How common are ear, nose and throat disorders in children with Noonan syndrome and other RASopathies?

INTRODUCTION: Noonan syndrome and related conditions (RASopathies) are known to be associated with abnormalities in many organ systems. It is our impression that few otolaryngologists are familiar with the manifestations of these syndromes and we therefore reviewed our hospital's patient cohort to identify the prevalence of ear, nose and throat disorders in these children. METHODS: We cross-referenced various hospital department databases (otolaryngology, audiology, cardiology, haematology and genetics) to try to identify as many children with Noonan and other RASopathies as possible. We then performed a retrospective review of electronic patient records. RESULTS: We identified 67 children with Noonan, Costello, LEOPARD and other RASopathy syndromes. Around half have been seen in otolaryngology and audiology clinics. Otitis media with effusion requiring ventilation tubes occurred in 4% of children. 10% have suffered recurrent acute otitis media. 9% have a sensorineural hearing loss. 7% have undergone adenotonsillectomy for obstructive sleep apnoea. Airway anomalies and head and neck malformations occur but are rare. DISCUSSION: Children with Noonan and other RASopathies present commonly to otolaryngology and audiology clinics. The prevalence of sensorineural hearing loss is high and audiological screening is likely to be worthwhile. Surgeons should be aware that complications of surgery are common and can be very severe, especially in those with cardiac anomalies.

Sinonasal Issues After Maxillectomy With Free Flap Reconstruction: Incidence and Clinical Approach.

OBJECTIVE: To characterize the clinical features associated with sinonasal complaints after maxillectomy with free flap reconstruction as well as propose a screening and treatment algorithm. METHODS: Retrospective review of patients who underwent maxillectomy and free flap reconstruction at a tertiary care center. RESULTS: Fifty-eight patients were included, 25 (43.1%) of them had documented sinonasal complaints postoperatively. Eleven patients subsequently underwent revision surgery for sinonasal complaints. Among the 25 patients with sinonasal complaints, 22 patients (88.0%) had nasal crusting, 17 (68.0%) had nasal obstruction, 12 (48.0%) had rhinorrhea, 9 (36.0%) had facial pain or pressure, and 7 (28.0%) had foul odor. Twenty-two patients (88.0%) had multiple sinonasal complaints. There was a higher incidence of both sinonasal complaints and surgical intervention in patients who underwent adjuvant radiation, but this was not statistically significant (47.7% vs 28.6%, P = .235; 29.4% vs 7.1%, P = .265). CONCLUSIONS: Sinonasal complaints are common following free flap reconstruction for a maxillectomy defect and should be screened for at postoperative visits, with early referral to a rhinologist for consideration of endoscopic sinus surgery. Nonsurgical treatment strategies include large-volume nasal saline irrigations, xylitol irrigations for persistent inflammatory symptoms, and culture-directed antibiotic irrigations for persistent infectious symptoms. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:67-72, 2022.

Self-reported work activities, eye, nose, and throat symptoms, and respiratory health outcomes among an industrial hog operation worker cohort, North Carolina, USA.

INTRODUCTION: Respiratory disease among industrial hog operation (IHO) workers is well documented; however, it remains unclear whether specific work activities are more harmful and if personal protective equipment (PPE), as used by workers, can reduce adverse health outcomes. METHODS: IHO workers (n = 103) completed baseline and up to eight bi-weekly study visits. Workers reported typical (baseline) and transient (bi-weekly) work activities, PPE use, and physical health symptoms. Baseline and longitudinal associations were assessed using generalized logistic and fixed-effects logistic regression models, respectively. RESULTS: At baseline, reports of ever versus never drawing pig blood, applying pesticides, and increasing years worked at any IHO were positively associated with reports of eye, nose, and/or throat irritation. Over time, transient exposures, associated with dustiness in barns, cleaning of barns, and pig contact were associated with increased odds of sneezing, headache, and eye or nose irritation, particularly in the highest categories of exposure. When PPE was used, workers had lower odds of symptoms interfering with sleep (odds ratio [OR]: 0.1; 95% confidence interval [CI]: 0.01-0.8), and eye or nose irritation (OR: 0.1; 95% CI: 0.02-0.9). Similarly, when they washed their hands eight times or more per shift (median frequency) versus less frequently, the odds of any respiratory symptom were reduced (OR: 0.3; 95% CI: 0.1-0.8). CONCLUSIONS: In this healthy volunteer worker population, increasingly unfavorable IHO activities were associated with self-reported eye, nose, throat, and respiratory health symptoms. Strong protective associations were seen between PPE use and handwashing and the odds of symptoms, warranting further investigation.

Nasal symptoms increase the risk of snoring and snoring increases the risk of nasal symptoms. A longitudinal population study.

PURPOSE: Humans have a preference for nasal breathing during sleep. This 10-year prospective study aimed to determine if nasal symptoms can predict snoring and also if snoring can predict development of nasal symptoms. The hypothesis proposed is that nasal symptoms affect the risk of snoring 10 years later, whereas snoring does not increase the risk of developing nasal symptoms. METHODS: In the cohort study, Respiratory Health in Northern Europe (RHINE), a random population from Denmark, Estonia, Iceland, Norway, and Sweden, born between 1945 and 1973, was investigated by postal questionnaires in 1999-2001 (RHINE II, baseline) and in 2010-2012 (RHINE III, follow-up). The study population consisted of the participants who had answered questions on nasal symptoms such as nasal obstruction, discharge, and sneezing, and also snoring both at baseline and at follow-up (n = 10,112). RESULTS: Nasal symptoms were frequent, reported by 48% of the entire population at baseline, with snoring reported by 24%. Nasal symptoms at baseline increased the risk of snoring at follow-up (adj. OR 1.38; 95% CI 1.22-1.58) after adjusting for age, sex, BMI change between baseline and follow-up, and smoking status. Snoring at baseline was associated with an increased risk of developing nasal symptoms at follow-up (adj. OR 1.22; 95% CI 1.02-1.47). CONCLUSION: Nasal symptoms are independent risk factors for development of snoring 10 years later, and surprisingly, snoring is a risk factor for the development of nasal symptoms.

Oronasal Fistula Risk After Palate Repair.

OBJECTIVE: To assess risk factors for oronasal fistula, including 2-stage palate repair. DESIGN: Retrospective analysis. SETTING: Tertiary children's hospital. PATIENTS: Patients with non-submucosal cleft palate whose entire cleft repair was completed at the study hospital between 2005 and 2013 with postsurgical follow-up. INTERVENTIONS: Hierarchical binary logistic regression assessed predictive value of variables for fistula. Variables tested for inclusion were 2 stage repair, Veau classification, sex, age at surgery 1, age at surgery 2, surgeon volume, surgeon, insurance status, socioeconomic status, and syndrome. Variables were added to the model in order of significance and retained if significant at a .05 level. MAIN OUTCOME MEASURE: Postoperative fistula. RESULTS: Of 584 palate repairs, 505 (87%) had follow-up, with an overall fistula rate of 10.1% (n = 51). Among single-stage repairs (n = 211), the fistula rate was 6.7%; it was 12.6% in 2-stage repairs (n = 294, P = .03). In the final model utilizing both single-stage and 2-stage patient data, significant predictors of fistula were 2-stage repair (odds ratio [OR]: 2.5, P = .012), surgeon volume, and surgeon. When examining only single-stage patients, higher surgeon volume was protective against fistula. In the model examining 2-stage patients, surgeon and age at hard palate repair were significant; older age at hard palate closure was protective for fistula, with an OR of 0.82 (P = .046) for each additional 6 months in age at repair. CONCLUSIONS: Two-stage surgery, surgeon, and surgeon volume were significant predictors of fistula occurrence in all children, and older age at hard palate repair was protective in those with 2-stage repair.

Prevalence of Frontal Sinus Aplasia in Jordanian Individuals.

Our paper aims to investigate the prevalence of frontal sinus aplasia among Jordanian individuals and compare it with values from different populations. Retrospectively, the study was done for 167 of the paranasal sinuses coronal tomography (CT) for patients who attended the clinic from January 2019 to August 2019 in King Abdullah University Hospital Irbid, Jordan. The authors analyzed the multiplanar CT scans to perform our work. The authors excluded the following images from our study: patients younger than 18 years old, patients with history of skull base trauma that impaired visualization of the frontal sinus, and images with fibro-osseous lesions that impaired the visualization of the frontal sinus. The prevalence of bilateral frontal sinus aplasia is 4.2% among Jordanian individuals and 6.6% is the prevalence of unilateral frontal sinus aplasia. Both values are consistent with average values among different populations. Moreover, we noticed the higher prevalence in both the bilateral and unilateral frontal sinus aplasia in males compared to females. The prevalence of frontal sinus aplasia among Jordanian individuals is almost within the same values among different populations. These numbers regarding the bilateral and unilateral frontal sinus aplasia is crucial to push the surgeon to evaluate CT of the paranasal sinuses preoperatively and focus on the presence of frontal sinuses on CT images to prevent unwanted complications during sinus surgeries.

Evaluation of nasal pathologies accompanying rhinoplasty cases at otorhinolaryngology and plastic, reconstructive, & aesthetic surgery clinics.

BACKGROUND AND OBJECTIVES: Rhinoplasty is a frequently performed surgical procedure. Besides an aesthetically successful outcome, determination of additional nasal pathologies and making a simultaneous intervention on these are also essential for a successful functional outcome. This study aims to determine the rates of accompanying nasal pathologies in patients undergoing rhinoplasty and reveals whether any additional interventions were performed during operation. MATERIALS AND METHODS: In this cross-sectional study, 496 Caucasian Turkish patients who underwent rhinoplasty at the Plastic, Reconstructive & Aesthetic Surgery and Otorhinolaryngology clinics of our research hospital between 2015 and 2018 were retrospectively examined. Among them, the results of 271 patients who had preoperative paranasal computerized tomography scans were evaluated. RESULTS: The mean age of the 271 patients was 28.8 years (Range: 17-55). Among them, 156 (57%) were female and 115 (42%) were male. The numbers of patients with septal deviation, inferior concha hypertrophy, unilateral/bilateral concha bullosa, nasal polyp, mucosal thickening, and retention cysts at the Otorhinolaryngology clinic were found to be 126 (82%), 77 (50%), 20 (13%), 10 (6.5%), 77 (50%), and 41 (27%), respectively, and these numbers were respectively 97 (82%), 60 (51%), 11 (9.3%), 2 (1.7%), 57 (48%), and 17 (14%) at the Plastic, Reconstructive & Aesthetic Surgery clinic. A statistically significant difference was found in terms of the presence of retention cysts between the clinics (95% CI -0.11% to 0.16%; P=.014; P<.005), and the presence of retention cysts was significantly higher at the Otorhinolaryngology clinic. A total of 18 (12%) patients were found to have additional surgical interventions. CONCLUSIONS: This study showed that additional nasal pathologies were frequently present in patients undergoing rhinoplasty operations. Hence, for a successful operation, it is essential to have Otorhinolaryngology consultation and detect accompanying pathologies in rhinoplasty cases which will be performed by Plastic, Reconstructive & Aesthetic Surgery specialists. LEVEL OF EVIDENCE: 4.

Environmental reservoir dynamics predict global infection patterns and population impacts for the fungal disease white-nose syndrome.

Disease outbreaks and pathogen introductions can have significant effects on host populations, and the ability of pathogens to persist in the environment can exacerbate disease impacts by fueling sustained transmission, seasonal epidemics, and repeated spillover events. While theory suggests that the presence of an environmental reservoir increases the risk of host declines and threat of extinction, the influence of reservoir dynamics on transmission and population impacts remains poorly described. Here we show that the extent of the environmental reservoir explains broad patterns of host infection and the severity of disease impacts of a virulent pathogen. We examined reservoir and host infection dynamics and the resulting impacts of Pseudogymnoascus destructans, the fungal pathogen that causes white-nose syndrome, in 39 species of bats at 101 sites across the globe. Lower levels of pathogen in the environment consistently corresponded to delayed infection of hosts, fewer and less severe infections, and reduced population impacts. In contrast, an extensive and persistent environmental reservoir led to early and widespread infections and severe population declines. These results suggest that continental differences in the persistence or decay of P. destructans in the environment altered infection patterns in bats and influenced whether host populations were stable or experienced severe declines from this disease. Quantifying the impact of the environmental reservoir on disease dynamics can provide specific targets for reducing pathogen levels in the environment to prevent or control future epidemics.

Nasal Valve Insufficiency in Dermatologic Surgery.

BACKGROUND: Few publications have addressed nasal valve (NV) insufficiency as a complication of Mohs micrographic surgery. OBJECTIVE: To comprehensively review the literature and further characterize the causes, prevalence, evaluation, and management of NV insufficiency as it relates to cutaneous oncology. MATERIALS AND METHODS: A PubMed search was completed to identify articles related to the NV in the context of Mohs micrographic surgery, cutaneous oncology, and reconstruction. RESULTS: Nasal valve insufficiency may occur during tumor removal and/or reconstruction. Defect size larger than 1 cm; location on the ala, sidewall, or alar crease; lack of structural support; and poorly planned flaps are the main risk factors for NV insufficiency. Several surgical techniques have been described to avoid and correct this complication. CONCLUSION: Nasal valve insufficiency may be an underrecognized and underreported complication of Mohs micrographic surgery. Nasal valve insufficiency may be identified with relatively simple tests. Knowledge of NV anatomy and preoperative function assessment is essential. Recognition of this potential complication and awareness of its prevention and management may decrease morbidity in patients undergoing cutaneous surgery.

Causative Factors for Complications in Transpalatal Advancement.

INTRODUCTION: Transpalatal advancement (TPA) is a procedure that is used when modern variants of uvulopharyn-gopalatoplasty are unable to provide enough anterior traction. Although successful in reduction of obstructive sleep apnea (OSA) parameters, it also comes with procedure-specific risks. Formation of an oro-nasal fistula (ONF) is a complication that results in significant morbidity and a protracted treatment course. METHODS: After approval from the University of Wollongong Health Research Ethics Committee, a retrospective chart review of all cases undergoing TPA performed by a single surgeon over a 10-year period from 2008 to 2018 was performed. Patients underwent pre- and postoperative level 1 or 2 polysomnography. Factors potentially contributing to palatal complications, as well as pre- and postoperative polysomnographic parameters, subjective sleep questionnaires, and body mass index (BMI) were statistically analyzed where a P value <.05 was considered a significant result. RESULTS: A total of 59 patients were included. Overall palatal complication rate was 25.4% (15/59), with the most common being transient velo-palatal insufficiency (VPI) (8/59, 13.6%). ONF developed in 4/59 (6.8%) of patients. None of the analyzed contributing factors for palatal complications were statistically significant, except the presence of a high-arched palate and development of ONF. All analyzed sleep parameters, as well as BMI, were significantly different when comparing pre- to postoperative results. CONCLUSION: This study suggests that TPA has a role in current sleep surgery paradigms and can significantly improve both objective and subjective outcome measures of OSA. Surgeons contemplating TPA on patients with high-arched hard palates should do so with caution.

A retrospective study of more than 400 feline nasal biopsy samples in the UK (2006-2013).

OBJECTIVES: The main objective of this study was to utilise a large database from a UK-based, commercial veterinary diagnostic laboratory to ascertain the prevalence of different forms of nasal disease within the feline population. Further objectives included using this database to detect any breed, sex or age predilections, or associations between the degree of brachycephalism, and the different conditions diagnosed. METHODS: Records from the laboratory were searched for feline submissions received between 31 May 2006 and 31 October 2013. For all samples taken from the nasal cavity, the diagnosis was recorded together with the breed, age, sex and neuter status of the cat, whether the clinical presentation was uni- or bilateral and whether a nasal discharge was present. Pedigree breeds were further subclassified according to skull conformation into brachycephalic, mesocephalic and dolichocephalic. Logistic regression models were constructed to assess the adjusted magnitude of association of significant risk factors with each disease, and each disease was also used as a potential independent risk factor for each other disease. RESULTS: The most prevalent nasal disease was rhinitis, followed by neoplasia and polyps. The most commonly diagnosed neoplasm was lymphoma, followed by adenocarcinoma and undifferentiated carcinoma, with benign tumours being very uncommon. No significant association was found between skull conformation and nasal diseases. The only statistically significant association was polyps being more likely to arise in younger male cats, with a mesocephalic skull conformation and no nasal discharge. CONCLUSIONS AND RELEVANCE: No significant association was found between skull conformation and nasal diseases, contrary to what might be expected. The only significant association found between any of the potential risk factors and various forms of nasal disease was polyps being more likely to arise in younger cats; other identified associations are only likely to be weak.

Virally-vectored vaccine candidates against white-nose syndrome induce anti-fungal immune response in little brown bats (Myotis lucifugus).

White-nose syndrome (WNS) caused by the fungus, Pseudogymnoascus destructans (Pd) has killed millions of North American hibernating bats. Currently, methods to prevent the disease are limited. We conducted two trials to assess potential WNS vaccine candidates in wild-caught Myotis lucifugus. In a pilot study, we immunized bats with one of four vaccine treatments or phosphate-buffered saline (PBS) as a control and challenged them with Pd upon transfer into hibernation chambers. Bats in one vaccine-treated group, that received raccoon poxviruses (RCN) expressing Pd calnexin (CAL) and serine protease (SP), developed WNS at a lower rate (1/10) than other treatments combined (14/23), although samples sizes were small. The results of a second similar trial provided additional support for this observation. Bats vaccinated orally or by injection with RCN-CAL and RCN-SP survived Pd challenge at a significantly higher rate (P = 0.01) than controls. Using RT-PCR and flow cytometry, combined with fluorescent in situ hybridization, we determined that expression of IFN-γ transcripts and the number of CD4 + T-helper cells transcribing this gene were elevated (P < 0.10) in stimulated lymphocytes from surviving vaccinees (n = 15) compared to controls (n = 3). We conclude that vaccination with virally-vectored Pd antigens induced antifungal immunity that could potentially protect bats against WNS.

The Effect of Gastric Helicobacter pylori Colonization on Nasal Functions.

The aim of this study is to investigate the effect of gastric Helicobacter pylori colonization on nasal functions. The study enrolled patients (n = 100) who underwent endoscopy for gastroesophageal reflux disease. Patients with laryngopharyngeal reflux (LPR) were identified by Reflux Symptom Index (RSI) and Reflux Finding Score (RFS). Patients were divided into 2 groups: LPR (+) (n = 64) H pylori (+), RSI > 13, RFS > 7; LPR (-) (n = 36) H pylori (+), RSI < 13, RFS < 7. Visual analog scale (VAS), sinonasal outcome test-22 (SNOT-22), peak nasal inspiratory flowmeter (PNIF), mucociliary clearance (MCC), and olfactory tests were used to evaluate the nasal functions. The average VAS for nasal obstruction, PNIF, and MCC did not differ significantly between the LPR (+) and LPR (-) groups (P > .05). However, the average olfactory test scores were lower in the LPR (+) patients than the LPR (-) patients (P < .05). Also, the SNOT-22 scores were significantly higher in LPR (+) patients than in LPR (-) (P < .01). Nasal functions and symptom scores were also evaluated according to the H pylori grading. The PNIF, MCC, SNOT-22, and olfactory test results deteriorated as the gastric mucosal H pylori colonization increased (P < .05). In conclusion, nasal functions differed between LPR disease and GERD only, while the density of H pylori colonization in the gastric mucosa had an effect on nasal function.

Environmental Triggers Associated With Empty Nose Syndrome Symptoms: A Cross-Sectional Study.

OBJECTIVES: Empty nose syndrome (ENS) is thought to have multiple etiologies, one of which is a postsurgical phenomenon resulting from excessive loss of nasal tissues, particularly the inferior turbinate. Given that the inferior turbinate is instrumental in maintaining nasal homeostasis in different environments, it is believed that ENS symptoms arise only in more arid regions of the world. The aim of this study was to recruit an international population of individuals with ENS to investigate the association of local climate factors on the incidence and severity of ENS-specific symptoms. METHODS: A cross-sectional study was performed of individuals from an international ENS database. ENS status was determined on the basis of a positive ENS questionnaire score (Empty Nose Syndrome 6-Item Questionnaire) and sinus computed tomographic imaging with supporting medical documentation. Participants completed a survey encompassing demographic, geographic, and symptom indicators. Climate variables were collected from global climate databases. Participant location was classified according to the Köppen-Geiger climate system. Pearson correlation analysis was performed using α = 0.05 to determine significance. RESULTS: Fifty-three individuals with ENS were included. Participants were distributed across 5 continents and 15 countries (representing 4 distinct Köppen-Geiger zones). Although local climate factors varied significantly within this cohort, no significant association was found between Empty Nose Syndrome 6-Item Questionnaire symptom severity and these climate factors. However, most study participants reported exacerbation of their ENS symptoms in response to dry air (94%), air conditioning (64%), changes in season and weather (60%), and transitioning between indoors and outdoors (40%). This suggests that everyday local environmental factors may influence the well-being of these patients more than global, climate-level shifts. CONCLUSIONS: ENS symptom severity does not appear to be related to climate or geographic factors. These findings deviate from the traditional dogma that ENS is experienced only in arid regions (or precluded in humid regions) and highlight the importance of recognizing this condition independent of geographic location.

Upper airway cough syndrome in 103 children.

BACKGROUND: In China, upper airway cough syndrome (UACS) is only less frequent than cough-variant asthma and accounts for 24.71% of chronic cough. This study aimed to determine the pathogenetic constituents and factors affecting UACS in children of different age groups, and to identify clinical clues for diagnosing UACS and a method for curative effect evaluation. METHODS: A total of 103 children with UACS whose chief complaint was chronic cough were studied from January to November 2013 at Children's Hospital, Capital Institute of Pediatrics. According to their age, children with UACS were divided into 3 groups: nursing children, pre-school children, and school-age children. We analyzed the differences in pathogenetic constituents and factors affecting UACS in children. The effect of UACS treatment was evaluated by the visual analog scale (VAS) and an objective examination. Chi-squared test and analysis of variance were performed with the SPSS 19.0 statistical software. RESULTS: There was a high incidence of UACS in school-age children. Rhinitis with adenoid hypertrophy was the main cause of 103 suspected UACS cases. Adenoidal hypertrophy was the major cause of UACS in the pre-school children group, while rhinitis was the major reason in the nursing children and school-age children groups. Among the 103 children, there were 45 allergen-positive children, with no significant difference among different age groups. VAS scores in the different disease groups after treatment were lower than those before treatment (all P < 0.01). VAS scores in different disease groups showed significant differences, except for 12 vs. 24 weeks after treatment (P = 0.023). Different age groups had different secondary complaints. CONCLUSIONS: There are different pathogeneses in different UACS age groups. Clinical treatment efficacy of children with UACS can be evaluated by the VAS combined with an objective examination. We recommend that the course of treatment should be 12 weeks.

The incidence of velopharyngeal insufficiency and oronasal fistula after primary palatal surgery with Sommerlad intravelar veloplasty: A retrospective study in Isfahan Cleft Care Team.

OBJECTIVES: This study was designed to determine the incidence of velopharyngeal insufficiency (VPI), oronasal fistula development and facial grimace in patients seen by Isfahan Cleft Care Team (ICCT) after primary Sommerlad intravelar veloplasty (SIVV). Furthermore the association of gender, cleft type and age at primary surgery with the incidence of hypernasality and fistula is determined. METHODS: A group of 40 patients with history of cleft palate with or without cleft lip were identified from the records of ICCT between 2011 and 2014. The main outcome measures were the incidence of hypernasality and fistula after primary palate repair with SIVV. Speech recordings were analyzed by consensus by two speech therapists according to the Cleft Audit Protocol for Speech- Augmented (CAPS-A), (Kappa = 82.4). Deciding whether or not to have a fistula was based on the oral examination videos. RESULTS: Severe and moderate hypernasality was observed in 42.5% of patients. Normal resonance and mild/borderline hypernasality was observed in 37.5% and 20% of patients, respectively. The frequency of fistulas was 7.5%. There was a significant association between hypernasality with cleft type and the age at primary surgery (p < 0.05). CONCLUSION: Significant progress has been made in the outcomes of the primary palate surgeries with the SIVV technique compared to the previous study in the ICCT.

Sinonasal and Laryngeal Sarcoidosis-An Uncommon Presentation and Management Challenge.

Sarcoidosis is a chronic inflammatory disease of uncertain etiology characterized by the formation of noncaseating granulomas. The thorax is involved in 95% of cases, but any organ can be involved. Sinonasal or laryngeal involvement is uncommon and can be difficult to diagnose. The reported incidence of sarcoidosis in the upper airway clearly depends on study characteristics, and this creates uncertainty about the actual incidence. In a large prospective study in the United States, upper respiratory tract involvement occurred in 3% of the patients. Some patients have upper airway involvement without thoracic disease, and this presentation may cause delays in diagnosis. These patients have nonspecific symptoms which range from minimal nasal stuffiness to life-threatening upper airway obstruction. Currently, there is no established standard therapy for the management of upper airway sarcoidosis. These patients often respond poorly to nasal and/or inhaled corticosteroids and require long courses of oral corticosteroids. Patients with poor responses to oral corticosteroids or severe side effects may respond to tumor necrosis factor alpha inhibitors. In this review, we will discuss the clinical presentation, pathogenesis, diagnostic tests, drug treatment, surgical management options and the challenges clinicians have managing these patients.

Outcomes of Primary Palatoplasty: An Analysis Using the Pediatric Health Information System Database.

BACKGROUND: Previous attempts at reporting oronasal fistula development and secondary speech surgery following cleft palate surgery have been limited to single-center case series. This limitation can be overcome by querying large databases created by health care governing bodies or health care alliances. The authors examined the effect of cleft type and demographic variables on the clinical outcomes. METHODS: Data from the Pediatric Health Information System database were queried for patients, aged 6 to 18 months, who had undergone primary palatoplasty between 2004 and 2009. Subsequent repair of an oronasal fistula and/or secondary speech surgery between 2004 and 2015 was identified by procedure codes. Logistic regression models were used to assess the associations between cleft type with oronasal fistula and with secondary speech surgery. RESULTS: Seven thousand three hundred twenty-five patients were identified, and 6.4 percent (n = 468) had a subsequent repair of an oronasal fistula and 18.5 percent (n = 1355) had a secondary speech operation. Adjusted for age, sex, and race, patients with cleft lip and palate have increased odds of oronasal fistula (OR, 5.60; 95 percent CI, 4.44 to 7.07) and secondary speech surgery (OR, 2.32; 95 percent CI, 2.05 to 2.63). CONCLUSIONS: Using a large, multi-institution billing database, the authors were able to estimate the prevalence of oronasal fistula and surgically treated velopharyngeal insufficiency following primary palatoplasty in the United States. In addition, the authors demonstrated that patients with isolated cleft palate develop fewer oronasal fistulas and require less secondary speech surgery than patients with cleft lip and palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.