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The phenotypically heterogeneous, autosomal recessive Vici syndrome was first described in 1988 in a sister and brother with oculocutaneous albinism, agenesis of the corpus callosum, cataract, cardiomyopathy, cleft lip, and immunodeficiency. Only 14 cases of Vici syndrome have yet been reported, several involving morphologic and functional defects in addition to those described in the initial case. We report on a 3-month-old Turkish girl with Vici syndrome associated with laryngomalacia, further expanding the clinical spectrum. We also review clinical features in all 15 Vici syndrome patients, to distinguish general from less common signs. To the best of our knowledge, this report is the first of a Turkish patient with Vici syndrome., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: Laryngomalacia is best known as a self-resolving infantile disorder characterized by inspiratory stridor with occlusion of the larynx by collapse of arytenoid tissues due to Bernoulli forces. Adult laryngomalacia has been sporadically described in the literature. We identified a series of patients with aerodynamic supraglottic collapse mimicking laryngomalacia in our Otolaryngology clinic., Study Design: Case series., Methods/patients: A series of five patients from our Otolaryngology clinic with aerodynamic supraglottic collapse presented with complaints ranging from noisy breathing to dyspnea with exertion. Diagnosis was made using rest and exercise flexible laryngoscopy., Results: Symptoms resolved in all patients who underwent traditional or modified supraglottoplasty., Conclusions: These patients, all with abnormal corniculate/cuneiform motion occluding the airway during forceful inspiration, reinforce the diagnostic role of rest and exercise flexible laryngoscopy in patients with dyspnea and stridor. These results may suggest that aerodynamic supraglottic collapse is an underdiagnosed clinical entity., (Published by Mosby, Inc.)

Objective: To identify and appraise the evidence for an association between laryngomalacia (LM) and acid reflux through a systematic review of the existing literature., Data Sources: MEDLINE, EMBASE, the Cochrane Library, Google Scholar, and collected additional publications cited in bibliographies., Review Methods: Literature search by both authors with structured criteria to select studies evaluated for systematic review. The Oxford Centre for Evidence-Based Medicine (CEBM) guidelines were applied to assess study quality of evidence., Results: Twenty-seven studies, representing 1295 neonates with LM, were included. Levels of evidence varied from CEBM level 2a (n = 1) to 4 (n = 23). Although reflux definitions were diverse, overall reflux prevalence in this group was 59% (pooled odds ratio [OR] of 4 controlled studies = 1.15, P = .67). Further evidence supporting an association between reflux and LM included the ubiquity of acid reflux using dual-probe pH monitoring in children with LM (2 studies; n = 84), the increased prevalence of reflux in severe as compared with mild LM (3 studies; n = 237; pooled OR = 9.86, P < .0001), case series and reports of LM improvement with antireflux therapy (6 studies; n = 275), and histological evidence of reflux-related laryngeal inflammation in children with LM (2 studies; n = 18)., Conclusion: The literature shows a coexistence between acid reflux and LM, but the evidence for a causal association is limited. In view of the widespread use of antireflux treatment in LM, a randomized controlled trial of antireflux medication vs placebo appears justified.

Laryngomalacia is defined as collapse of supraglottic structures during inspiration. It is the most common laryngeal disease of infancy. Laryngomalacia presents in the form of stridor, a high-pitched, musical, vibrating, multiphase inspiratory noise appearing within the first 10 days of life. Signs of severity are present in 10% of cases: poor weight gain (probably the most contributive element), dyspnoea with permanent and severe intercostal or xyphoid retraction, episodes of respiratory distress, obstructive sleep apnoea, and/or episodes of suffocation while feeding or feeding difficulties. The diagnosis is based on systematic office flexible laryngoscopy to confirm laryngomalacia and exclude other causes of supraglottic obstruction. Rigid endoscopy under general anaesthesia is only performed in the following cases: absence of laryngomalacia on flexible laryngoscopy, presence of laryngomalacia with signs of severity, search for any associated lesions prior to surgery, discrepancy between the severity of symptoms and the appearance on flexible laryngoscopy, and/or atypical symptoms (mostly aspirations). The work-up must be adapted to each child; however, guidelines recommend objective respiratory investigations in infants presenting signs of severity., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

The most common cause of stridor in newborns is instability of the upper larynx, called laryngomalacia. In approximately 10% of children normal development is impaired. The diagnostics and therapy must follow a clear plan that is also comprehensible to the parents. The obstruction should be localized endoscopically, the anatomical characteristics determined, and the surgical possibilities evaluated. Additional pathological changes of the upper airway (e.g., vocal fold paresis) need to be excluded. Surgery for laryngomalacia, called supraglottoplasty, allows reduction of excess mucus, transectioning of aryepiglottic folds that are too short, and in some cases epiglottic fixation to the base of the tongue. In extreme cases tracheotomy is unavoidable. Surgery is only recommended for severe cases; when carried out correctly according to the medical indications, the success rate is over 90%.

Obstructive sleep apnea in infants has a distinctive pathophysiology, natural history, and treatment compared with that of older children and adults. Infants have both anatomical and physiological predispositions toward airway obstruction and gas exchange abnormalities; including a superiorly placed larynx, increased chest wall compliance, ventilation-perfusion mismatching, and ventilatory control instability. Congenital abnormalities of the airway, such as laryngomalacia, hemangiomas, pyriform aperture stenosis, choanal atresia, and laryngeal webs, may also have adverse effects on airway patency. Additional exacerbating factors predisposing infants toward airway collapse include neck flexion, airway secretions, gastroesophageal reflux, and sleep deprivation. Obstructive sleep apnea in infants has been associated with failure to thrive, behavioral deficits, and sudden infant death. The proper interpretation of infant polysomnography requires an understanding of normative data related to gestation and postconceptual age for apnea, arousal, and oxygenation. Direct visualization of the upper airway is an important diagnostic modality in infants with obstructive apnea. Treatment options for infant obstructive sleep apnea are predicated on the underlying etiology, including supraglottoplasty for severe laryngomalacia, mandibular distraction for micrognathia, tonsillectomy and/or adenoidectomy, choanal atresia repair, and/or treatment of gastroesophageal reflux.

Objectives: Rapid anatomical evaluation is essential to establish the severity of cases with upper respiratory obstruction and to define the degree of respiratory distress. Detailed airway endoscopy is required in most patients, not only for diagnosis, but also to treat the condition. In this study, as two of the largest paediatric pulmonology centres in Turkey, we reviewed the data of our bronchoscopy patients, and aimed to document the upper airway abnormalities that we detected during these procedures., Patients and Methods: A retrospective analysis was made of the records of 1076 paediatric cases with pulmonary/airway disease who had undergone flexible bronchoscopy between 2007 and 2011., Results: Upper airway malacia disorders were the most common (79.6%, n=259) bronchoscopic findings detected in the patients. The other most common pathologies were laryngeal edema (12.9%, n=42), external tracheal compression (12.3%, n=40), subglottic stenosis (4.0%, n=13), tracheal stenosis (2.8%, n=9), and vocal cord paralysis/irregularity (2.8%, n=9). The mean duration of symptoms was shortest in patients with vocal cord paralysis, and longest in patients with tracheal nodules (p<0.001)., Conclusion: Paediatricians should keep in mind the possibility of malacia disorders and other congenital and acquired upper airway abnormalities in children with chronic respiratory problems. Diagnosis of underlying diseases, as soon as possible, permits the withdrawal of antibiotics or antiasthmatic drugs often used unnecessarily for long periods to treat these children., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

The objective of the present work was to study epidemiology of congenital stridor as a leading symptom of laryngeal malformation. The continuous sampling method was employed to perform the retrospective analysis of the growth charts of the patients attending three children's polyclinics in Moscow (9.625 patients born between 2005 and 2009). In addition, the medical histories of 4.623 newborn and breast-fed babies under the age of 1 year admitted to the Department of Newborn and Neonatal Pathology, Saint Vladimir City Children's Clinical Hospital, and 347 patients of the Department of Reconstructive Laryngeal Surgery were analysed. The children with the history of tracheal intubation in the preceding period were excluded from the study. The frequency of congenital stridor annually diagnosed in the aforementioned polyclinics varied from 0.17 to 5.8% compared with 1.5% in the general population. It was 2.21 to 3.14% (mean 2.47%) among the children treated at the Clinical Hospital. In the children under the age of 1 year, congenital malformations accounted for 90.8% of all laryngeal diseases. The principal cause of stridor was shown to be laryngomalacia. This pathology was diagnosed in 91.9% of the cases included in this study. In 11.2% of the patients, this condition occurred in combination with other congenital pathologies. It is concluded that the diagnosis of congenital stridor is an indication for laryngeal endoscopy regardless of the children's age starting from the first day of life. Meeting this recommendation allows the cause of stridor to be established and the treatment strategy to be developed on an individual basis.

Objective: To determine the clinical and polysomnography outcomes in infants with laryngomalacia undergoing supraglottoplasty., Methods: Infants (<1 year old) who underwent polysomnography pre- and post-supraglottoplasty at our tertiary referral center between 2003 and 2009 were reviewed retrospectively. Outcome measures included changes in stridor, sleep disordered breathing, swallowing, and polysomnography parameters before and after surgery. Pre- and postoperative distributions were compared using a Wilcoxon signed-rank test., Results: 20 children met inclusion criteria. Statistically significant improvements were found in pre- to postoperative median values for apnea-hypopnea index (AHI) (median change: -6.4 points, p=0.02) and obstructive apnea index (median change: -2.9 points, p=0.01) values. Obstructive AHI, O₂ saturation nadir, and percentage of sleep spent with <90% O₂ saturation improved, although not significantly. Postoperative AHI scores correlated with improvements in stridor in 80% of patients. Stratification showed similar improvements in AHI after supraglottoplasty regardless of reflux treatment, secondary airway lesions, age at supraglottoplasty, or time lag between supraglottoplasty and postoperative polysomnography. Secondary airway lesions are the only potential confounders shown to have significantly better improvements in O₂ nadir. While the sleep study improved in patients with AHI >5, infants with AHI <5 had worse polysomnography parameter values postoperatively. Five patients had dysphagia that resolved within one month postoperatively; otherwise, supraglottoplasty resulted in no complications., Conclusion: Supraglottoplasty may be an effective and safe option to improve moderate to severe obstructive sleep apnea in infants with laryngomalacia. In addition, polysomnography may be useful in infants with laryngomalacia to identify obstructive sleep apnea and/or to objectively measure outcomes after supraglottoplasty. This pilot study warrants a larger, prospective, and controlled study to validate these findings., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objectives: To determine how often the pediatric otolaryngology service is involved in the initial care of infants with an apparent life-threatening event (ALTE), to assess the usefulness of bronchoscopy and laryngoscopy in diagnosing the underlying etiology, and to describe the long-term airway outcomes and whether these patients were seen by the pediatric otolaryngology service over a 5-year follow-up period., Design: Retrospective observational study., Setting: Tertiary children's hospital affiliated with a university hospital., Patients: Screened were 187 903 patient visits for infants younger than 12 months. A total of 1148 infants with an ALTE were identified, 471 of whom met study inclusion criteria. To identify the study population, these infants were cross-referenced against a database of 5156 patients who underwent airway evaluation by the pediatric otolaryngology service., Main Outcome Measures: Airway evaluation with or without intervention., Results: Four hundred seventy-one infants with an ALTE met study inclusion criteria, 9 of whom subsequently underwent airway evaluation via bronchoscopy, laryngoscopy, or both. Three were referred during their initial ALTE admission, and 6 were seen later in childhood. Five of 9 patients had normal findings, 3 patients had laryngomalacia (including 2 with laryngeal edema), and 1 patient had adenotonsillar hypertrophy. Interventions consisted of 2 supraglottoplasties and 1 adenotonsillectomy., Conclusions: Among well-appearing infants hospitalized with an ALTE, 98.1% (462 of 471) did not undergo subsequent airway evaluation, and only 0.6% (3 of 471) ultimately required pediatric otolaryngologic surgical intervention during 5 years after the event. This study shows that otolaryngologists are not frequently consulted for well-appearing infants with an ALTE and that airway abnormalities are rare.

Objective: To document the prevalence of secondary airway lesions (SALs) among children with laryngomalacia (LM)., Study Design: A cross-sectional observational study., Setting: Single practice in a tertiary care pediatric facility., Subjects and Methods: Patients diagnosed with LM were initially identified from a prospectively kept surgical database. Patients with cardiac/large-vessel malformations, prematurity, repeated or prolonged intubations, acute infections, or prior airway surgery were excluded. Only children who had a full examination of the airway were included. Other associated diagnoses along with demographics, surgical intervention, and type of LM were included., Results: Over an 8-year period, 1112 patients underwent laryngoscopy and bronchoscopy. A total of 108 consecutive cases were diagnosed with LM (78 primary diagnosis and 30 secondary diagnosis). After excluding 17 with disorders associated with large airway disease, 91 LM patients were included. The mean age was 553.23 days (ranging between 5 days and 15 years); 60 were boys and 31 were girls. The prevalence of SALs was 7.7% (95% confidence interval, 7.65-7.76; 7 children; 4 subglottic stenosis, 4 tracheomalacia, 1 bronchomalacia, and 1 choanal atresia). Fifty-nine patients underwent supraglottoplasties, while 1 patient had a tracheostomy., Conclusions: After accounting for conditions known to be associated with large airway lesions, the prevalence of SALs associated with laryngomalacia was much less than previously reported. The authors hypothesize that the upper-end figures are a reflection of highly select cohorts and the inclusion of patients with other pathology known to be associated with subglottic stenosis, tracheomalacia, and bronchomalacia and low thresholds for diagnosing mild subglottic stenosis.

Two infants presented with intermittent stridor and evidence of laryngomalacia on flexible laryngoscopy. The first was a 10-month-old girl who had undergone 3 supraglottoplasty surgeries at an outside institution, without long-term resolution of symptoms. She was found during our evaluation to have a Chiari malformation. Laryngomalacia symptoms resolved after suboccipital decompression and C1 laminectomy, and the patient remained symptom-free at 6-month follow-up. The second infant was a 24-day-old boy with velocardiofacial syndrome who was found to have posterior cervicomedullary junction compression at the level of C1. He underwent C1 laminectomy for decompression of the brain stem, which resulted in immediate resolution of symptoms, and he remained symptom-free at 12-month follow-up. Neurologic abnormalities have been reported in up to 50% of infants with laryngomalacia. As such, brain stem dysfunction should be considered among the causes of laryngomalacia during evaluation, especially in patients with failure of supraglottoplasty. Both of these infants had resolution of symptoms after their neurosurgical procedures.

Aspiration is a suspected cause of chronic respiratory disease in infants. We assessed the probability of aspiration by immunocytochemical staining of alveolar macrophages for milk proteins (α-lactalbumin and β-lactoglobulin) and compared these findings with the Oil-Red-O staining score. Broncho-alveolar lavage (BAL), 24-hr esophageal pH-measurement and/or gastro-esophageal scintigraphy were performed in 111 children. Seventy-nine patients were enrolled. Ten exclusively soya milk formula fed children served as a control group. Individual scores, expressed as the mean percentage of positive staining macrophages counted by three blinded authors were made. Relying on the control group, a positive score was defined as a value higher than 1%. A positive score was found in 26% (18/69). Forty-four percent (8/18) of them had positive gastro-esophageal reflux (GER) tests. In 61% (11/18) a concomitant diagnosis of laryngo-/tracheomalacia was made. A positive score was found in 48% (11/23) of patients with laryngo-/tracheomalacia, compared to 15% (7/46) in infants with normal laryngeal and tracheal anatomy. No correlation was found between the immunocytochemical staining score for milk proteins and the Oil-Red-O staining score. We conclude that assuming the 1% criterion, persistent respiratory symptoms were associated with a positive immunostaining score, suggestive for aspiration, in 26% of infants, in 48% in case of concomitant laryngo- and/or tracheomalacia and in 15% of infants with normal laryngeal and tracheal anatomy. No correlation was found between the immunocytochemical staining score for cow milk proteins and the Oil-Red-O staining score., (Copyright © 2010 Wiley-Liss, Inc.)

Objective: To analyse the efficacy of CO(2) laser arytenoidectomy in the management of bilateral vocal cord paralysis in children., Methods: Retrospective series of 17 patients who underwent laser arytenoidectomy for bilateral vocal cord between 1995 and 2008 in a tertiary care institution. All patients had bilateral laryngeal paralysis, in isolation (n=5) or associated with concomitant airway conditions (n=12). All cases had anterior prolapse of the arytenoids with partial obstruction of the airway on inspiration. 12/17 patients (70.5%) were tracheotomy-dependant, 2/17 were in-extubatable, and 3/17 had severe airway limitation, effort dyspnea and poor sleep pattern. Main outcome measures were decannulation rate for patients with tracheotomy, occurrence of aspiration and quality of voice., Results: The mean age was 2.8 years old. 9/12 patients with tracheotomy (75%) were decannulated with a median delay of 2 months (2 days to 18 months). Both of the intubated patients were extubated with a median delay of 36h. One of the decannulated patients who re-presented with a residual dyspnea after the arytenoidectomy was improved by a further laser cordotomy. 2/17 patients (11.7%) had post-operative persistent aspirations (with pneumopathies in one case), 5/17 patients were dysphonic, 3 improved with speech therapy and 2 with intracordal lipoinjection., Conclusions: Laser arytenoidectomy is effective for improving the breathing in children presenting with a bilateral vocal fold paralysis associated with obstructive arytenoid prolapse. Results are good as a first-line surgery or following laryngo-tracheal surgery. Voice outcomes are satisfactory. However, aspiration is a rare complication., (Copyright (c) 2010 Elsevier Ireland Ltd. All rights reserved.)

Introduction: Laryngomalacia is the most frequent congenital anomaly of airways, and it may cause obstructive sleep apneas. The associated vocal cord paralysis may aggravate the symptoms of upper airway obstruction., Case Report: In a 14 month old boy severe laryngomalacia and bilateral vocal cord paralysis were diagnosed by flexible bronchoscopy. A sleep study showed a severe obstructive sleep apnoea (OSA). The patient was ventilated at home via the face mask with non invasive mechanical ventilation (CPAP) for a year. The level of pressure had to be set at 7 cm H2O to correct desaturation with an improvement in mean SpO2. On the follow up bronchoscopic examination laryngomalatia was improved, vocal cord paralysis persisted and sleep study revealed significant improvement., Discussion: In the patient with severe laryngomalatia and bilateral vocal cord paralysis with OSA conservative treatment with CPAP was used instead of a surgical intervention. Non invasive ventilation was used every night, for at least 6 hours, without adverse events. Invasive measurement of transdiaphragmatic pressure is the best way of titrating of CPAP level. This case report suggests the efficacy of noninvasive titrating of CPAP level by the hemoglobin oxygen saturation trend measurement., Conclusion: In case of severe laryngomalatia and associated vocal cord paralysis, followed by OSA non invasive ventilation by nasal CPAP represents an effective and safe alternative to surgery.

Waardenburg syndrome (WS) is a rare autosomal dominant condition characterised by sensorineural hearing loss, in conjunction with pigmentary abnormalities and defects of the neural crest-derived tissues. Depending on the additional phenotypic characteristics, WS is classified into four types, viz. WS1, WS2, WS3 and WS4. We report a 45-day-old male infant with WS1, who presented with inspiratory stridor associated with difficulty in respiration. Direct flexible laryngoscopic examination during evaluation confirmed laryngomalacia as the cause of the symptoms. The baby was managed conservatively and was discharged with appropriate advice to the mother, including the need for evaluation at regular intervals. There was gradual improvement in his symptoms, and by one year of age, he was completely symptom free. To our knowledge, laryngomalacia as a part of WS has not been documented to date in the English literature. We also briefly discussed the probable embryological basis for the observed association.

Objective: Supraglottoplasty is well documented as an effective procedure in the management of moderate to severe laryngomalacia. Traditionally assessed outcomes in the treatment of laryngomalacia include: reduced stridor, improved feeding and a resumption of weight-gain. Previous authors have documented the use of polysomnography in assessing the severity of paediatric laryngeal abnormalities. The aim of the current study was to objectively assess the efficacy of supraglottoplasty for laryngomalacia by comparing data from pre- and post-operative polysomnography., Methods: A retrospective review was performed of patients undergoing supraglottoplasty for laryngomalacia at a tertiary referral paediatric hospital over a 35-month period. Patients were required to have undergone full overnight polysomnography both before and after supraglottoplasty. Pre- and post-operative polysomnographic results were compared and the statistical significance between mean values was determined., Results: From a total of 46 patients, 10 were suitable for inclusion into the study. Mean age at first presentation was 2 months and 19 days (range 30-134 days). The surgical procedure performed was bilateral division of aryepiglottic folds alone in 1 patient (1/10), bilateral division of aryepiglottic folds and bilateral trimming of arytenoid mucosa in 5 patients (5/10) and bilateral aryepiglottic fold division, bilateral arytenoid mucosal trimming and epiglottic trimming or epiglottopexy in 4 patients (4/10). Statistically significant improvements occurred in mean values for Total Sleep Time (TST) (P=0.049), Lowest Oxygen Saturation Levels (SpO2 nadir) (P=0.006), Obstructive Apnoea Hypopnoea Index (OAHI) (P=0.009) and Respiratory Disturbance Index (RDI) (P=0.002), following supraglottoplasty. An improvement in mean Transcutaneous Carbon Dioxide (TcCO2) value occurred, but did not achieve statistical significance (57.1 vs. 52.8) (P=0.259). The mean age at which post-operative polysomnography confirmed a reversal of abnormal respiratory parameters following surgery was 5 months and 18 days., Conclusions: Polysomnography is an effective method for objectively assessing the efficacy of supraglottoplasty for laryngomalacia. Supraglottoplasty effectively reverses the abnormal respiratory parameters occurring in moderate to severe laryngomalacia.

Objectives: We examined the incidence and significance of aspiration in infants with severe laryngomalacia (LM) who undergo supraglottoplasty., Methods: We performed a 5-year retrospective review of a prospective database from 2 tertiary care pediatric institutions. The studied patients were 50 consecutive infants with severe LM who underwent supraglottoplasty (median age, 4.5 months) and functional endoscopic evaluation of swallowing (FEES) with or without laryngopharyngeal stimulation testing. The intervention was cold-knife microlaryngeal supraglottoplasty and reflux management. The main outcome measure was aspiration resolution., Results: Preoperative FEES identified laryngeal penetration in 44 infants (88%) with severe LM. Laryngeal penetration with aspiration beyond the vocal folds was noted in 36 infants (72%). Postoperative FEES (median follow-up, 3.8 months) indicated resolution of laryngeal penetration and aspiration in 36 (81.8%) and 31 (86.1%) of these patients, respectively. The 14 patients without preoperative aspiration showed no evidence of aspiration after supraglottoplasty. In patients with aspiration, the mean preoperative laryngopharyngeal stimulation test threshold was 8.45 mm Hg. This improved on average by 4.0 mm Hg after supraglottoplasty (paired t-test, p <0.0001). Multiple medical comorbidities were present in the 5 patients who had persistent aspiration after supraglottoplasty, including congenital heart disease (all 5 patients), congenital syndromes (4 patients), neurologic disorders (2 patients), and a need for tracheostomy (2 patients)., Conclusions: Laryngeal penetration and aspiration improve after cold-knife supraglottoplasty. Supraglottoplasty does not cause aspiration in patients who do not have preoperative aspiration. Supraglottoplasty may not improve aspiration in patients with multiple medical comorbidities.

Objectives: Stridor is a relatively common symptom during the neonatal period. The most probable cause of inspiratory stridor and supralaryngeal airway obstruction in infancy is laryngomalacia. Laryngeal cysts are known to be found in association with supraglottic prolapse and are a rare yet potentially lethal cause of respiratory distress in the newborn. In the absence of more alarming presenting signs, the vallecular cyst, a form of laryngeal cyst, frequently defies diagnosis. We present a series of illustrative cases to raise awareness of vallecular cyst to help reduce the therapeutic delays that are currently encountered clinically., Methods: A retrospective case series of four patients was reviewed. Each patient presented initially with stridor and additional signs of upper airway obstruction shortly after birth and was later diagnosed with vallecular cyst., Results: The predominant presenting signs were stridor (four cases), signs of respiratory distress (three cases), failure to thrive (three cases), poor feeding (two cases), and cyanotic spells (one case). Age at presentation ranged from 16 days to 8 months. A definitive diagnosis was achieved by flexible laryngoscopy in all four cases. Coexistent laryngomalacia was found in three of the four cases. Primary outcomes at 1 month following marsupialization were complete remission in all four cases., Conclusions: The challenge in making an early diagnosis of vallecular cyst, especially when laryngomalacia is comorbid, has been reaffirmed in our case series. Diagnosis requires a high index of clinical suspicion in combination with careful inspection of the tongue base with direct examination and/or appropriate imaging modalities.

Objectives: We sought to determine the incidence of secondary airway lesions in infants with laryngomalacia and to compare the incidences of these concomitant airway lesions in infants with severe, moderate, and mild laryngomalacia; to determine whether infants with mild or moderate laryngomalacia have a higher incidence of surgical intervention when a secondary airway lesion is present; and to determine whether the incidence of gastroesophageal reflux disease (GERD) is affected by the presence of a secondary airway lesion., Methods: We performed a retrospective review of a database consisting of 201 infants with a diagnosis of laryngomalacia treated at a pediatric tertiary referral center between June 1998 and June 2003. Data pertaining to demographic information, severity of laryngomalacia, presence of secondary airway lesions, and diagnosis of GERD were collected and analyzed., Results: Of the 201 infants, 104 (51.7%) were found to have a secondary airway lesion. Subglottic stenosis was found in 38.8%, and tracheomalacia in 37.8%. Of the infants with severe laryngomalacia, 30 (79%) had a diagnosis of a secondary lesion, compared with 51 (61.5%) of those with moderate and 23 (28.8%) of those with mild disease. Among infants with mild or moderate disease, those with secondary airway lesions were more likely to require surgical intervention than were infants without secondary airway lesions (27% versus 5.6%; p = 0.0002). There was no difference in the rates of secondary airway lesions in infants managed with supraglottoplasty versus tracheotomy. The incidence of GERD in this patient population was 65.6%. Infants with a secondary airway lesion were more likely to have GERD than were those without a secondary airway lesion (84.6% versus 45.4%; p <0.0001)., Conclusions: The incidence of secondary airway lesions in our population of infants with laryngomalacia was higher than those previously reported. The severity of disease correlated with the diagnosis of a secondary airway lesion. Secondary airway lesions lead to an increased incidence of surgical intervention and GERD in infants with laryngomalacia.

Objectives: We describe the clinical characteristics and management of vocal fold paralysis in infants who were born with a tracheoesophageal fistula (TEF)., Methods: This retrospective case series included all infants born with TEFs who presented to our pediatric otolaryngology unit and intensive care unit because of dyspnea or aphonia in the years 2005 and 2006, and who were found to have vocal fold paralysis., Results: Five boys and 1 girl were studied. One infant had stridor before TEF repair, and 5 after it. All children underwent flexible laryngotracheobronchoscopy and were treated in the pediatric intensive care unit before diagnosis of the vocal fold paralysis (5 bilaterally and 1 unilaterally) was made. The ages at diagnosis of paralysis ranged between 14 days and 14 months. Five infants required tracheostomy., Conclusions: Vocal fold paresis in infants is difficult to diagnose. The risk for recurrent laryngeal nerve injury associated with TEF and TEF repair should be emphasized in these children. We recommend that all newborns with TEF should be examined by an otolaryngologist before operation to confirm the mobility of the vocal folds and to rule out other associated airway malformations, and examined after operation if respiratory difficulties develop.

Laryngomalacia is the most common laryngeal anomaly and cause of stridor in newborns. Up to 20% of infants with laryngomalacia present with life-threatening disease that necessitates surgical management in the setting of severe airway obstruction and feeding disorders. Surgical correction of laryngomalacia has evolved over the past century from open tracheostomy to endoscopic modalities. This article provides a guide to patient assessment, surgical indication, operative technique, and perioperative management of patients with laryngomalacia. A review of surgical outcomes and complications is presented. Medical comorbidities often accompany patients with the least successful outcomes. Although complications are rare, they most commonly include persistent disease, supraglottic stenosis, and lower respiratory tract infections.