Laryngomalacia is the condition responsible for 75% of the cases of stridor in children aged up to 30 months, in which there is supraglottic collapse during inhalation. Inspiratory stridor is a characteristic telltale. As many as 20% of the patients are severely affected and require surgery. Supraglottoplasty is the procedure of choice and the presence of comorbidities is the most relevant prognostic factor for surgery success.To describe a series in a tertiary pediatric hospital, its success rates, and surgery prognostic factors.This retrospective cohort study enrolled 20 patients submitted to supraglottoplasty between July 2007 and May 2011.Thirteen (65%) patients were males; mean age at the time of the procedure was 6.32 months. Endoscopic examination showed that 12 subjects had combined forms of laryngomalacia, 40% had associated pharyngomalacia, and three also had tracheomalacia. Thirteen subjects had isolated laryngomalacia and seven had gastroesophageal reflux disease. Fifteen (75%) patients underwent aryepiglottic fold resection. After the procedure, eleven patients were asymptomatic and two required tracheostomy. Presence of comorbidities was the strongest predictor of unfavorable postoperative outcome (p = 0.034).Supraglottoplasty is a safe therapeutical procedure for select patients with laryngomalacia.

We report on a father and his 4-year-old son sharing a characteristic dysmorphic facial phenotype (including hyperteleroism, prominent forehead, and wide nasal bridge), macrocephaly, hearing loss, palatal clefting, developmental delay, hypotonia and bony abnormalities including marked cranial sclerosis and sclerosis of the ribs and long bones, which evolved in severity in the son between the ages of 2 and 4 years. The father's radiographs also showed prominent coarse striations, patchy metaphyseal sclerotic plaques, markedly increased bone density and cortical thickening of long bones, and significant degenerative changes in the thoracic spine. The son has an additional history of sleep apnea resulting from multi-level airway obstruction that includes adenoid hypertrophy, lingual tonsil hypertrophy, subglottic stenosis, and supra-arytenoid tissue consistent with laryngomalacia and tracheomalacia. The clinical, radiographic, and genetic findings in father and son are consistent with a sclerosing skeletal dysplasia syndrome with similarities to mixed sclerosing bone dysplasia (MSBD) including metaphyseal plaques, osteopathia striata, and cranial sclerosis (OS-CS). This family may represent one of the first descriptions of familial inheritance and evolving phenotype in MSBD. The evidence for male-male transmission would support the existence of an autosomal mechanism of inheritance for a novel form of MSBD with characteristic syndromic features.

The aim is to determine clinical characteristics, flexible bronchoscopy (FB) findings including associated airway abnormalities and other conditions, treatment modalities and long term follow-up of children with congenital stridor. Medical records of children, who underwent FB for the evaluation of stridor between 1 January 2004 and 31 December 2009 were retrospectively reviewed. Demographic characteristics, symptoms and physical examination findings at presentation, FB findings, follow-up data including the time to resolution of symptoms and treatment modalities, presence of associated conditions were assessed. 109 children were enrolled to the study. Laryngomalacia was the most common etiology for stridor. Laryngomalacia was isolated in 37 patients and 54 patients had secondary airway lesions (SALs). Diagnoses other than laryngomalacia such as subglottic hemangioma, subglottic web, isolated tracheomalacia were found in 18 patients. In 90 % of patients, stridor resolved before 3 years of age without any surgical intervention and there was no significant difference in terms of the persistence of stridor between patients with isolated laryngomalacia and associated SALs. Duration of stridor was significantly longer in both patients with neurological abnormalities and reflux symptoms. Surgical procedure was performed in 19 of the patients. There is a high incidence of SALs in patients with laryngomalacia. FB is helpful for identifying anomalies requiring surgical treatment.

To report on airway endoscopic findings and gastrointestinal and atopic conditions in a large consecutive series of atypical croup.Case series with chart review.Tertiary pediatric referral center.A surgical database was searched for all children who underwent full airway endoscopy to investigate atypical croup. The primary outcome measure was the prevalence of large airway lesions in patients with atypical croup undergoing endoscopy. Demographics, secondary diagnoses, and rate of positive findings were documented. Age and atopy were correlated using Spearman's correlation coefficient, and multivariate analysis identified predictors of large airway lesions.Eighty patients were identified over a period of 8 years (58 boys; mean [SD] age 4.8 [3.8] years; range, 46 days to 13.7 years). Of the 80 children, 31 had positive airway findings, with 33 large airway lesions demonstrated, including 10 subglottic stenosis, 7 laryngeal clefts, 6 subglottic hemangiomas, 4 tracheomalacia, and 3 laryngomalacia. Esophagitis was diagnosed in 36 children, 5 of whom had eosinophilic esophagitis. Thirty-five children had an atopic condition including asthma, allergic rhinitis, eosinophilic esophagitis, and food allergies. Age correlated with associated atopy (coefficient 0.4, P < .0001) and predicted the presence of any airway lesion (coefficient -0.0625, P < .001) and subglottic stenosis in particular (coefficient -0.0362, P = .001). Prior intubation predicted subglottic stenosis (coefficient 0.267, P = .011).Thirty-nine percent of airway endoscopies demonstrated large airway lesions. When eosinophilic esophagitis was sought, it was confirmed in over 1:10 patients. The findings bolster the case for airway endoscopy coupled with allergy and gastrointestinal investigations.

Protracted bacterial bronchitis is defined as the presence of more than 4 weeks of chronic wet cough that resolves with appropriate antibiotic therapy, in the absence of alternative diagnoses. The diagnosis of protracted bacterial bronchitis is not readily accepted within the pediatric community, however, and data on the incidence of bacterial bronchitis in children are deficient. The objective of this study was to determine the frequency of bacterial bronchitis in children with chronic wet cough and to analyze their bronchoscopic findings.We performed a retrospective review of charts of children who presented with chronic wet cough, unresponsive to therapy, before referral to the pediatric pulmonary clinic.A total of 197 charts and bronchoscopy reports were analyzed. Of 109 children who were 0 to 3 years of age, 33 (30.3%) had laryngomalacia and/or tracheomalacia. The bronchoscopy showed purulent bronchitis in 56% (110) cases and nonpurulent bronchitis in 44% (87). The bronchoalveolar lavage bacterial cultures were positive in 46% (91) of the children and showed nontypable Haemophilus influenzae (49%), Streptococcus pneumoniae (20%), Moraxella catarrhalis (17%), Staphylococcus aureus (12%), and Klebsiella pneumoniae in 1 patient. The χ(2) analysis demonstrated that positive bacterial cultures occurred more frequently in children with purulent bronchitis (74, 69.8%) than in children with nonpurulent bronchitis (19, 19.8%) (P < .001).Children who present with chronic wet cough are often found to have evidence of purulent bronchitis on bronchoscopy. This finding is often indicative of a bacterial lower airway infection in these children.

The aim of this study was to describe and compare the airway findings in term and preterm infants with laryngomalacia.A retrospective review of 130 patients diagnosed as having laryngomalacia at a tertiary referral center between July 2004 and August 2009 was conducted. Medical records were reviewed for demographic data, supraglottic and glottic airway findings, concomitant airway lesions, and the need for intervention.The mean gestational age and age at diagnosis was 36 and 15 weeks, respectively. Combined posterior and anterior supraglottic collapse was the most common finding (31%). Posterior collapse alone occurred in 25%, anterior collapse in 14%, and lateral collapse in 10%. Twelve percent of patients had all 3 sites of collapse. Forty-one percent of patients had a secondary airway lesion, with tracheomalacia being the most common. Preterm infants had significantly higher rates of reflux and more sites of collapse than did term infants (P < .0001). Eight patients required an intervention for their symptoms.Children with laryngomalacia tend to have more than 1 area of supraglottic collapse, and more than one third have a secondary lesion. All patients who required an intervention had more than 1 area of collapse, and 63% of these patients had a secondary airway lesion. Our high incidence of secondary lesions is similar to recent reports.

Inflation and assessment of the endotracheal tube cuff pressure is often not appreciated as a critical aspect of endotracheal intubation. Appropriate endotracheal tube cuff pressure, endotracheal intubation seals the airway to prevent aspiration and provides for positive-pressure ventilation without air leak.Correlations between manual methods of assessing the pressure by an experienced anesthesiologists and assessment with maintenance of the pressure within the normal range by the automated pressure controller device were studied in 100 patients divided into two groups. In Group M, endotracheal tube cuff was inflated manually by a trained anesthesiologist and checked for its pressure hourly by cuff pressure monitor till the end of surgery. In Group C, endotracheal tube cuff was inflated by automated cuff pressure controller and pressure was maintained at 25-cm H(2)O throughout the surgeries. Repeated measure ANOVA was applied.Repeated measure ANOVA results showed that average of endotracheal tube cuff pressure of 50 patients taken at seven different points is significantly different (F-value: 171.102, P-value: 0.000). Bonferroni correction test shows that average of endotracheal tube cuff pressure in all six groups are significantly different from constant group (P = 0.000). No case of laryngomalacia, tracheomalacia, tracheal stenosis, tracheoesophageal fistula or aspiration pneumonitis was observed.Endotracheal tube cuff pressure was significantly high when endotracheal tube cuff was inflated manually. The known complications of high endotracheal tube cuff pressure can be avoided if the cuff pressure controller device is used and manual methods cannot be relied upon for keeping the pressure within the recommended levels.

In children with persistent respiratory symptoms despite regular anti-asthma inhalation treatment, diagnostic investigations to exclude underlying disease are warranted. 124 children were prospectively enrolled, and 24-h oesophageal pH measurement and fibreoptic bronchoscopy with bronchoalveolar lavage (BAL) were performed. BAL fluid (BALF) was processed for neutrophil counting and bacterial culture. Inflammation of the respiratory mucosa was assessed. A structural abnormality of the central airways was found in 47% of subjects (40% females). In 19% of subjects, neither anatomical anomalies nor inflamed respiratory mucosa were observed, whereas in 64%, definite macroscopic mucosal inflammation was observed. Inflammation of the respiratory mucosa was associated with a significantly higher percentage of neutrophils in the BALF: median (interquartile range) 48 (14-82)% compared with 7 (0-16)% (p<0.025). A positive BALF culture was found in 62% of the infants with mucosal inflammation compared with 25% in the group without inflammation (p<0.016). 56% of the BALF samples were positive for bacterial culture. In children with persistent respiratory symptoms, nearly half have anatomical anomalies of the central airways. In 62% of the children with mucosal inflammation, a positive BAL culture and a significantly higher percentage of BALF neutrophils were detected.

To document the prevalence of secondary airway lesions (SALs) among children with laryngomalacia (LM).A cross-sectional observational study.Single practice in a tertiary care pediatric facility.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.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.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.

The Robin sequence, previously known as the Pierre Robin syndrome, is characterized by the sequence of clinical events that result from a small mandible. The tongue becomes posteriorly displaced (glossoptosis) and obstructs the airway. The obstructing tongue also makes oral feeding difficult and, in severe cases, impossible. Patients with Robin sequence may also present with a cleft palate, although this is not necessary to make the diagnosis. The small mandible either results from an inherent growth problem (genetic or syndromic) or is deformational where intrauterine growth of the mandible is restricted. Conditions of syndromic patients are generally more severe and difficult to treat. The phenomenon of "catch-up" growth is disputed, although it is likely to occur in deformational but not syndromic patients. Patients with Robin sequence are best managed by a multidisciplinary team familiar with the diagnoses and treatment of difficult pediatric airways. Approximately 70% of Robin infants are successfully managed by prone positioning alone. Tracheostomy is necessary in approximately 10% of these patients, especially for subglottic obstructions including laryngomalacia and tracheomalacia. Tracheostomy should be avoided if possible. Treatment of the remaining 20% remains highly contentious. Tongue-lip adhesion, nasopharyngeal airways, and distraction osteogenesis all have strong advocates. Ideally, treatment should be individualized. Patients who will have catch-up growth of the mandible will only need a tongue-lip or nasopharyngeal airway as a temporary measure. Patients who we know will not have catch-up growth will benefit from early distraction osteogenesis.