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The Fires, Asian, and Stratospheric Transport–Las Vegas Ozone Study (FAST-LVOS) was conducted in May and June of 2017 to study the transport of ozone (O3) to Clark County, Nevada, a marginal non-attainment area in the southwestern United States (SWUS). This 6-week (20 May–30 June 2017) field campaign used lidar, ozonesonde, aircraft, and in situ measurements in conjunction with a variety of models to characterize the distribution of O3 and related species above southern Nevada and neighboring California and to probe the influence of stratospheric intrusions and wildfires as well as local, regional, and Asian pollution on surface O3 concentrations in the Las Vegas Valley (≈ 900 m above sea level, a.s.l.). In this paper, we describe the FAST-LVOS campaign and present case studies illustrating the influence of different transport processes on background O3 in Clark County and southern Nevada. The companion paper by Zhang et al. (2020) describes the use of the AM4 and GEOS-Chem global models to simulate the measurements and estimate the impacts of transported O3 on surface air quality across the greater southwestern US and Intermountain West. The FAST-LVOS measurements found elevated O3 layers above Las Vegas on more than 75 % (35 of 45) of the sample days and show that entrainment of these layers contributed to mean 8 h average regional background O3 concentrations of 50–55 parts per billion by volume (ppbv), or about 85–95 µg m−3. These high background concentrations constitute 70 %–80 % of the current US National Ambient Air Quality Standard (NAAQS) of 70 ppbv (≈ 120 µg m−3 at 900 m a.s.l.) for the daily maximum 8 h average (MDA8) and will make attainment of the more stringent standards of 60 or 65 ppbv currently being considered extremely difficult in the interior SWUS.

BACKGROUND Bleeding is an important complication in children following tonsillectomy. Screening with coagulation tests prior to procedure is common to assess bleeding risk in the perioperative period, although ASH/ASPHO Choosing Wisely guidelines recommend against routine PT/PTT testing. Our aim was to compare von Willebrand factor antigen (VWF:Ag) and activity levels among patients with postoperative bleeding following tonsillectomy to evaluate for potential risk for bleeding. PROCEDURE Eligible subjects were aged 0-18 without significant personal or family history of major bleeding. Postoperative bleeding diaries were collected and symptoms measured using a postoperative bleeding score. Plasma VWF levels were drawn at time of anesthesia administration. RESULTS Postoperative bleeding occurred in 248 cases out of 1399 total subjects. Median VWF:Ag was 86 in patients with postoperative bleeding scores of 1-2, 86 for scores 3-4, 84 for scores 5-6, and 83 for scores >6, with no significant difference among groups (p = .98). Additionally, no difference was observed for subjects with multiple days of postoperative bleeding as compared to those with only 1 day of postoperative bleeding. Finally, no difference in VWF:Ag was observed for subjects whose first reported bleed occurred early in the postoperative course compared to those whose first reported bleed occurred later. VWF:Ag does not correlate with severity of bleeding, time of onset of first bleeding event, or recurrence of bleeding in healthy children with no personal or family history of bleeding who have postoperative bleeding following tonsillectomy. CONCLUSIONS This data does not support routine von Willebrand disease screening prior to tonsillectomy.

Congenital anomalies of the head and neck manifest from errors in its complex embryology developing into mature structures. These abnormalities may herald at birth or become evident later in life, even to the tenth decade. The results of aberrant development may be difficult to diagnose, but knowledge of the relevant embryological pathways is key to identification and successful treatment. This chapter reviews the anomalies of the embryonic neck tissues: the branchial apparatus, skin and adnexal tissues, mesoderm, and skeleton.

von Willebrand disease is a common bleeding disorder, but diagnosis can be difficult in young children who have not had bleeding challenges. We sought to evaluate the correlation between bleeding and von Willebrand factor (VWF) levels in children undergoing surgical challenge with tonsillectomy. Children ages 0 to 18 undergoing tonsillectomy without a personal or family history of bleeding were enrolled prospectively following informed consent and institutional review board approval. VWF levels were obtained at the time of surgery. VWF antigen (VWF:Ag) and VWF activity (VWF:GPIbM) were tested via enzyme-linked immunosorbent assay. Bleeding score was calculated using the International Society of Hematology bleeding assessment tool (BAT). Surgical and postoperative bleeding were determined using questionnaires filled out by the surgeon and patient/family. A total of 1399 subjects were enrolled with evaluable data, with a median age of 5 years. The median VWF:Ag was 85 IU/dL and the median VWF:GPIbM was 100 U/dL. Median BAT for the entire population was 0, including those with postoperative bleeding. There was no difference in VWF level between those who experienced postoperative bleeding and those who did not, with median VWF:Ag 85 vs 85 (P = .89) and mean VWF:GPIbM 98 vs 100 (P = .5). Interestingly, there was a difference in VWF levels with age, with median VWF:Ag 81 for those younger than 3 years, 82 for those 3 to 6 years, 90 for those 7 to 10 years, and 100 for those 11 to 18 years. A similar trend was noted for VWF:GPIbM. Of the 2 to 6 year olds, 5% had VWF:Ag 2. These data suggest that low VWF levels do not correlate with bleeding in children undergoing tonsillectomy. In addition, VWF levels outside the adult normal range in young children may be more common than previously thought and do not necessarily predict surgical bleeding.

Midday summertime flight data collected in the atmospheric boundary layer (ABL) of California's San Joaquin Valley (SJV) are used to investigate the scalar budgets of NOx, O3, and CH4 in order to quantify the individual processes that control near surface concentrations yet are difficult to constrain from surface measurements alone: most importantly, horizontal advection and entrainment mixing from above. The setting is a large mountain-valley system with a small aspect ratio where topography and persistent temperature inversions impose strong restraints on ABL ventilation. In conjunction with the observed time rate of change this airborne budgeting technique enables us to deduce net photochemical ozone production rates and emission fluxes of NOx and CH4. Measured NOx emissions from our principal flight domain were 216 (±33) metric tons/day averaged over six flights in July and August, which is nearly double the California government's NOx inventory for the surrounding three county region. We consider several possibilities for this discrepancy including the influence of wildfires, the temporal bias of the airborne sampling, instrumental interferences, and the recent hypothesis presented by Almaraz et al. (2018) of localized high soil NO emissions from intensive agricultural application of nitrogen fertilizers in the region and find the latter to be the most likely explanation. The methane emission average was 438 Gigagrams/year (±143), which exceeds an emissions inventory for the region by almost a factor of two as well. Measured ABL ozone during the six afternoon flights averaged 74 ppb (𝜎 = 9.8 ppb). The average mid-afternoon ozone rise of 2.8 ppb/h was found to be comprised of −0.8 ppb/h due to horizontal advection of lower O3 levels upwind, −2.2 ppb/h from dry deposition loss, −0.5 ppb/h from dilution by entrainment mixing, and 6.7 ppb/h net in-situ photochemical production. The O3 production rates exhibited a dependence on NO2 concentrations (r2 = 0.35), and no discernible dependence on methane concentrations (r2 ~ 0.02) which are correlated with many of the dominant VOC's in the region, suggesting that the ozone chemistry was predominantly NOx-limited on these flight days. Additionally, in order to determine the heterogeneity of the different scalars autocorrelation lengths were calculated for potential temperature (18 km), water vapor (18 km), ozone (30 km), methane (27 km), and NOx (28 km). The spatially diffuse pattern of CH4 and NOx seem to imply a preponderance of broad areal sources rather than localized emissions from cities and/or highway traffic within the SJV.

Objective The goal of this study is to describe a unique finding of ulcerative lesions of the larynx in two pediatric patients presenting with prolonged acute laryngotracheitis and compare to previously described reports to determine the typical clinical picture, need for intervention, and management model. Methods We present two cases of ulcerative lesions of the larynx in immunocompetent children, one with PCR positive HSV, which presented as severe croup requiring intensive care unit admission. Literature review was completed to assess for current knowledge of this entity. Our cases are discussed in the context of previously reported cases of HSV laryngotracheitis. Descriptive analysis was completed focusing on presentation, physical exam findings, treatment, length of therapy, and outcomes. Results Literature review uncovered six case reports including 10 individual cases of prolonged croup with findings of HSV laryngitis and one retrospective review describing 15 cases of prolonged croup found to be caused by ulcerative laryngitis. All patients underwent direct laryngoscopy and bronchoscopy for evaluation. Analysis was completed comparing the studies to our patients with significant findings including high intubation rate of 77%, ulcerative stomatitis in 63%, and treatment with antiviral medication directed at HSV in 85% with improvement in symptoms. Conclusion It is important to consider HSV as a possible pathogen in cases of prolonged or atypical croup. Laryngoscopy should be used for diagnostic intervention and identification of ulcerative lesions. Stomatitis may be an indication for earlier direct inspection. Treatment with anti-viral therapy and with discontinuation or taper of steroid is suggested.

Measurements from a field-deployed dual frequency comb spectrometer are coupled with gas transport models in a Bayesian inversion to quantify small, variable trace gas emissions from distances over 1 km.

In situ flight data from two distinct campaigns during winter and summer seasons in the San Joaquin Valley (SJV) of California are used to calculate boundary layer entrainment rates, ozone photochemical production rates, and regional methane emissions. Flights near Fresno, California in January and February 2013 were conducted in concert with the NASA DISCOVER–AQ project. The second campaign (ArvinO3), consisting of eleven days of flights spanning June through September 2013 and in June 2014 focused on the southern end of the SJV between Bakersfield and the small town of Arvin, California, a region notorious for frequent violations of ozone air quality standards. Entrainment velocities, the parameterized rates at which free tropospheric air is incorporated into the atmospheric boundary layer (ABL), are estimated from a detailed budget of the inversion base height. During the winter campaign near Fresno, we find an average midday entrainment velocity of 1.5 cm s−1, and a maximum of 2.4 cm s−1. The entrainment velocities derived during the summer months near Bakersfield averaged 3 cm s−1 (ranging from 0.9–6.5 cm s−1), consistent with stronger surface heating in the summer months. Using published data on boundary layer heights we find that entrainment rates across the Central Valley of California have a bimodal annual distribution peaking in spring and fall when the lower tropospheric stability (LTS) is changing most rapidly. Applying the entrainment velocities to a simple mixed–layer model of three other scalars (O3, CH4, and H2O), we solve for ozone photochemical production rates and find wintertime ozone production (2.8 ± 0.7 ppb h−1) to be about one-third as large as in the summer months (8.2 ± 3.1 ppb h−1). Moreover, the summertime ozone production rates observed above Bakersfield/Arvin exhibit an inverse relationship to a proxy for the VOC : NOx ratio (aircraft [CH4] divided by surface [NO2]), consistent with a NOx–limited photochemical environment. A similar budget closure approach is used to derive the regional emissions of methane, yielding 100 Gg yr−1 for the winter near Fresno and 170 Gg yr−1 in the summer around Bakersfield. These estimates are 3.6 and 2.4 times larger, respectively, than current state inventories suggest. Finally, by performing a boundary layer budget for water vapour, surface evapotranspiration rates appear to be consistently ~ 55 % of the reference values reported by the California Irrigation Management Information System (CIMIS) for nearby weather stations.

Puppa G, Senore C, Sheahan K, Vieth M, Lugli A, Zlobec I, Pecori S, Wang L M, Langner C, Mitomi H, Nakamura T, Watanabe M, Ueno H, Chasle J, Conley S A, Herlin P, Lauwers G Y & Risio M (2012) Histopathology Diagnostic reproducibility of tumour budding in colorectal cancer: a multicentre, multinational study using virtual microscopy Aims: Despite the established prognostic relevance of tumour budding in colorectal cancer, the reproducibility of the methods reported for its assessment has not yet been determined, limiting its use and reporting in routine pathology practice. Methods and results: A morphometric system within telepathology was devised to evaluate the reproducibility of the various methods published for the assessment of tumour budding in colorectal cancer. Five methods were selected to evaluate the diagnostic reproducibility among 10 investigators, using haematoxylin and eosin (H&E) and AE1-3 cytokeratin-immunostained, whole-slide digital scans from 50 pT1-pT4 colorectal cancers. The overall interobserver agreement was fair for all methods, and increased to moderate for pT1 cancers. The intraobserver agreement was also fair for all methods and moderate for pT1 cancers. Agreement was dependent on the participants' experience with tumour budding reporting and performance time. Cytokeratin immunohistochemistry detected a higher percentage of tumour budding-positive cases with all methods compared to H&E-stained slides, but did not influence agreement levels. Conclusions: An overall fair level of diagnostic agreement for tumour budding in colorectal cancer was demonstrated, which was significantly higher in early cancer and among experienced gastrointestinal pathologists. Cytokeratin immunostaining facilitated detection of budding cancer cells, but did not result in improved interobserver agreement.

Objectives/Hypothesis: Children with neurologically impaired (NI) swallow can undergo tonsillectomy safely and effectively. Study Design: Retrospective review. Methods: Forty-five children with documented dysphagia were compared to age- and procedure-matched normal children for operating room and clinical experience. Three further studies were limited to the NI children only: preoperative and postoperative video swallow studies (VSS), and polysomnography (PSG) for sleep-disordered breathing history. Long-term telephone follow-up of clinical outcomes was performed. Results: There were no differences between the two groups measured by tonsil size or intraoperative or late post-tonsillectomy hemorrhage, but lowest measured oxygen levels were statistically different. No intraoperative complications, early post-tonsillectomy hemorrhage, hospital readmission, or mortality occurred in either group. Three NI children each had an episode of aspiration pneumonia (early or late) without sequelae. Of the 32 VSS available for review, postoperative aspiration incidence was significantly improved, but with new-onset aspiration occurring in five children. Of available matched pre- and postoperative PSG, 91% confirmed resolution of identified preoperative obstructive sleep apnea. Long-term telephone follow-up of 20 NI children revealed improved breathing (95%), communication (90%), and feeding efficiency (55%). Conclusions: This study suggests tonsillectomy in NI children can be performed safely with appropriate monitoring and precautions with a 48-hour hospital postoperative stay recommended. Swallowing safety appears to improve both objectively and subjectively in most NI children following tonsillectomy. Both preoperative and postoperative VSS are recommended for any NI child undergoing tonsillectomy. Long-term follow-up identified improved quality of life measures for the majority of the NI children queried.

Objective To evaluate incidence of complications and hospital readmission as a result of ultrasound-guided botulinum toxin injections to manage sialorrhea. Study design Case series with chart review. Setting Children's Hospital of Wisconsin. Subjects and methods A case series with chart review was performed of all cases of ultrasound-guided injection of botulinum toxin by pediatric otolaryngologists from March 5, 2010, to September 26, 2014,. Primary outcomes included complications such as dysphagia, aspiration pneumonia, and motor paralysis. Secondary outcomes included hospitalization, intubation, and nasogastric tube placement. Results There were 48 patients, 111 interventions, and 306 intraglandular injections identified. Botulinum toxin type A and type B were utilized in 4 and 107 operative interventions, respectively. Type A was injected into 4 parotid and 4 submandibular glands, utilizing doses of 20 U per parotid and 30 U per submandibular gland. Type B was injected into 98 parotid and 200 submandibular glands, with average dosing of 923 U per parotid and 1170 U per submandibular gland, respectively. There were 2 instances of subjectively worsening of baseline dysphagia that self-resolved. No cases were complicated by aspiration pneumonia or motor paralysis. No patients required hospital readmission, intubation, or nasogastric tube placement. Conclusion Prior published data indicated 16% complication incidence with ultrasound-guided injection of botulinum toxin. Our study found a low complication rate (0.6%) with ultrasound-guided injections of botulinum toxin to manage sialorrhea, without cases of aspiration pneumonia or motor paralysis. Of 306 intraglandular injections, there were 2 cases of worsening baseline subjective dysphagia that self-resolved.

The frequency of tonsillectomy in the pediatric population and the significant amount of postoperative morbidity that accompanies surgical removal of the tonsils continues to engender significant debate regarding the optimal surgical technique. In addition, these factors also drive surgeons and manufacturers to devise new techniques and methodologies in limiting postoperative morbidity. This review discusses some of the most common techniques and innovations in tonsillectomy surgery and reviews current literature pertaining to these techniques.

BACKGROUND. Recurrent respiratory papillomatosis in children is an uncommon but potentially life-threatening benign tumor of the respiratory tract with laryngeal predilection. The diagnosis of recurrent respiratory papillomatosis may be challenging unless there is a high index of suspicion and awareness of the variable presentations. METHODS. We reviewed the medical charts of children with recurrent respiratory papillomatosis treated at a tertiary children’s hospital. The presentation of recurrent respiratory papillomatosis is illustrated by a series of case reports. We provide a paradigm to assist in the early diagnosis of children with recurrent respiratory papillomatosis. RESULTS. Five patients, aged 2 to 6 years, were erroneously diagnosed with recurrent croup, asthma, laryngeal hemangioma, and tracheomalacia after presenting with variable degrees of chronic dyspnea, cough, stridor, dysphonia, weak cry, and syncope. Once the diagnosis of recurrent respiratory papillomatosis was made, recurring surgical ablation of papillomata was initiated. CONCLUSIONS. Any child presenting with a voice disturbance with or without stridor is recommended to have diagnostic flexible fiber-optic laryngoscopy. Recurrent respiratory papillomatosis should be considered in children when other common pediatric airway diseases either do not follow the natural history or do not respond to treatment of the common disorder.

Summary Objective To evaluate the rate of control of epistaxis in patients with bilateral complaints using silver nitrate and to assess short-term complications from this therapy. Study design and setting Retrospective chart review with prospective incident case identification in a tertiary referral pediatric otolaryngology practice. Subjects and interventions Forty-six consecutive patients presenting to a single pediatric otolaryngologist with complaints of bilateral epistaxis received bilateral silver nitrate cauterization to the anterior septum and were seen in follow-up 2 months later to assess for control of epistaxis and complications from this therapy. Results Ninety-three percent (43/46) of patients followed-up at 2 months after therapy. About 93% (40/43) had total or near-total resolution of their epistaxis. Five percent (2/43) of patients returned to the clinic after initial successful control for a second cauterization during the period of study. No significant complications of infection, perforation, long-term crusting, tattooing or mucocutaneous or allergic reactions were identified. Conclusions Bilateral epistaxis can be treated at a single visit with bilateral silver nitrate therapy in children. This treatment is highly effective, well-tolerated, and appears to have a low rate of complications.

Objective Investigations that seek to generalize findings or to understand uncommon diseases must be conducted at multiple centers. This study describes the process of obtaining regulatory approval for a minimal risk genetic study in a multi-center setting as undertaken by the Recurrent Respiratory Papillomatosis (RRP) Task Force. Study design and setting Sequential cohort of American children’s hospitals. A single protocol was submitted to each Institutional Review Board (IRB). Results Documentation was prepared for 14 IRBs over 2.5 years. The median time between enlistment and approval at the first 8 sites was 15 months. Institutions varied considerably in their requirements and in the issues that were raised. Protocols were submitted sequentially and accumulated experience was used in the preparation of applications to subsequent IRBs. Nevertheless, there was no correlation between the accumulated experience and the number of issues that were raised. Conclusion Despite uniform federal standards, all local IRBs required unique and individualized submissions. For multicenter studies, investigators should seriously consider the establishment of cooperative authorization agreements. On a simpler level, a standardized format for applications needs to be adopted nationwide. EBM rating: B-3b

This review continues the series of articles by the Editorial Board reflecting upon the first volume of the journal published in 1964 and celebrating the 50th anniversary of The Cleft Palate-Craniofacial Journal. This editorial examines the contributions of Otolaryngology-Head and Neck Surgery to interdisciplinary cleft care. The aerodigestive tract has many functions that are impacted by the cleft anomaly. Progress in the development of selected therapies will be reviewed.

Background Posttransplantation lymphoproliferative disease (PTLD) is associated with Epstein-Barr virus (EBV) infection after solid organ and bone marrow transplantation. Methods We did a retrospective analysis of cases with a diagnosis of PTLD at Children's Hospital of Wisconsin. Results Ten patients were identified. Seven of 10 cases (70%) were associated with bone marrow transplantation and 3 with solid organ transplantation. Three patients (30%) died of PTLD. The average time to development of PTLD after transplantation was 120 days. Conclusions Otolaryngologic symptoms and findings are often the first manifestations of PTLD. Associated findings in this series included tonsillar necrosis, tonsillitis, airway obstruction, lymphadenitis, sinusitis, and otitis media. Diagnosis generally requires pathologic evaluation of tonsillar or adenoid tissue. Surgical intervention may also be important for relief of airway obstruction when present. Prompt recognition, diagnosis, and intervention with reduction in immunosuppression and antiviral therapy are essential to reduce the mortality of PTLD.

The prevalence of chronic otitis media with effusion (COME) was investigated in a 4-year retrospective study of a pediatric tracheotomy population followed in the outpatient Tracheotomy-Ventilation Clinic of Children's Hospital of Wisconsin. After exclusions, 83 patients comprised the study population. In a given patient, COME was defined by the presence of middle ear effusion in more than 50% of ear evaluations during the 4-year study period, or tympanostomy tube placement. The prevalence of COME was 60% in the study population as a whole. Special population groups had prevalence as follows: 90% in craniofacial anomaly patients, 79% in chronically ventilated patients, and 48% in nonventilated patients. Chronically ventilated patients had a statistically significant higher prevalence of COME than the nonventilated group (P < 0.025). These data indicate that COME is a prevalent condition in the pediatric tracheotomy population.

Although there is an established relationship between cleft lip and overt cleft palate, the relationship between isolated cleft lip and submucous cleft palate has not been investigated. To test the hypothesis that patients with isolated cleft lip have a greater association with submucous cleft palate, a double-armed prospective trial was designed. A study group of 25 consecutive children presenting with an isolated cleft lip, with or without extension through the alveolus but not involving the secondary palate, was compared with a control group of 25 children with no known facial clefts. Eligible patients were examined for the presence of physical criteria associated with classic submucous cleft palate, namely, (1) bifid uvula, (2) absence of the posterior nasal spine, and (3) zona pellucida. Nasoendoscopy was subsequently performed just after induction of general anesthesia, and the findings were correlated with digital palpation of the palatal muscles. Patients who did not satisfy all three physical criteria and in whom nasoendoscopy was distinctly abnormal relative to the control group were classified as having occult submucous cleft palate. Classic submucous cleft palate was found in three study group patients (12 percent), all of whom had flattening or a midline depression of the posterior palate and musculus uvulae on nasoendoscopy and palpable diastasis of the palatal muscles under general anesthesia. An additional six study group patients (24 percent) had similar nasoendoscopic criteria and palpable diastasis of the palatal muscles; they were classified as having occult submucous cleft palate. No submucous cleft palate was identified in the control group. Seventeen patients in the study group had an alveolar cleft with a 53 percent (9 of 17) prevalence of submucous cleft palate. In the present study, classic submucous cleft palate in association with isolated cleft lip was 150 to 600 times the reported prevalence in the general population. All children with an isolated cleft lip should undergo peroral examination and speech/resonance assessment no later than the age of 3 years. Any child with an isolated cleft lip with velopharyngeal inadequacy or before an adenoidectomy should be assessed by flexible nasal endoscopy to avoid missing an occult submucous cleft palate.

Purpose: To evaluate the relationship between facial cephalometric measures and response to uvulopalatopharyngoplasty (UPPP). Patients and Methods: Retrospective analysis of skeletal cephalometric measures obtained from a consecutive sample of 43 patients with obstructive sleep apnea syndrome (OSAS) who underwent body mass index (BMI) measures, UPPP, upright lateral cephalometric radiographs, and preoperative and postoperative polysomnography. Significant clinical effect by uvulopalatopharyngoplasty was arbitrarily defined as having a 50% reduction in the respiratory disturbance index (RDI). The cephalometric measurements used were based solely on skeletal landmarks. Results: No skeletal measurement predicted response to UPPP for the entire study population. When the patients were classified on the basis of retrognathia, 33 were identified without retrognathia. In that group, posterior airway length was the greatest predictor of response to UPPP (P < .05; odds ratio, 83.2). The distance between hyoid and mandible and the maxillary-mandibular relationship were also predictive of response (P < .05). Conclusion: The skeletal anatomy supporting the airway directly impacts the response to UPPP. Prediction of response requires stratification by skeletal subtype.

Purpose: To review current literature with respect to the diagnosis and assessment of velopharyngeal inadequacy (VPI), including present knowledge about the most common causes of VPI. Methods: Data sources include published reports over the past 20 years derived from computerized databases and bibliographies of pertinent articles and books. Indexing terms used were “velopharyngeal incompetence,” “velopharyngeal inadequacy,” “velopharyngeal insufficiency.” Conclusion: VPI is most commonly associated with cleft palate, submucous cleft palate, and following adenoidectomy. The otolaryngologist can prevent the latter by preoperative identification of physical stigmata associated with VPI. Perceptual assessment is the criterion standard for diagnosis of VPI. Multiview videofluorography and flexible nasal endoscopy provide the best direct assessments to help plan and direct the optimal treatment of VPI.

Unilateral nasopharyngeal paralysis is a relatively rare cause of velopharyngeal incompetence. Few therapeutic modalities for this problem have been described. A modification of the orticochea sphincter pharyngoplasty was developed using only a unilateral flap for treatment of the symptoms of hypernasality and nasal reflux. This preliminary report summarizes the operative approach and the successful outcomes in 2 patients.

Objective: 1) Report a unique case of a complete maxillary sinus septation (MSS) obstructing the ostio-meatal complex with resulting chronic unilateral maxillary and ethmoid sinusitis. 2) Report a novel treatment of a MSS with dual approach via Caldwell-Luc and endscopic maxillary antrostomy. 3) Perform a literature review of MSS.Method: This is an individual case report of a pediatric patient treated in 2010 for chronic right maxillary and ethmoid sinusitis resulting from an obstructive complete MSS. Initial treatment was medical, followed by a unique combined surgical approach. The patient was followed clinically and with computed tomography (CT).Results: The patient did not have recurrence of sinusitis either clinically or on imaging during the follow-up period.Conclusion: This is a unique case of MSS causing chronic sinusitis. There is scant mention of MSS in otolaryngology literature, with most mentions coming from oral and maxillofacial surgery literature. This may present an area for future investi...

Children with neurologically impaired (NI) swallow can undergo tonsillectomy safely and effectively.Retrospective review.Forty-five children with documented dysphagia were compared to age- and procedure-matched normal children for operating room and clinical experience. Three further studies were limited to the NI children only: preoperative and postoperative video swallow studies (VSS), and polysomnography (PSG) for sleep-disordered breathing history. Long-term telephone follow-up of clinical outcomes was performed.There were no differences between the two groups measured by tonsil size or intraoperative or late post-tonsillectomy hemorrhage, but lowest measured oxygen levels were statistically different. No intraoperative complications, early post-tonsillectomy hemorrhage, hospital readmission, or mortality occurred in either group. Three NI children each had an episode of aspiration pneumonia (early or late) without sequelae. Of the 32 VSS available for review, postoperative aspiration incidence was significantly improved, but with new-onset aspiration occurring in five children. Of available matched pre- and postoperative PSG, 91% confirmed resolution of identified preoperative obstructive sleep apnea. Long-term telephone follow-up of 20 NI children revealed improved breathing (95%), communication (90%), and feeding efficiency (55%).This study suggests tonsillectomy in NI children can be performed safely with appropriate monitoring and precautions with a 48-hour hospital postoperative stay recommended. Swallowing safety appears to improve both objectively and subjectively in most NI children following tonsillectomy. Both preoperative and postoperative VSS are recommended for any NI child undergoing tonsillectomy. Long-term follow-up identified improved quality of life measures for the majority of the NI children queried.

Objective: To determine the frequency of serious bacterial infection in well appearing infants aged 0-8 weeks with isolated otitis media (OM). Methods: Infants with confirmed OM underwent tympanocentesis with middle ear fluid (MEF) culture and complete sepsis evaluation. Enrolled infants were admitted to the hospital for parenteral antibiotics until blood, urine, and CSF cultures were negative for 48 hours. Results: Forty non-toxic appearing infants were enrolled between January 1994 and April 1995, of whom 15 (38%) had a documented rectal temperature ≥38°C. Bacterial pathogens were isolated from MEF cultures in 25 (62.5%) infants. All afebrile infants had negative blood, urine, and cerebrospinal fluid cultures (upper limit (UL) 95% CI 0.11). Only two febrile infants had positive cultures from sites other than the MEF (UL 95% CI 0.36). Conclusion: In our study population, previously healthy, non-toxic appearing afebrile infants aged 2-8 weeks and having isolated OM infrequently have an associated serious bacterial infection, suggesting that outpatient treatment with oral antibiotics and close follow-up may be an option. Further studies with large numbers of infants are necessary to confirm this conclusion.

Background RRP 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 Findings Papilloma 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 Abstract The 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.

Objectives To investigate the efficacy and quality of life impact of intra-oral surgery for sialorrhea and to provide long-term outcome measures. Methods A retrospective review and telephone survey of patients seen in a multi-disciplinary saliva control clinic who underwent surgery between 1999 and 2003. Results Sixteen of 31 patients underwent 4-duct ligation, 12 patients underwent submandibular gland duct ligation, and three patients underwent a 3-duct ligation procedure. The mean presurgical drooling score (maximum 10) was 9.5, 8.8, and 9, respectively. Recurrence was seen in 68% of patients at a mean of four months. Additional surgery was required in five patients. Phone survey was accomplished in 10 patients. Two of 10 caregivers reported sustained improvement of sialorrhea at two and three years. Conclusions Intra-oral surgery provides minimal long-term control of sialorrhea. Additional medical and surgical therapy was needed in a significant portion of patients in this long-term review.

Summary Objective Epidermodysplasia verruciformis is a skin disease characterized by abnormal susceptibility to human papilloma viruses. Recently four mutations in the Epidermodysplasia verruciformis 1 gene ( EVER1 , also known as TMC6 ) have been associated with the disease. Because of the phenotypic similarity between Epidermodysplasia verruciformis and recurrent respiratory papillomatosis, we decided to investigate whether any of these mutations accounts for the susceptibility to human papilloma viruses in subjects with recurrent respiratory papillomatosis (RRP). Methods Allele-specific PCR and restriction fragment length polymorphisms (RFLPs) were employed for genotyping a cohort of 101 patients with recurrent respiratory papillomatosis. Results None of these four mutations were found in the studied subjects. Conclusion The absence of these mutations in RRP patients might indicate that EVER 1 alleles are not associated with susceptibility to RRP, or that other, as yet unidentified, mutations in the Epidermodysplasia verruciformis 1 gene, might account for the susceptibility to RRP.

External auditory canal polyps are most commonly inflammatory in nature but may also manifest more severe disease. Prolonged conservative therapy may delay the correct diagnosis and appropriate intervention. A case is presented of a child with chronic otorrhea treated for 4 months with topical drops and antibiotics. On referral, a large external auditory canal polyp was confirmed to represent a foreign body granuloma covering a large electrical cap, with erosion approaching the facial nerve. External auditory canal polyps that fail to respond promptly to conservative medical therapy warrant a computed tomography scan and surgical exploration with biopsy.

The youngest reported patient with stress velopharyngeal incompetence is presented. The patient's symptoms responded to rest with a possible contribution from palatal exercise. Surgical correction would appear to best be reserved for the professional musician with this unusual condition.

Objective: To describe the long-term outcome of a large anterior lingual thyroglossal duct cyst, diagnosed antenatally by ultrasound that completely obstructed the oral cavity and had almost completely replaced the anterior two-thirds lingual musculature. Methods: Longitudinal observation. Results: Anticipated airway obstruction at birth did not occur. Complete cyst removal occurred at day 2 of life with a rim of lingual muscle observed present only on the left side. Complete and safe oral feeding was achieved by day 11 of life. Progressive muscle mass development and function with mild asymmetry was observed during sequential assessments. Vigorous speech therapy was started at age 7 months achieved 80–100% intelligible speech with minimal misarticulations at age 33 months. Conclusion: This case demonstrates the remarkable compensatory ability of the tongue to achieve almost normal function with minimal anterior musculature that is critical to deglutition and articulation. Early speech therapy appears key to improving functional outcomes of speech. A strong central nervous system basis for suckle development is suggested by the rapid development of a safe and effective suckle and swallow soon after cyst resection and in spite of in utero tongue fixation.

Objective: To determine the clinical course of velopharyngeal incompetence in children with myasthenia gravis (MG). Methods: A 30-year retrospective study was performed using the medical records of 538 children who presented with velopharyngeal inadequacy (VPI) to a tertiary care academic pediatric center. Children with velopharyngeal incompetence due to myasthenia gravis were identified and their clinical courses were reviewed. Results: Four children were identified with velopharyngeal incompetence associated with myasthenia gravis. All four children required intervention for improvement of speech intelligibility. A speech prosthesis was the uniform intervention. Conclusion: Neonatal myasthenia gravis patients should be followed long-term as symptoms may recur as speech impairment. In addition, a high index of suspicion for this entity is required for early diagnosis due to the highly variable presentation and clinical course.

Plastic bronchitis is a rare disorder characterized by the formation of branching mucoid bronchial casts. Several pathophysiologic conditions are associated with development of these intrabronchial casts, including congenital heart defects. The management of plastic bronchitis presents an unusual and interesting bronchoscopic challenge. We describe a patient who underwent a Fontan procedure for correction of a congenital heart defect and subsequently developed respiratory distress secondary to plastic bronchitis on two occasions. In both cases, endoscopic intervention was required to remove these casts. A review of the literature, including the proposed etiologies, diagnosis, and current medical and surgical management, is also undertaken.

Background/Purpose: The most important aspects in management of pyriform sinus malformations are awareness of the diagnosis, familiarity with the clinical manifestations, and complete surgical excision of the entire tract. Pyriform sinus anomalies are the least common branchial apparatus malformations and present anatomically as sinus tracts with or without cystic dilatation. The clinical presentations can include lateral neck mass, thyroid abscess, suppurative thyroiditis, retropharyngeal abscess, neonatal airway obstruction, and even carcinoma. Recurrent symptoms after surgery suggest incomplete identification and excision of the tract. Methods: Cadaveric dissections were performed to show both the proposed embryologic course and clinical manifestations of third and fourth branchial apparatus pyriform sinus anomalies. Results: Illustrations and digital camera images of the cadaveric models are presented to explain the course of pyriform sinus fistula tracts. Conclusions: The authors discuss 3 case presentations of pyriform sinus anomalies with emphasis on their proposed embryologic origin and anatomic basis for surgical management. Surgical excision is the mainstay of therapy. Understanding the embryologic basis for pyriform sinus malformations aids in recognition of the diagnosis despite the myriad of clinical presentations. Laryngoscopy with sinus cannulation facilitates removal of the entire sinus tract with preservation of the recurrent and superior laryngeal nerves.

Objective: Timely and experienced intervention for esophageal foreign bodies generally allows for removal with minimal morbidity. However, esophageal foreign bodies present a risk for esophageal perforation and subsequent mediastinitis, especially if the diagnosis of the foreign body is delayed. Although much has been written about the management of esophageal foreign bodies and their complications, little has been mentioned in recent literature about the specific complication of mediastinitis. This review was performed to examine our experience with this uncommon complication of esophageal foreign bodies. Methods: A retrospective review of the esophageal foreign body database at Children's Hospital of Wisconsin from 1987 to 1997 was performed to identify patients with esophageal foreign bodies and subsequent mediastinitis. Results: Four patients with esophageal perforation with associated mediastinitis secondary to retained esophageal foreign bodies were identified. Three of the four patients were treated with conservative measures consisting of foreign body removal, intravenous antibiotics and discontinuing of oral nutrition. These patients all achieved resolution of their mediastinitis and esophageal perforation with subsequent return to normal diets and no significant morbidity. One patient, with vascular erosion, required aggressive, invasive therapy. Conclusion: From review of this limited number of patients, in the absence of major vascular erosion, conservative methods of treating children with foreign body esophageal perforation and subsequent mediastinitis appears to be effective.

Objective: There is considerable variation in opinion regarding the optimal management of patients with Pierre Robin sequence (PRS). No single method of airway intervention or feeding strategy is universally appropriate and effective. This study was performed to examine methods used for airway and feeding management and to identify specific problems encountered. Study Design: A retrospective study of 252 patient charts between 1989 and 1997 at Children's Hospital of Wisconsin. Methods: Patient information was collected regarding perinatal history, genetics evaluation, and airway and feeding evaluations and intervention. A group of 47 patients was determined as having Pits. Results: Secondary respiratory difficulties, defined as respiratory abnormalities in addition to the expected Pits obstruction, were identified in 23% of patients. Also, intrinsic feeding abnormalities not associated with airway obstruction were identified in 11% of patients. Analysis by Fisher's Exact Test revealed patients with a syndromic diagnosis to have a significantly higher rate for tracheotomies and gastrostomy tube placement (P = .041, and P = .0004, respectively). Syndromic patients were also found to have significantly lower Apgar scores and longer hospital stays. Positioning techniques, tongue-lip adbesion, and tracheotomy were also employed effectively with specific indications and specific difficulties that need to be considered. Conclusion : Patients with PRS require thorough airway and feeding evaluation. Those with additional syndromic diagnoses demonstrate higher rates of more invasive interventions. Patients with Pits must undergo individualized approaches with consideration of multiple factors for successful management.

Our objective was to evaluate the relationship between posterior facial cephalometric measures and obstructive sleep apnea syndrome (OSAS). We used a consecutive sample of 60 patients with OSAS who underwent upright lateral cephalograms, uvulopalatopharyngoplasty (UPPP), and preoperative and postoperative polysomnography. Successful responders to UPPP were arbitrarily defined as having a respiratory disturbance index reduced to fewer than 20 events per hour. Standard cephalometric measurements were used. Posterior facial height measures were constructed, based on a plane perpendicular to the Frankfort horizontal placed at hyoidale. The total and lower airway lengths were shorter and posterior mandibular height was longer in UPPP responders compared to nonresponders (p ≤ .05). There was no difference between the two groups by standard cephalometric measurements. Responders and nonresponders to UPPP have significant differences in posterior airway measures that are not reflected in standard cephalometric measures. Airway length likely is a critical factor in OSAS and surgical response.

A retrospective review was performed on 39 patients with deep space neck infection admitted to the Children's Hospital of Wisconsin over a nine year period. The signs and symptoms as well as issues of diagnosis and treatment were reviewed with attention to the use of computed tomography (CT) scans. Beta hemolytic streptococcus was the most commonly cultured organism followed by alpha hemolytic streptococcus. Sixteen patients underwent CT scans. Twelve were diagnosed with an abscess by CT scan. An abscess was confirmed in ten patients. Necrotic lymph nodes mimicked abscesses on CT scans and resulted in negative surgical findings. Airway obstruction occurred in 30% of the children in the study. Children under the age of 36 months had a significantly higher rate of airway obstruction upon presentation compared to older children (P < 0.05). There should be a high index of suspicion of upper airway compromise in children under the age of 36 months with a deep neck infection.