The following statements summarize our interpretation of the literature regarding submucous cleft palate: Incidence and Diagnosis of Submucous Cleft Palate 1. In surveys of classic stigmata of submucous cleft palate among the general population, the incidence has been reported to be 0.02 to 0.08 percent. In the larger of these series, the incidence of velopharyngeal inadequacy among patients identified to have submucous cleft palate was 1 to 9. The incidence of occult submucous cleft palate is not known, since these patients will only be detected during the evaluation of patients who present with velopharyngeal inadequacy. 2. The diagnosis of submucous cleft palate is made by identification of the classic stigmata on physical examination. The diagnosis of occult submucous cleft palate is only pursued if the patient has velopharyngeal inadequacy. 3. For consistency in evaluating and reporting data, patients with an overt cleft of the secondary palate that extends beyond the uvula should be reported as having a cleft palate, and not a submucous cleft palate, even if a submucous cleft exists in a portion of the palate anterior to the overt cleft. 4. The true incidence of otitis media with effusion in the presence of submucous cleft palate has yet to be determined using a prospective study. Surgical Treatment of Velopharyngeal Inadequacy in Patients with Submucous Cleft Palate 1. The technique that has most consistently been documented to result in a significant correction of velopharyngeal inadequacy is the pharyngeal flap. There is recent evidence from one large center supporting the efficacy of the Furlow Z-plasty in selected patients with submucous cleft palate. Both these procedures appear to be most effective in patients with good lateral pharyngeal wall motion. 2. If a pharyngeal flap is performed as the primary procedure to act as an obturator against which the lateral pharyngeal walls appose for closure, we do not see the need for adjunctive palatal procedures. The dynamic component of velopharyngeal competence following such a pharyngeal flap consists of lateral wall motion, which is not enhanced by further surgical manipulation of the palate. However, a pharyngeal flap may be performed as an adjunctive procedure to a palatal pushback in order to provide lining for the resultant defect in the nasal mucosa. 3. The present literature does not support "prophylactic" operations on patients who present with the physical stigmata of submucous cleft palate prior to reaching an age at which it can be demonstrated by perceptual speech assessment that velopharyngeal inadequacy remained refractory to speech therapy. A significant number of patients will never develop velopharyngeal inadequacy; therefore, surgery would be unnecessary. In addition, objective data regarding the outcomes of different surgical techniques cannot be gathered if patients with submucous cleft palate are operated on without having had velopharyngeal inadequacy documented prior to those operations. 4. In order to objectively compare the outcomes...

BackgroundRRP is a devastating disease in which papillomas in the airway cause hoarseness and breathing difficulty. The disease is caused by human papillomavirus (HPV) 6 or 11 and is very variable. Patients undergo multiple surgeries to maintain a patent airway and in order to communicate vocally. Several small studies have been published in which most have noted that HPV 11 is associated with a more aggressive course.Methodology/Principal FindingsPapilloma biopsies were taken from patients undergoing surgical treatment of RRP and were subjected to HPV typing. 118 patients with juvenile-onset RRP with at least 1 year of clinical data and infected with a single HPV type were analyzed. HPV 11 was encountered in 40% of the patients. By our definition, most of the patients in the sample (81%) had run an aggressive course. The odds of a patient with HPV 11 running an aggressive course were 3.9 times higher than that of patients with HPV 6 (Fisher's exact p = 0.017). However, clinical course was more closely associated with age of the patient (at diagnosis and at the time of the current surgery) than with HPV type. Patients with HPV 11 were diagnosed at a younger age (2.4y) than were those with HPV 6 (3.4y) (p = 0.014). Both by multiple linear regression and by multiple logistic regression HPV type was only weakly associated with metrics of disease course when simultaneously accounting for age.Conclusions/Significance AbstractThe course of RRP is variable and a quarter of the variability can be accounted for by the age of the patient. HPV 11 is more closely associated with a younger age at diagnosis than it is associated with an aggressive clinical course. These data suggest that there are factors other than HPV type and age of the patient that determine disease course.

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Patients with PRS require thorough airway and feeding evaluation. Those with additional syndromic diagnoses demonstrate higher rates of more invasive interventions. Patients with PRS must undergo individualized approaches with consideration of multiple factors for successful management.

Subglottic stenosis is the most common cause of chronic airway obstruction. It results in prolonged tracheal cannulation of infants and children. Following the widespread adoption over the past 20 years of prolonged intubation for respiratory support in neonates, the incidence of acquired subglottic stenosis increased dramatically. On January 1, 1987, we began a 3-year prospective study to delineate potential etiologic factors involved in the development of subglottic stenosis in neonates. The present study analyzes data from 289 infants. Relationships between birth weight, gestational age, endotracheal tube size, duration of intubation and ventilation, number and difficulty of intubations, and the subsequent need for medical and surgical therapy are discussed. Whole organ larynges from autopsy specimens provide histological correlation.

Background Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. Methods A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included. Exploratory data analysis was conducted followed by inferential analyses with frequentist and Bayesian statistics. Results We examined 339 subjects: 82% were diagnosed prior to the age of 18 years, 65% were infected with HPV6, and 69% had an aggressive clinical course. When comparing age at diagnosis with clinical course, the probability of aggressiveness is high for children under five years of age then drops rapidly. For patients diagnosed after the age of 10 years, an indolent course is more common. After accounting for confounding between HPV11 and young age, HPV type was minimally associated with aggressiveness. Fast and Frugal Trees (FFTs) were utilized to determine which algorithms yield the highest accuracy to classify patients as having an indolent or aggressive clinical course and consistently created a branch for diagnostic age at ~5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors. Conclusion In the largest cohort of its type, we have identified a critical age at diagnosis which demarcates a more aggressive from less aggressive clinical course.

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