The diagnosis is based on the etiological features, clinical presentation, laryngoscopic findings, and objective examinations. Laryngeal electromyography may be used to support the diagnosis in difficult cases, but its reliability depends on the practitioner's experience. The primary differential diagnosis is posterior glottis stenosis, which needs to be excluded regarding therapeutic and management differences with PBVCP. Transient surgical procedures consist of tracheotomy or laterofixation of the vocal fold. Current permanent procedures include uni- or bilateral partial arytenoidectomy, posterior transverse cordotomy, cricoid splits, and laryngeal selective reinnervation. There is no evidence of the superiority of some procedures over others. PBVCP is the second most common laryngeal disorder in the pediatric population. Diagnosis is based on etiological and clinical findings and may require the use of laryngeal electromyography. Therapeutic management may involve several transient or permanent surgical procedures that are associated with overall subjective improvements in symptoms, laryngeal findings, and low complication rates.

Neonatal stridor presents at home – vocal fold paralysis as rare presenting feature of CHARGE syndrome

Abstract Objectives To present an unusual presentation and diagnosis of CHARGE syndrome with vocal fold paralysis, a rarely associated congenital laryngeal anomaly, as the presenting feature. Case presentation A four-day old, full-term, male infant born via uncomplicated vaginal delivery with a nursery course significant for failed hearing screen presented to an emergency department (ED) with respiratory distress and worsening stridor. He was transferred to a level III neonatal intensive care unit (NICU) for further evaluation and required intubation due to progressive hypercarbia. Laryngoscopy revealed left-sided unilateral vocal fold paralysis (VFP). He underwent further evaluation that included a normal MRI brain, neck and chest. Genetics was consulted with concern for dysmorphic features on physical exam. Following gene panel testing, VFP was attributed to known association with CHARGE syndrome. Airway edema was noted on laryngoscopy that prevented extubation until two months of age. Further features of CHARGE syndrome identified included colobomas, glaucoma, sensorineural hearing loss, and genital abnormalities. He was discharged in room air and following gastrostomy tube placement with otolaryngology follow up. Conclusions Although choanal abnormalities are classically associated with CHARGE syndrome, other upper airway anomalies such as VFP may be present. VFP is a rarely reported anomaly in association with CHARGE syndrome (Naito Y, Higuchi M, Koinuma G, Aramaki M, Takahashi T, Kosaki K. Upper airway obstruction in neonates and infants with CHARGE syndrome. Am J Med Genet 2007;143A:1815–20; Morgan D, Bailey M, Phelps P, Bellman S, Grace A, Wyse R. Ear-nose-throat abnormalities in the CHARGE association. Arch Otolaryngol Head Neck Surg 1993;119:49–54).

Surgical Management of Anterior Glottic Webs.

Congenital webs are rare and represent <5% of all congenital laryngeal anomalies. They are usually a partial laryngeal atresia rather than a true web, and present as a thick and fibrotic web with subglottic extension and associated subglottic stenosis. All patients with a congenital anterior glottic web should be evaluated for chromosome 22q11.2 deletion syndrome. Management strategies are mainly based on the severity of airway obstruction and the anatomical extension of the webs. Simple division of the web endoscopically may be adequate for rare thin webs, however, an open approach is usually warranted for thick glottic webs regardless of Cohen grades. Open repair can be either with keel placement or reconstruction of the anterior commissure.

Complete polysomnographic parameters in infants with severe laryngomalacia prior to and after supraglottoplasty

IntroductionLaryngomalacia is the most common congenital laryngeal anomaly. Because of supraglottic prolapse, laryngomalacia may be associated with obstructive sleep apnea (OSA) and sleep disturbances. The effects of OSA and sleep disorders in children include failure to thrive, cognitive and behavioral disturbances, cardiovascular compromise, and an association with sudden infant death syndrome. ObjectiveTo evaluate the presence of OSA and sleep disturbances in children with severe laryngomalacia through complete nocturnal polysomnography, as well as to establish the effects of supraglottoplasty in each of the polysomnographic parameters. ResultsNine infants with severe laryngomalacia were included, all with a complete polysomnographic study prior to and after supraglottoplasty. The average age was 5.5 months. All patients presented an Apnea-Hypopnea Index (AHI) within the range of severe OSA. After supraglottoplasty, a significant reduction in AHI was found, from 34.87 ± 20.34 to 9.44 ± 5.28 after surgery (p: 0.022). Additionally, sleep efficiency had a significant increase, from 21.4% to 56.29% of total sleep time (p: 0.0013). All patients presented a significant decrease in obstructive apnea episodes (p < 0.0001), as well as in hypopnea episodes (p: 0.0154). The mean and minimum peripheral oxygen saturation (SpO2) had a significant increase after supraglottoplasty from 88.2% to 94.09% (p: 0.0002), and from 81.01% to 89.33% (p < 0.0001), respectively. ConclusionPolysomnography (PSG) may provide better surgical sustenance in infants with severe laryngomalacia and OSA, as well as, serving as a monitoring tool of success. However, the surgical decision should not be reduced to polysomnographic results, and a good history and examination remain as the fundamental criteria.

Pediatric Laryngeal Anomalies

Summarize current knowledge regarding the epidemiology, presentation, workup, and management of common and major congenital laryngeal anomalies. Familiarize readers, particularly those providing primary care pediatric services, with these lesions. Knowledge of these anomalies continues to evolve rapidly, with ongoing wide variation in workup and treatment strategies. Nevertheless, some core principles can be extracted from the current medical literature. The pediatric larynx is anatomically small but functionally critical. Many anomalies can mimic each other despite requiring very different workup and treatment strategies. In some cases, these anomalies may be life-threatening or may significantly impair function. Familiarity with the nuances of these conditions will likely be useful to primary and specialty providers caring for pediatric patients.

Increased prevalence of obstructive sleep apnea in patients with pectus excavatum: A pilot study.

Objective:Laryngomalacia is the most common congenital laryngeal anomaly and is associated with pectus excavatum (PE). Patients with laryngomalacia and patients with obstructive sleep apnea (OSA) both experience upper airway obstruction, and patients with laryngomalacia had been found to have a higher prevalence of PE. However, no studies have established the prevalence of OSA in patients with PE. We conducted this pilot study to evaluate the prevalence of OSA in patients with PE.Materials and Methods:A total of 42 patients ≥20 years old with PE who were admitted for Nuss surgery to correct PE in Taipei Tzu Chi Hospital between October 2015 and September 2016 were invited to participate in the study; 31 of the 42 patients agreed. All 31 patients completed an Epworth sleepiness scale questionnaire to evaluate excessive daytime sleepiness (EDS) and underwent overnight polysomnography to evaluate OSA before Nuss surgery.Results:The prevalence of snoring in the study participants was 100%. Ten of 31 patients (32.3%) reported EDS. The overall prevalence of OSA with an apnea/hypopnea index ≥5/h was 25.8%, and all patients with OSA were men.Conclusions:The prevalence of OSA in patients with PE seemed to be higher than that previously reported in the general population, implying that OSA might be a potential etiology or, at least, an aggravating factor for the development or progression of PE or might be responsible for the postoperative recurrence of PE in some patients. Further studies are needed to clarify this relationship.

Laryngomalacia: Our Clinical Experience.

The aim of this study is to analyse the clinical symptoms, follow-up and treatment properties of the laryngomalacia patients that we encountered between 2009 and 2014. Records of 81 laryngomalacia patients who were followed up in our clinic between 2009 and 2014 were retrospectively analysed. Patients' gender, age, time of onset of the symptoms, chief complaints, other co-existing congenital laryngeal anomalies and treatment and follow-up properties were evaluated. Of the 81 patients, 48 were male and 33 were female, and the mean age was 4.9 months. The average period of follow-up was 12.1 months. The chief complaints at the time of admission were stridor (100%) and episodic cyanosis with feeding (27.16%). Symptoms of 75 patients were resolved at an average of 8.2 months with conservative treatment. Three patients underwent supraglottoplasty. Tracheotomy and posterior cordotomy was performed for a patient with co-existing vocal cord paralysis. Additional tracheotomy was necessary for a patient with pulmonary co-morbidities and for another with co-existing subglottic stenosis. Laryngomalacia is the most common cause of stridor in infants. The majority of laryngomalacia patients can be managed conservatively by close follow-up. For patients in whom respiratory and feeding problems persist or growth retardation develops, surgical treatment is performed. Tracheotomy may be necessary for a small group of patients with additional diseases.

Surgical Management of Severe Case of Laryngomalacia

ABSTRACT Incidence of congenital laryngeal anomalies is between 1:10,000 and 1:50,000 births. Laryngomalacia is the most common cause of congenital stridor, characterized by partial or complete collapse of the supraglottic structures during inspiration. Patient was having inspiratory stridor at birth or immediately after birth. No active intervention is required in 90% of cases. So we are reporting this rare case that required active intervention and will also show how we managed it. How to cite this article Mahore D, Kadam S, Rane SV, Paikrao Y. Surgical Management of Severe Case of Laryngomalacia. Int J Phonosurg Laryngol 2016;6(1):47-48.

Surgical treatment of severe laryngomalacia: Six month follow up.

Laryngomalacia (LM) is the most common congenital anomaly of larynx that causes stridor in children. We evaluated the efficacy of epiglottic suture and laser epiglottopexy for treatment of infants with severe LM. Surgical intervention was performed in 19 patients with severe LM, after the diagnosis was established using flexible laryngotracheobronchoscopy. Five patients had isolated type 1 LM, and 14 patients had a combination of type 1 and 3 LM. Indication for surgical treatment was the presence of LM with at least one of the following: malnutrition (body mass index [BMI] Z score < -2 SD), dysphagia or symptoms of gastroesophageal reflux and mean oxygen saturation (SaO2 ) <92% with oxygen desaturation index (ODI) > 3. Epiglottic suture was performed in 11 patients, and laser epiglottopexy in eight, at mean age 3.95 ± 2.4 months. Rate of operation was 2.2-fold greater for more severe anomaly (combination of type 1 and 3 LM) than for isolated type 1. At 6 month follow up symptoms had gradually improved, as well as nutritional status, with increase of mean BMI Z score from -3.7 to -0.9 (P < 0.01). Mean preoperative SaO2 was 89.4 ± 4.3% with mean ODI of 5.8. At 6 month follow up, mean SaO2 was 96.7 ± 1.1%, and mean ODI was 1.2 (P < 0.01). Epiglottic suture and laser epiglottopexy are efficient surgical techniques that lead to significant improvement of symptoms, oxygenation and nutritional status in patients with LM.

Upper airway obstruction.

1. Jordan Virbalas, MD* 2. Lee Smith, MD† 1. *Department of Otorhinolaryngology–Head and Neck Surgery, Albert Einstein College of Medicine, Bronx, NY. 2. †Department of Otolaryngology, Steven and Alexandra Cohen Children’s Medical Center, Hofstra North Shore LIJ School of Medicine, New Hyde Park, NY. * Abbreviations: BT: : bacterial tracheitis CT: : computed tomography HIB: : Haemophilus influenzae type B The differential diagnosis of upper airway obstruction in children includes infectious and noninfectious causes (Table). When evaluating a child with stridor, the clinician must know how to differentiate between various anatomical anomalies (laryngomalacia, tracheomalacia, and subglottic stenosis) and infectious conditions (croup, epiglottitis, and bacterial tracheitis) to promptly implement appropriate management. View this table: Table. Common Causes of Upper Airway Obstruction After completing this article, readers should be able to 1. Know that upper respiratory tract infections and airway obstruction in young infants can lead to respiratory distress. 2. Know the clinical presentation of laryngomalacia. 3. Know the risks of examination of patients with suspected epiglottitis. 4. Know how to treat a child with epiglottitis. 5. Know the clinical manifestations of laryngotracheitis (croup). 6. Know the appropriate management of croup. 7. Differentiate the clinical and radiographic findings of viral croup from those of epiglottitis and bacterial tracheitis. 8. Distinguish between viral and noninfectious croup. 9. Recognize the signs and symptoms of bacterial tracheitis. 10. Know the typical clinical course of bacterial tracheitis, including biphasic illness, precipitous worsening, requirement for intubation, and relatively prolonged intubation. 11. Know the treatment of bacterial tracheitis. 12. Know the microbiology of bacterial tracheitis. 13. Know that tracheomalacia can occur as a complication of long-term mechanical ventilation in children. 14. Know that tracheoesophageal fistula may result in tracheomalacia. 15. Know the clinical manifestations of tracheomalacia. ### Definitions Laryngomalacia refers to the prolapse of supraglottic structures into the laryngeal airway on inspiration, which usually manifests as a primarily inspiratory stridor in young children. ### Introduction Laryngomalacia is the most common congenital laryngeal anomaly and is the most frequent congenital cause of stridor in infants. Often symptoms are not present at birth, and affected children typically develop stridor in the first 2 …

Idiopathic bilateral vocal cord paralysis in infants: Case series and literature review.

Vocal fold paralysis is the second most common congenital laryngeal anomaly in newborns. Bilateral paralysis is a severe condition and often remains of unknown etiology. We report our experience of congenital idiopathic bilateral vocal fold paralysis in newborns and infants, and discuss the therapeutic options. Retrospective review. A retrospective review was carried out at a single tertiary referral center over a 15-year period of children presenting with congenital idiopathic bilateral laryngeal paralysis (CIBP). Twenty-six patients were identified and managed over period of the study (mean follow-up, 6.7 years). A tracheostomy was eventually performed in 14 patients, 12 during the neonatal period. Endoscopic treatment was performed in 16 patients and an external approach in five patients after endoscopic failure. Three patients were managed by watchful waiting. Spontaneous recovery occurred in 16 patients (median age, 14.5 months), including nine with a tracheostomy. Decannulation was achieved in 12 patients (median age, 42 months), and noninvasive positive pressure ventilation (NPPV) was applied in six patients. Only inhomogeneous series of pediatric bilateral laryngeal palsies have been published so far, making it difficult to identify prognostic and predictive markers, as well as therapeutic guidelines. Focusing on CIBP, our data suggest that the management strategy must take into account the possibility of a delayed spontaneous recovery. NPPV is an interesting therapeutic tool in this situation. A systematic somatic assessment is mandatory to look for associated conditions.

Congenital laryngeal anomalies

IntroductionIt is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. ObjectivesTo familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. MethodsRevision article, in which the main aspects concerning airway management of infants will be analyzed. ConclusionsIt is critical for clinicians to understand issues relevant to the airway management of infants.

Heterotopic Epiglottic Lymphoid Tissue (HELT) Causing Recurrent Epiglottitis

Problem: A unique case of recurrent infectious epiglottitis in an immunocompetent, fully immunized, non-syndromic 23 year old female. Method: Case report Results: Patient was successfully managed with an endoscopic partial epiglottectomy at two years of follow-up. Pathology revealed what appears to be sub-mucosal germinally-active heterotropic lymphoid tissue within the epiglottic soft tissues as the likely cause of the recurrent epiglottitis. Conclusions: The embryology of the lingual tonsils and epiglottis may provide a clue to the etiology of this case. Heterotrophic epiglottic lymphoid tissue may be considered a newly described, clinically significant congenital laryngeal anomaly that may not present until adulthood and should be considered in rare cases of recurrent infectious epiglottitis.

Utility of sleep study in children with laryngomalacia

Introduction Laryngomalacia (LM) is one of the most frequent congenital laryngeal anomalies in children and it has been associated with sleep disorder. Our main objective was to describe the sleep study findings in children with LM using polysomnography (PSG) or respiratory polygraphy (RP). Materials and methods We studied 18 children diagnosed with laryngomalacia by flexible nasolaryngoscopy. We performed a sleep study: PSG or RP and complete medical history on all of them. Results 18 children were studied, 5 by PSG and 13 by RP. 11 (61.1%) of them were females, with a mean age of 3.38 ( ± 3.01) months and weight 4674 ( ± 1418) g. LM type 1 was present in 7 (38.9%), type 2 in 6 (33.3%) and type 3 in 5 (27.8%) children. Premature birth described 4 (22.2%) children. Stridor was the most frequent symptom, in 15 (83.3%) followed by feeding difficulties in 8 (44.4%). Apneas were referred in 7 (38.8%), intercostal retraction and regurgitation in 6 (33.3%). Gastroesophageal reflux disease was confirmed in 10 (55.5%) by pHmetry requiring surgery in 2 cases. Echocardiography was performed in 14 (77.8%) showing cardiac anomalies in 4 (22.2%). In the sleep study the mean values were: sleep efficiency 83% ( ± 5.85), NREM% 36.9 ( ± 11.1), REM% 30.5 ( ± 7.7) and indeterminate 32.4% ( ± 18.2), apnea-hypopnea index (AHI) 13.74 ( ± 10.90), hypopnea 30.5 ( ± 48.2), obstructive apneas 58.8 ( ± 67.3), central apneas 13.6 ( ± 20.8), cumulative percentage of time spent at saturation ± 4.72), mean oxygen saturation 96% ( ± 1.94) and heart rate 126 ( ± 14.24) bpm. 16 children (88.8%) were diagnosed with sleep-disordered breathing (SDB). Conclusion Our findings confirmed the necessity to do a sleep study in children with laryngomalacia, regardless of the type of laryngomalacia in nasolaryngoscopy. Acknowledgements The authors would like to thank Dra. Navazo Eguia and the Sleep Unit staff at the Universitary Hospital of Burgos for their help.

Pectus excavatum in children with laryngomalacia

BackgroundLaryngomalacia is the most common congenital laryngeal anomaly and is associated with several disorders including gastric reflux, sleep apnea, hypotonia and failure to thrive. Pectus excavatum (PE) is the most common chest wall deformity affecting 1–300/1000 individuals. Though many authors presume a relationship between PE and laryngomalacia, there is no published data to establish this association. GoalTo test the hypothesis that patients referred to our pediatric otolaryngology clinic for evaluation of laryngomalacia exhibit higher rates of PE than the general population. MethodsRetrospective review of prospectively enrolled children who presented with laryngomalacia (January 2008–June 2012) to a tertiary care, hospital based, pediatric otolaryngology practice. Each chart was examined for a concurrent diagnosis of pectus deformity. ResultsOf the 137 laryngomalacia patients, 9 (6.6%) had documented PE. This represents a significantly increased rate of PE when compared to children without laryngomalacia (p=0.001). Four of the 9 children with PE underwent supraglottoplasty for laryngomalacia, a significantly greater proportion than the 9/128 of the children with isolated laryngomalacia who underwent supraglottoplasty (p=0.004). ConclusionsThis study suggests an association between laryngomalacia and PE. Pediatric otolaryngologists should be cognizant of this relationship, though further studies are needed to elucidate the nature of this association.

Etiology of laryngeal stridor in 297 cases of infants

Objective To summarize the cause of laryngeal stridor in infants in order to make accurate diagnosis and treatment of the disease.Methods We reviewed medical records of 297 cases of patients less than 3 year of age with the presenting symptom of stridor who were initially evaluated in the outpatient setting of otorhinolaryngological department from Jan 2005 to Jan 2010.The causes of stridor were clarified by examinations of ultrafine electronic laryngoscope,throat three-dimensional CT,and bronchoscopy in all cases.Patients underwent history-taking,physical examination and flexible laryngoscopy,CT examination or bronchoscopy evaluation in the operating room.Results Of all 297 patients,199 cases ( 67.0％ ) were diagnosed as congenital airway abnomalities for cause of stridor,which included congenital laryngeal abnomalities in 169(84.9％,169/199) and congenital tracheal abnormalities in 30 cases( 15.1％,30/199).Another 98 cases (33.0％,98/297) were diagnosed as acquired disease for cause of stridor.The most congenital laryngeal anomaly was laryngomalacia ( 159,94.1％,159/169 ).The most congenital tracheal abnormalities was tracheomalacia ( 14,46.7％,14/30 ).Sixty-four cases ( 65.3％,64/98 ) were diagnosed as foreign body in airway and 26 cases (26.5％,26/98) were respiratory infection,which were the first and second most common causes of acquired disease for stuidor.Conclusion Congenital airway structural abnormalities as a major cause of infant laryngeal stridor,followed by acquired disorders,including airway foreign body and infection. Key words: Laryngeal stridor; Laryngomalacia; Airway obstruction; Infant

Postextubation stridor in a child with asymptomatic trifid epiglottis with polydactyly – A rare case report

Abstract Trifid epiglottis is an extremely rare congenital laryngeal anomaly. Trifid anomaly of epiglottis has not been described in literature. In the embryo, epiglottis, hypothalamus, and digital buds develop synchronously. Some disturbances during this stage may account for the concurrence of trifid epiglottis and polysyndactyly. We report a trifid epiglottis in a 6 year old child with congenital bilateral polydactyly of hand and foot who was asymptomatic and developed postexubation mild stridor after general anesthesia for excision of the extra digit which was managed conservatively.

Congenital glottic web: Management and anatomical observation

Congenital anterior glottis web is a rare congenital laryngeal anomaly presenting usually by either dysphonia or airway obstruction. Several ways for managing this condition have been proposed including endoscopic, as well as external techniques. The purpose of this study is to present our experience and to review the experience of others with cases of congenital glottic web regarding their diagnosis, classification and management. It is a retrospective study, which included six infants and children; two groups were identified; first group included 3 patients who were treated by endoscopic laser lysis of webs, followed by topical application of mitomycin C (MMC) to the fresh wound, and the other group included 3 patients who underwent open surgical correction, by Laryngotracheal reconstruction (LTR). Information recorded included; age, sex, presentation, grading of lesions, surgery performed, residual web, and outcome. Assessment and documentation of subglottic extension independent from the glottic extension of the web was also done. All webs were grade 2, 3 and 4 according to Cohen's classification. We observed in five cases that high grade webs (grade 3 and 4) usually had thick subglottic extension, but in one case (case 2 laser) high grade web (grade 4) had thin subglottic extension. Complications included; two recurrences one after laser surgery and the other one after LTR. All appropriate patients were decannulated post LTR. Use of laser and MMC is good in thin glottic webs; LTR is indicated in glottic webs with thick subglottic extension. Not all high grade webs have thick subglottic extension. So, thickness of web and subglottic extension has to be assessed during microlaryngeal surgery, by laser incision and examination of web thickness to determine the appropriate management.

Stridor in a newborn caused by infantile myofibromatosis—A case review

Infantile myofibromatosis is a common fibrous tumor of infancy, however still considered a rarity. A newborn boy was examined due to inspiratory stridor and subcostal retractions since birth. Several subcutaneous nodules were present on the trunk and legs. Transnasal fiberoptic laryngoscopy and trachea-laryngoscopy showed discrete edema of the anterior part of the vocal cords but no other apparent abnormality. MRI raised suspicion of a laryngeal tumor. Through an external laryngofissure a lesion extending from the right hemilarynx was exposed and excised in fractions. Pathological examination showed infantile myofibromatosis. Stridor in newborns is frequently caused by congenital laryngeal anomalies, i.e. laryngomalacia but a neoplastic process should be considered as a differential diagnosis.

Stridor in a newborn caused by a congenital laryngocele and bifid epiglottis: A case report and review of the literature

A laryngocele is a rare, an abnormal cystic dilatation of the laryngeal saccule. A bifid epiglottis is also an extremely rare congenital anomaly of larynx that is often associated with other congenital defects. In this article, we report a case of an internal laryngocele and bifid epiglottis in a one hour old newborn girl that presented as acute airway obstruction who also have imperforate hymen, hydrocolpos, bilateral hydronephrosis and polydactyly. The patient underwent endoscopic microsurgical excision of internal laryngocele using suspension laryngoscopy and cold blade. We also review the anatomy, etiology and clinic course of the different types of laryngoceles, bifid epiglottis and related congenital defects.