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To explore patient-reported outcome measures of pediatric paradoxical vocal fold motion through a multi-institutional study of geographically diverse United States medical facilities to assess long-term management and outcomes.Eligible participants8 years of age diagnosed with PVFM over a 10-year period from 7 tertiary pediatric hospitals were invited to complete a survey addressing study objectives.65 participants completed the survey, of whom 80% were female, 75% reported a 3.5 grade point average or better, and 75% identified as competitive athletes or extremely athletic individuals. Participants rated their perceived efficacy of 13 specific treatments. Only five treatments were considered effective by a majority of the participants who tried them. The treatments that participants tried most often were breathing exercises (89.2%), bronchodilator treatments (45%), and allergy medications (35.4%). 78.8% of participants reported receiving more than one treatment and 25% reported receiving a combination of bronchodilators, anticholinergics, and steroids. At the time of PVFM diagnosis, 38% of participants had no idea when their symptoms would completely resolve. 23.3% of participants did not experience symptom resolution until greater than 1 year after diagnosis.Traditional management tools such as breathing exercises and biofeedback treatments may not provide the long-term benefit that providers anticipate. In addition to these commonly used management strategies, highly efficacious techniques such as counseling and lifestyle management should be incorporated into the long-term management of patients whose symptoms are refractory to traditional care.4 Laryngoscope, 2022.

Objective To assess the association between commonly obtained endoscopic and serologic data and bronchoalveolar lavage pepsin assay (BAL) results in children with chronic cough. Study design We performed a retrospective chart review of seventy-two children with a BAL pepsin obtained through our Aerodigestive Center over an 18-month period. BAL outcomes include evidence of viral, bacterial, or fungal infection, presence of lipid laden macrophages, and cytology (eosinophils, neutrophils, and lymphocytes). Gastrointestinal outcomes include esophagogastroduodenoscopy (EGD) and pH impedance probe findings. Other characteristics include serum eosinophil, neutrophils, and lymphocytes; spirometry; FeNO; and IgE. Results Seventy-two patients underwent BAL pepsin testing. Median age was 4.9 years, 30.6% had severe persistent asthma, and 59.2% were on reflux medication. There was an association between positive BAL pepsin assay and positive viral panel (p=0.002) or fungal culture (p=0.027). No significant association found between positive BAL bacterial culture; BAL cytology; presence of BAL lipid laden macrophages; IgE; spirometry; FeNO; CBC neutrophil, eosinophil, or lymphocytes; pH impedance testing parameters; or EGD pathology. Conclusions BAL pepsin is associated with a positive BAL viral PCR or fungal culture. Lack of correlation between pepsin-positivity and pH-impedance parameters or EGD pathology suggests microaspiration may be due to an acute event (such as a respiratory infection) rather than chronic GERD. This may be especially true in the presence of a positive viral panel or fungal culture when a BAL pepsin is obtained. This article is protected by copyright. All rights reserved.

Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal.Blinded modified Delphi consensus process.Tertiary care center.A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items.The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus.It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated.5. Laryngoscope, 131:1168-1174, 2021.

Introduction:Tracheal agenesis (TA) is rare and usually fatal. Few survivors with concomitant tracheoesophageal fistulae (TEF) who underwent ligation of the distal esophagus with creation of a spit-fistula and neo-trachea from the proximal esophagus exist. We report a novel surgical technique whereby the esophagus is divided longitudinally to preserve a functional alimentation tract and a parallel neo-trachea. We review the literature of reported cases, including survivors beyond 12 months.Methods:Case report and literature review.Results:A female infant with prenatal polyhydramnios was born at 35 weeks gestation with immediate respiratory distress and absent cry. Oxygenation was maintained with a laryngeal mask airway. Despite a normal appearing larynx, she could not be intubated and emergent neck exploration disclosed no cervical trachea. The patient was placed on extra corporeal membranous oxygenation (ECMO), and later diagnosed with TA, Floyd Type I. Parental desire for reconstruction but refusal of a spit-fistula necessitated a novel procedure. The esophagus was divided longitudinally via a microstapler to preserve the original alimentary tract and create a parallel neo-trachea originating from the TEF and terminating as a cervical stoma. The healing process was complicated but the baby was ultimately discharged to home where she developed normally neurologically until succumbing one night to accidental decannulation at 16 months of age.Conclusion:We describe a novel surgical approach to manage TA. This includes avoiding creation of a spit fistula and preserving the native esophagus. We then survey the literature, reporting the survivorship duration and operative management of 174 reported cases of TA.

Objectives: To highlight the importance of recognizing the postcricoid cushion as a surgically treatable cause of newborn aspiration in select cases unresponsive to conservative measures. Methods: A retrospective review was performed on a single case of neonatal aspiration at a tertiary care pediatric hospital. Results: Resolution of aspiration was achieved in an otherwise healthy six-week old term infant following ablation of a postcricoid cushion using microlaryngeal instrumentation and coblation. Prior airway and swallowing evaluations, along with a trial of reflux therapy and pacing of feeds, preceded the surgical excision, successfully avoiding gastrostomy tube placement. Conclusions: Otolaryngology consults for neonatal dysphagia and aspiration are challenging. The postcricoid cushion is considered an anatomic variant that can engorge with crying. When large, it can prevent feeds from entering the esophagus and lead to pooling and aspiration. The postcricoid cushion is evident on flexible fiberoptic laryngoscopy, but without a vigilant eye may be easily overlooked. This single case report suggests that surgical ablation may be effective management in select cases after ruling out concomitant aerodigestive pathology and neurodevelopmental causes of aspiration and only after conservative therapy has failed. A video demonstrating the surgical ablation is included.

Objectives:To explore long-term patient reported outcome (PRO) measures of pediatric paradoxical vocal cord motion (PVCM) including ease of diagnosis, management, symptom duration and effect on quality of life.Methods:All children >8 years of age diagnosed with PVCM at a tertiary pediatric hospital between 2006 and 2017 were invited to complete a survey addressing study objectives.Results:21/47 eligible participants could be contacted and 18/21 (86%) participated. 78% were female with a mean age at diagnosis of 11.6 and 15.0 years at survey completion. Common PVCM symptoms reported were dyspnea (89%), globus sensation (56%), and stridor (50%). The median time to diagnosis was 3 months (IQR 2-5 months). Nearly all reported being misdiagnosed with another condition, usually asthma, until being correctly diagnosed usually by an otolaryngologist. Participants reported undergoing 3.7 diagnostic studies (range 0-8); pulmonary function testing was most common. Of numerous treatments acknowledged, breathing exercises were common (89%) but only reported helpful by 56%. Use of biofeedback was recalled in 1/3 of subjects but reported helpful in only 14% of them. Anti-reflux, allergy, anticholinergics, inhalers and steroids were each used in >50%, but rarely reported effective. PVCM was reportedly a significant stressor when initially diagnosed but despite 2/3 of participants still reporting ongoing PVCM symptoms, the perceived stress significantly decreased over time (Z = 3.26, P = 0.001).Conclusions:This first PVCM PRO study endorses that diagnosis is often delayed and prescribed treatments often viewed as ineffective. While biofeedback and breathing exercises may be critical for short-term control of PVCM episodes, lifestyle changes and stress reduction are likely necessary for long-term management. Increased awareness and improvements in management are needed for this condition.

Objectives: Chronic recalcitrant cough is present in 2/3 of pediatric patients evaluated in our tertiary-care multidisciplinary aerodigestive clinic (ADC). This study aimed to determine the impact of chronic cough and efficacy of ADC treatment using the validated Pediatric-Cough Quality-of-Life-27 tool (PC-QOL-27). Methods: The PC-QOL-27 survey was administered to ADC patients with chronic cough at initial clinic visit and 6 to 12 weeks after cough management. Pre and post survey scores, demographic data, treatment and evaluation season were collected over 16 months. Results: Twenty parents completed pre and post PC-QOL-27 surveys (mean 12.1 weeks later). Patient median age was 6.04 years (IQR: 2.2-10.44 years). A total of 65% were males and 65% were African American. Management was tailored based on clinical assessment and diagnostic studies, including direct laryngoscopy/bronchoscopy (4), pulmonary function tests (PFT’s 9), esophagogastroduodenoscopy (9), and flexible bronchoscopy/lavage (9). Following ADC management, changes in physical, social and psychological domain scores of the PC-QOL-27 each met the threshold for minimal clinical important difference (MCID) indicating a clinically meaningful improvement. Improvements were most notable in the physical domain where post survey scores significantly improved from pre-survey scores ( P = .009) regardless of age, gender, ethnicity, history of endoscopy and season. Conclusions: The physical impact of chronic cough in pediatric patients who failed prior management by a single specialist was lessened by an ADC team approach to management.

Objectives/hypothesis Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal. Study design Blinded modified Delphi consensus process. Setting Tertiary care center. Methods A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items. Results The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus. Conclusions It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated. Level of evidence 5. Laryngoscope, 131:1168-1174, 2021.

Objectives: We report obstructing laryngeal manifestations of Crohn’s disease in a toddler with very early onset-IBD (VEO-IBD) who required tracheotomy tube placement at 27 months of age for relief of recalcitrant airway obstruction unresponsive to maximal medical therapy. We review the literature for the frequency of extra-intestinal laryngeal manifestations of Crohn’s disease in adults and children. Methods: Case report and literature review of laryngeal manifestations of Crohn’s disease. Results: Laryngeal involvement of Crohn’s disease is very rare with only 14 other cases reported. Most cases appear in adults, with the supraglottis most commonly affected. This case marks the youngest report and only the second report of a patient requiring a tracheotomy for supraglottic obstruction when intensive medical management, including use of steroids and biologics, failed to relieve obstructing laryngeal inflammation. Despite ongoing Crohn’s disease, laryngeal manifestations improved permitting decannulation the following year. Conclusions: Laryngeal manifestations of Crohn’s disease are rare and usually affect adults. Most cases are managed with medical therapy, however surgical excision of obstructing lesions or tracheotomy placement is sometimes required for temporary relief of airway obstruction.

OBJECTIVE. To determine the feasibility of detecting and differentiating middle ear effusions (MEEs) using an optical coherence tomography (OCT) otoscope. STUDY DESIGN. Cross-sectional study. SETTING. US tertiary care children’s hospital. SUBJECTS AND METHODS. Seventy pediatric patients undergoing tympanostomy tube placement were preoperatively imaged using an OCT otoscope. A blinded reader quiz was conducted using 24 readers from 4 groups of tiered medical expertise. The primary outcome assessed was reader ability to detect presence/absence of MEE. A secondary outcome assessed was reader ability to differentiate serous vs nonserous MEE. RESULTS. OCT image data sets were analyzed from 45 of 70 total subjects. Blinded reader analysis of an OCT data subset for detection of MEE resulted in 90.6% accuracy, 90.9% sensitivity, 90.2% specificity, and intra/interreader agreement of 92.9% and 87.1%, respectively. Differentiating MEE type, reader identification of nonserous MEE had 70.8% accuracy, 53.6% sensitivity, 80.1% specificity, and intra/interreader agreement of 82.9% and 75.1%, respectively. Multivariate analysis revealed that age was the strongest predictor of OCT quality. The mean age of subjects with quality OCT was 5.01 years (n = 45), compared to 2.54 years (n = 25) in the remaining subjects imaged (P = .0028). The ability to capture quality images improved over time, from 50% to 69.4% over the study period. CONCLUSION. OCT otoscopy shows promise for facilitating accurate MEE detection. The imageability with the prototype device was affected by age, with older children being easier to image, similar to current ear diagnostic technologies.

OBJECTIVES Develop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies. METHODS Standard Delphi methods were applied. Participants proposed items in three categories: 1) patient/disease characteristics, 2) procedural/intraoperative/perioperative factors, and 3) outcome measures and complications. Both general and anatomic site-specific measures were elicited. Participants also suggested specific operations to be encompassed by this project. We then used iterative ranking and review to develop consensus lists via a priori Delphi consensus criteria. RESULTS Thirty-three pediatric airway experts from eight countries in North and South America, Europe, and Australia participated, representing otolaryngology (including International Pediatric Otolaryngology Group members), pulmonology, general surgery, and cardiothoracic surgery. Consensus led to inclusion of 19 operations comprising open expansion, resection, and slide procedures of the larynx, trachea, and bronchi as well as three endoscopic procedures. Consensus was achieved on multiple patient/comorbidity (10), disease/stenosis (7), perioperative-/intraoperative-/procedure-related (16) factors. Consensus was reached on multiple outcome and complication measures, both general and site-specific (8 general, 13 supraglottic, 15 glottic, 17 subglottic, 8 cervical tracheal, 12 thoracic tracheal). The group was able to clarify how each outcome should be measured, with specific instruments defined where applicable. CONCLUSION This consensus statement provides a framework to communicate results consistently and reproducibly, facilitating meta-analyses, quality improvement, transfer of information, and surgeon self-assessment. It also clarifies expert opinion on which patient, disease, procedural, and outcome measures may be important to consider in any pediatric airway reconstruction patient. LEVEL OF EVIDENCE 5 Laryngoscope, 129:244-255, 2019.

Objective: To determine the utility of a pediatric multidisciplinary aerodigestive clinic (ADC) in treating recalcitrant aerodigestive conditions. Methods: Longitudinal observational study of presenting complaints, evaluation, management, and outcome of patients seen during 12 monthly ADCs beginning August 2013. Results: Fifty-five patients were seen by the ADC team (otolaryngology/gastroenterology/pulmonology/speech pathology/nurse practitioner) and followed for a mean 17.6 months (range, 12-26 months). Mean age was 4.3 years (range, 0.5-19 years). All were seen by at least 1 specialist before ADC referral but without significant improvement. Chronic cough was the most common primary symptom (44%). Clinic evaluation included flexible nasopharyngolaryngoscopy (FFL, 53%) and pulmonary function testing (36%.) FFL influenced management in 79%. An operative procedure usually combined endoscopy was warranted in 58%. Endoscopy provided high diagnostic yield, detecting laryngeal cleft (8), adenoid hypertrophy (8), vocal cord dysfunction (4), pulmonary infection (4), reflux disease (3), laryngomalacia (3), tracheomalacia (2), cilia abnormality (2), celiac disease (1), Helicobacter pylori (1), duodenal web (1), and eosinophilic esophagitis (1). Outcome was available for 48 of 55 patients, with 73% reporting resolved to markedly improved symptoms and 27% minimal to no improvement. Conclusions: The ADC team approach resulted in resolved to markedly improved symptoms in 73% of patients whose symptoms persisted despite seeing a single specialist prior to referral.

Objective: Multidisciplinary clinics offer important value to pediatric patients with complex conditions that overlap specialties; however, such labor-intensive clinics are difficult to facilitate. We performed a cost analysis of our pediatric multidisciplinary aerodigestive clinic (MADC) to assess its financial feasibility at our tertiary care institution. Method: Revenue was based on net collections for clinic, professional, and hospital setting charges generated during 12 consecutive monthly MADCs beginning August 2013. Clinic charges included facility and speech pathologist fees. Professional charges included clinic and operative fees generated by providers and anesthesiologist. Hospital setting fees included facility and material charges for technical procedures. Direct expense calculations included all providers and staff salaries, benefits, and supply costs. Results: Charge capture for 54 consecutive patients seen during the study time included new visits 99203-99205 (n = 63), consults 99243-99245 (n = 60), and follow-up visits 99212-99215 (n = 196). Sixty percent of patients underwent a clinic nasopharyngeal or laryngeal endoscopy (92511 or 31575), and 60% underwent subsequent intraoperative procedures with 1 (n = 8) or 2 to 3 services (n = 24). Program net revenue totaled $828 136 and direct costs $518 867, accounting for a net positive margin of $309 269. Conclusions: When including direct downstream revenue, our MADC operates on a net positive margin, making it financially feasible.

Introduction: Horner syndrome is described as the clinical triad of miosis, ptosis, and anhidrosis. In pediatric patients the condition may be congenital or acquired from neoplastic, infectious or traumatic conditions, including birth trauma. Most cases of pediatric Horner syndrome present first to a pediatric ophthalmologist however since the neural pathways involve the cervical sympathetic chain otolaryngologists should understand the pathophysiology to avoid delay in management of potentially malignant cases. Objectives: To aid otolaryngologists in recognizing and managing pediatric Horner syndrome by describing 3 unique cases from malignant, traumatic and/or congenital causes. Methods: Case report of 3 pediatric patients with Horner syndrome presenting to our pediatric otolaryngology department. Results: Case #1 is 5-month-old female with ptosis and a left level II 1.5 cm neck mass. Magnetic resonance imaging showed the mass displacing the common carotid artery and excisional biopsy revealed a poorly differentiated neuroblastoma. Case #2 is a 9-year-old female with anisocoria appearing after suffering a severe playground injury. Case #3 is a 3-year-old-male who developed ptosis and anisocoria following re-excision of a recurrent cervical lymphatic malformation. Conclusion: Pediatric Horner syndrome may be a benign finding that is easily overlooked but may reflect a serious underlying condition. Otolaryngologists should be aware of the pathophysiology and differential diagnosis, including malignant causes, to appropriately manage patients.

Objective: Multidisciplinary vascular anomaly clinics (VACs) offer important value to pediatric patients with complex vascular anomalies whose care overlaps specialties. These clinics are labor intensive and costly to operate since providers see fewer patients compared to their individual specialty clinic. Our North American tertiary care institution’s VAC specialists include a pediatric otolaryngologist, pediatric surgeon, pediatric plastic surgeon, pediatric dermatologist, and interventional radiologist. To assess financial feasibility, we conducted a cost analysis of our VACs comprised of 2 half-day multidisciplinary physician attended clinics (5 specialists at our main campus and 2 specialists at a satellite clinic) and a half-day nurse practitioner clinic. Method: Assessment of net revenue based on net collections for clinic, professional, operative, hospital setting, and facility charges generated during 12 consecutive monthly VACs beginning July 1, 2015. Expense calculations included provider and staff salaries, benefits, supply costs, and clinic leasing costs. Results: There were 469 clinic visits, of which 202 were new patient evaluations. Sixty-eight patients underwent 93 procedures under general anesthesia, including procedures performed by our interventional radiologist, most commonly sclerotherapy or embolization (n = 37), surgical interventions including endoscopy (n = 36), or laser procedures (n = 20). Three patients were admitted. Fifty-seven patients received a new diagnosis different from that for which they were referred. Gross revenue was $1 810 525, and net revenue was 42.5%, or $783 152. Expenses totaled $453 415 for a net positive revenue of $329 737. Conclusion: When including direct downstream revenue, particularly from operative procedures, our VAC program operates on a net positive margin, making the program financially feasible.

PURPOSE OF REVIEW: Chronic cough is the most common presenting complaint in a pediatric aerodigestive clinic. The etiology of chronic cough is varied and often includes more than one organ system. This review aims to summarize the current literature for a multidisciplinary approach when evaluating a child with chronic cough. RECENT FINDINGS: There is very little medical literature focused on a multidisciplinary approach to chronic cough. In the limited data available, multidisciplinary clinics have been shown to be more cost-efficient for the families of children with complex medical problems, and also increase the likelihood of successfully obtaining a diagnosis. SUMMARY: There is no consensus in the literature on how to work-up a child with chronic cough presenting to an aerodigestive clinic. Current studies from these clinics have shown improved outcomes related to cost-effectiveness and identifying definitive diagnoses. Future studies evaluating clinical outcomes are necessary to help delineate the utility of testing routinely performed, and to demonstrate the impact of interventions from each specialty on quality of life and specific functional outcome measures.

Develop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies.Standard Delphi methods were applied. Participants proposed items in three categories: 1) patient/disease characteristics, 2) procedural/intraoperative/perioperative factors, and 3) outcome measures and complications. Both general and anatomic site-specific measures were elicited. Participants also suggested specific operations to be encompassed by this project. We then used iterative ranking and review to develop consensus lists via a priori Delphi consensus criteria.Thirty-three pediatric airway experts from eight countries in North and South America, Europe, and Australia participated, representing otolaryngology (including International Pediatric Otolaryngology Group members), pulmonology, general surgery, and cardiothoracic surgery. Consensus led to inclusion of 19 operations comprising open expansion, resection, and slide procedures of the larynx, trachea, and bronchi as well as three endoscopic procedures. Consensus was achieved on multiple patient/comorbidity (10), disease/stenosis (7), perioperative-/intraoperative-/procedure-related (16) factors. Consensus was reached on multiple outcome and complication measures, both general and site-specific (8 general, 13 supraglottic, 15 glottic, 17 subglottic, 8 cervical tracheal, 12 thoracic tracheal). The group was able to clarify how each outcome should be measured, with specific instruments defined where applicable.This consensus statement provides a framework to communicate results consistently and reproducibly, facilitating meta-analyses, quality improvement, transfer of information, and surgeon self-assessment. It also clarifies expert opinion on which patient, disease, procedural, and outcome measures may be important to consider in any pediatric airway reconstruction patient.5 Laryngoscope, 129:244-255, 2019.

Infantile hemangioma (IH), also known as hemangioma of infancy, is the most common soft tissue tumor of childhood. IH demonstrate significant clinical heterogeneity in their presentation, ranging from small self-limiting lesions to bulky tumors that impart significant morbidity. IH can present as superficial, deep, or mixed lesions depending on the depth of soft tissue involvement (Drolet et al, N Engl J Med 341: 173–181, 1999; Haggstrom et al, Pediatrics 117:698–703, 2006). Most lesions are superficial, presenting as bright red papules or plaques which are typically round and involve the superficial dermis, while deep lesions have a more bluish hue; mixed lesions have characteristics of both. The morphological pattern of IH tends to be localized or segmental (Haggstrom et al, Pediatrics 117:698–703, 2006; Chiller et al, Arch Dermatol 138: 1567–1576, 2002). Localized lesions tend to be discrete ovoid or rounded lesions, while segmental lesions, which impart higher risk of complications, tend to involve a broad region and are more geometric in shape.

To develop general and site-specific treatment effect and outcome measures to standardize the reporting of head and neck lymphatic malformation (HNLM) treatments.Consensus statement/expert opinion.Multiple tertiary academic institutions.The modified Delphi method is an iterative process of collecting expert opinions, refining opinions through discussion and feedback, statistically aggregating opinions, and using these aggregates to generate consensus opinion in the absence of other data. The modified Delphi method was used by a multi-institutional group of otolaryngology and interventional radiology experts in the field of vascular anomalies to formulate a list of recommended reporting outcomes for the study and treatment of head and neck lymphatic malformations.Through 3 rounds of iteration, 10 expert panelists refined 98 proposed outcome measures and 9 outcome categories to a final consensus set of 50 recommended outcome measures in 3 global categories (general, demographics, and treatment complications) and 5 site-specific categories (orbit, oral cavity, pharynx, larynx, and neck).We propose the first consensus set of standardized reporting measures for clinical and treatment outcomes in studies of HNLMs. Consistent outcome measures across future studies will facilitate comparison of treatment options and allow systematic review. We hope that these guidelines facilitate the design and reporting of subsequent HNLM studies.

Vascular anomalies are divided into tumors and malformations based on their clinical and cytologic attributes. Vascular malformations are further subcategorized as low-flow lymphatic, venous, capillary, or mixed lesions and as high-flow arteriovenous malformations. Treatment is reserved for vascular anomalies that are symptomatic or cosmetically disfiguring, and surgical and nonsurgical treatment options are widely varied with variable outcomes.

Introduction: Soft tissue occlusion of the external auditory canal (EAC) can cause intense pruritis, recurrent foul smelling otorrhea, recurrent otitis externa, and conductive hearing loss. Occlusion of the EAC can be challenging to treat as the area is prone to circumferential scarring. Methods: We describe the novel use of serial bipolar radiofrequency ablation (coblation) to treat 3 children with complete EAC occlusion from congenital and acquired conditions including lymphedema (1), microcystic lymphatic malformation (1), and venolymphatic malformation (1). Results: Patients underwent a mean of 3 procedures with postoperative EAC stenting (7 days) and antibiotic and steroid aural preparations (10 days). Otologic symptoms resolved in all patients, and their EACs remained patent 14 months after last procedure (range, 4-32 months). One patient experienced a pinpoint tympanic membrane perforation that healed spontaneously 2 weeks later. Conclusions: Coblation of soft tissue stenosis of the EAC can be an effective treatment for this problematic condition.

Rapidly involuting congenital hemangioma (RICH) is an uncommon vascular tumor that, unlike infantile hemangioma, is largely developed at birth and undergoes rapid postnatal involution. To date, RICH has often been described in the setting of an isolated lesion, whereas infantile hemangioma is a well-known feature of numerous syndromes and associations, including the association of posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies. The authors report a unique case of RICH in the setting of this association.

Objectives: We sought to determine the effect of propranolol on cardiovascular and blood glucose parameters in infants with symptomatic infantile hemangiomas who were hospitalized for initiation of treatment, and to analyze adverse effects of propranolol throughout the course of inpatient and outpatient treatment. Methods: A retrospective cohort analysis was performed on 50 infants (age less than 12 months) with symptomatic infantile hemangiomas who were hospitalized for propranolol initiation between 2008 and 2012. Demographic data and disease characteristics were recorded. Systolic and diastolic blood pressures, heart rate, blood glucose values, and adverse events recorded during hospitalization were analyzed. An additional cohort of 200 consecutively treated children was also assessed for adverse events associated with outpatient propranolol use. Results: The median age among the inpatient cohort was 3.4 months (range, 0.8 to 12.0 months). Infants older than 6 months were more likely to exhibit bradycardia than were younger infants (p < 0.001). Hypotensive and/or bradycardic periods were infrequent and were not associated with observable clinical symptoms. The mean systolic and diastolic blood pressures and the mean heart rate decreased significantly from day 1 of hospitalization to day 2 (p = 0.004; p = 0.008; p < 0.001), but not from day 2 to day 3, when the propranolol dose was increased to target. Hypoglycemia was rare (0.3% incidence.) Among the 250 outpatients, 2 infants developed lethargy and hypoglycemia during a viral illness and recovered without sequelae. One infant experienced recurrent bronchospasm with viral illnesses and required concomitant bronchodilator therapy. Conclusions: Frequent deviations from normal ranges of blood pressure and heart rate occur upon initiation of propranolol, but are clinically asymptomatic. These findings support that outpatient initiation of propranolol in healthy, normotensive infants appears to be a relatively safe alternative to inpatient initiation. Hypoglycemia is rare, but can occur throughout the treatment period; parent counseling is of paramount importance.

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available. Pediatrics 2013;131:128-140

Importance The field of vascular anomalies presents diverse challenges in diagnosis and management. Although many lesions involve the head and neck, training in vascular anomalies is not universally included in otolaryngology residencies and pediatric otolaryngology (POTO) fellowships. Objective To explore the education in, exposure to, and comfort level of otolaryngology trainees with vascular anomalies. Design, Setting, and Participants A survey was distributed to 39 POTO fellows and 44 residents in postgraduate year 5 who matched into POTO fellowships from April 22 through June 16, 2014. Main Outcomes and Measures Survey responses from trainees on exposure to, education on, and comfort with vascular anomalies. Results Forty-four residents in postgraduate year 5 who applied to POTO fellowships and 39 POTO fellows were emailed the survey. Fourteen respondents were unable to be contacted owing to lack of a current email address. Thirty-six of 69 residents and fellows (18 fellows and 18 residents [52%]) responded to the survey. Twenty-seven trainees (75%) reported no participation in a vascular anomalies clinic during residency; 6 of these 27 individuals (22%) trained at institutions with a vascular anomalies clinic but did not participate in the clinic, and 28 of the 36 respondents (78%) reported that they had less than adequate or no exposure to vascular anomalies in residency. Among POTO fellows, 11 of 17 (65%) did not participate in a vascular anomalies clinic during fellowship, even though 8 of the 11 had a vascular anomalies clinic at their fellowship program. During fellowship training, 12 of 18 fellows (67%) reported that they had adequate exposure to vascular anomalies. Only 20 respondents (56%) felt comfortable distinguishing among diagnoses of vascular anomalies, and only 4 residents (22%) and 9 fellows (50%) felt comfortable treating patients with vascular anomalies. All fellows believed that training in vascular anomalies was important in fellowship, and 100% of respondents indicated that increased exposure to diagnosis and management of vascular anomalies would have been beneficial to their ability to care for patients. Conclusions and Relevance These data indicate that most otolaryngology trainees do not receive formal training in vascular anomalies in residency and that such training is valued among graduating trainees. Conversely, most POTO fellows felt their exposure was adequate and 50% of fellows felt comfortable treating vascular anomalies. However, 65% of POTO fellows had no participation in a vascular anomalies clinic, where many patients are managed by a multidisciplinary team. This finding may indicate that POTO fellows may have a false sense of confidence in managing patients with vascular anomalies and that residency and fellowship programs may consider changes in didactic and clinical programs.

Objectives We describe our use of balloon dilation to repair choanal atresia in a series of patients. Study design Case series. Methods We performed a retrospective review of patients who underwent repair of choanal atresia using dilation with high pressure, non-compliant airway balloons between January 2009 and September 2010. For primary cases, balloon dilation was used in conjunction with transnasal endoscopic puncture, and repair of bony stenosis with backbiting forceps and microdebrider drill. Results 5 patients underwent balloon dilation repair of choanal atresia or stenosis. 4 patients presented for primary repair, with a mean age of 2.9 months. 1 patient presented at 10 years of age for revision repair several years after previous attempts performed elsewhere. The average number of procedures was 3.6, with an average of 2 balloon dilations. There were no complications stemming from balloon dilation. Follow-up ranged from 3 to 24 months. All patients demonstrated choanal patency on last follow-up. Conclusions This is a novel use of high pressure, non-compliant balloons. We found success in dilating membranous stenoses, post-repair granulation, and scar tissue in revision cases. Balloon dilation is an effective adjuvant tool for use in the repair of choanal atresia and stenosis.

Purpose of review Orbital cellulitis and abscess formation in pediatric patients usually arises as a complication of acute sinusitis and if untreated may cause visual loss or life-threatening intracranial complications. This review describes the current evaluation and management of this condition. Recent findings Computed tomography with contrast remains the optimal imaging study for orbital inflammation. Orbital inflammation is still classified by Chandler's original description as preseptal or postseptal and nearly all cases of preseptal cellulitis are managed with oral antibiotics. Most cases of postseptal cellulitis are managed with intravenous antibiotics, although surgical therapy is required for some abscesses, particularly large ones. Patients under 9 years respond to medical management more frequently than older patients but recent studies confirm that even children over 9 with small or moderate-sized abscesses and normal vision deserve a medical trial before surgical intervention. Medial subperiosteal abscesses that fail medical therapy are usually drained endoscopically, whereas lateral or intraconal abscesses require an open procedure. Summary Periorbital complications of sinusitis in pediatric patients often respond to medical therapy but may require surgical intervention to prevent serious complications. Continuous in-house evaluation of patients is necessary to observe for progression of symptoms and to optimize outcome.

Objectives To present case vignettes of unusual pediatric parotid pathologies and discuss management paradigms in the context of these lesions. Study design Retrospective case series. Setting Free-standing, academic tertiary care pediatric hospital. Methods All patients over the past 18 months undergoing parotidectomy for a parotid mass were reviewed (N = 5). Results Ages ranged from 17 months to 16 years. All presented with a remarkably similar clinical course, consisting of a persistent parotid mass for more than 3 months which was usually painless. Most (4/5 patients) had been treated with antibiotics prior to Otolaryngology consultation. Fine-needle aspiration (FNA) was performed on 3 patients and was diagnostic in one. Complete excision of the mass was performed in each child through a parotidectomy approach (3 total, 2 lateral lobe). The final pathology showed metastatic neuroblastoma (17 months old), undifferentiated primitive sarcoma (22 months old), mucoepidermoid carcinoma (11 years old), nodular fasciitis (12 years old), and hyperplastic lymph node (16 years old). The patient with neuroblastoma died from complications of bone marrow transplant. Conclusions The differential diagnosis for a persistent pediatric parotid mass is expansive and differs from that found in the adult population. As this series highlights, in many cases, it is impossible to discern the pathology, or rule out malignancy, based upon the clinical course, imaging, or FNA results. Surgical excision remains the standard for management of these patients and is both diagnostic and therapeutic. Our anecdotal case series highlights the importance of having a low threshold for parotidectomy in these children.

Controversies remain regarding the management of orbital cellulitis (OC). The objective of this study was to examine the outcomes of patients admitted to our institution for orbital cellulitis during a 7-year period.Case series with chart review.Tertiary referral pediatric hospital.Charts of 465 consecutive OC admissions were reviewed for presentation, imaging, medical and surgical treatment, and outcome.Of these patients, 189 were treated in the emergency room and 276 were admitted. CT scan was performed on 240 patients. Subperiosteal abscess (SPA) was noted in 68 patients. Of these, 47 were treated medically and 21 had surgery. Surgical patients were older (8.3 vs 6.2 years, P = 0.039), had larger abscesses (10 mm, P0.001), required a longer admission (10.2 vs 6.6 days, P0.001), and had higher temperatures on admission (38.0 degrees C vs 37.3 degrees C, P = 0.03).The majority of small SPAs as diagnosed on CT scans in younger children can be successfully treated medically. Surgery, however, should be considered for a worsening clinical examination. Our findings confirm those of previous reports on this clinical entity.

Importance Extracranial pituitary ectopia is an uncommon finding characterized by all or a portion of the pituitary gland situated in an aberrant location. Often these lesions come to clinical attention only once they begin to function abnormally or increase in size significantly. Observations We describe an incidentally noted, asymptomatic pedunculated nasopharyngeal lesion in a 13-month-old girl that may be an extracranial pituitary gland. Consideration of this rare anomaly cautioned against performing a biopsy and may have prevented inadvertent removal of functional pituitary tissue. We describe the embryologic origins of ectopic pituitary tissue, discuss the differential diagnosis for nasopharyngeal lesions in children, and emphasize physical and radiologic findings suggestive of ectopic pituitary tissue to prevent potential inadvertent removal of this rare anomaly. Conclusions and Relevance We report this unusual case to review embryologic origins of ectopic pituitary tissue and to alert otolaryngologists of the need to consider ectopic pituitary tissue in the diagnosis of nasopharyngeal lesions in children.

Objectives: There is no curative therapy for recurrent respiratory papillomatosis. Unmethylated dinucleotides of cytosine and guanine (CpG) are potent immune stimulants that have shown efficacy against tumors as monotherapy, as vaccine adjuvants, and in combination with chemotherapies. We examined the therapeutic effect of CpG oligodeoxynucleotides in the treatment of papillomavirus in a cottontail rabbit model (CRPV). Methods: Twenty rabbits were infected with CRPV; 10 were treated with 11 weekly CpG inoculations while treatment control rabbits received intralesional saline solution. Eight rabbits (4 treatment, 4 control) were rechallenged with CRPV 17 weeks after the initial viral challenge and monitored for new papilloma development. Results: Papillomas developed in all 20 rabbits (100%) within 4 weeks of infection. The diagnosis was confirmed histologically. There was no difference in the average tumor burden between the treatment and control groups after 11 weeks of CpG treatments or after 9 additional weeks of observation. There was no difference between the groups in papilloma size at the site of the injections, nor was there eradication of papillomas at remote sites in either group. No new papillomas developed in any of the 8 animals that were rechallenged. Conclusions: We have reproduced an effective mammalian papilloma model for preclinical immunotherapeutic testing. Despite the potency of CpG in triggering host immunity, CpG oligodeoxynucleotide did not show a therapeutic effect against the large papilloma burdens tested in this study. The lack of effect suggests that either enhanced papilloma antigen presentation or targeting of immune-evasive mechanisms used by the papillomas is needed to treat bulky disease with an immunotherapeutic strategy.

Objectives: To examine the prevalence of hearing loss (HL) in Union Army (UA) veterans by year, birth cohort, and occupation, and to compare Civil War pension and contemporary disability programs by examining monthly dollar awards. Study Design: A retrospective review of medical records for 17,722 UA veteran pension applicants, a subset of some 35,000 soldiers retrieved randomly from the Military Archives. Methods: The diagnosis of HL was based on review of medical records, which used gross measurements because of the unavailability of audiometric testing. Results: One third (5,891 or 33%) of pensioners sampled received compensation for HL. The veterans with HL suffered predominantly from left-sided HL (4,091 or 70%), which is consistent with noise-induced HL in a right-handed individual firing a rifle. Comparison of civilian occupations reveals minimal variation in prevalence of HL. Civil War pensions for unilateral HL averaged $134.04 per year, representing nearly one third of the average annual income in 1890. Bilateral HL received nearly twice that amount. Today, military veterans receive $1,248 annually for unilateral loss and $27,288 annually for bilateral loss. Social Security disability benefits are granted only for bilateral HL, with an average 60-year-old individual receiving $11,400 per year. Conclusion: HL was a common disability among UA Civil War veterans, with noise exposure a likely etiology for the HL. The differing levels of compensation for HL may reflect differing perceptions on the incapacitating effects of HL. Key Words: History of hearing loss, public policy, disability, Civil War. Laryngoscope, 114:2147–2153, 2004

Identifying central neurotransmitters that mediate laryngeal adductor activity may aid in managing pathological laryngeal adduction as occurs in laryngospasm or apparent life-threatening events in infants. We studied the effect on cricothyroid (CT) and thyroarytenoid (TA) electromyography (EMG) and on cardiovascular parameters of neurotransmitter injections into the rat nucleus tractus solitarius (NTS), the primary sensory relay center of the larynx. Twenty nanoliters of vasoactive intestinal peptide (VIP; 2.5 pmol), neurokinin B (NKB, 16 pmol), calcitonin gene-related peptide (3.0 pmol), neurokinin A (NKA; 35 pmol), or artificial cerebrospinal fluid (control solution) was stereotactically injected into the region of the NTS and the control nucleus gracilis in 119 studies performed in 24 mature, anesthetized Sprague-Dawley rats. Changes in diaphragm, CT, and TA EMG activity and blood pressure (BP) were compared. Injection sites were verified histologically. Injections of both VIP and NKB into the region of the NTS, but not the nucleus gracilis, induced life-threatening changes, including apnea, a marked decline in BP (p < .05), and increases in EMG activity of the CT and TA adductor muscles ranging from sustained contraction to mild phasic increases during inspiration. Calcitonin gene-related peptide injection also decreased BP (p < .05), but caused only mild increases in CT and TA EMG activity. NKA and control injections into the NTS did not alter respiration, BP, or CT or TA EMG activity. VIP and NKB may play important roles in modulating EMG activity of the CT and TA muscles. This information may prove useful in evaluating pharmacological targets of central reflex activity to manage life-threatening laryngeal adduction.

Background Controversy exists about the optimal management of subperiosteal orbital abscesses (SPOAs) in pediatric patients. Some otolaryngologists advocate immediate surgical drainage while others recommend medical management initially and reserve surgery for nonresponders. We hypothesized that patients who can be managed without surgery have identifiable features on presentation that may aid in predicting their response to medical therapy. Methods A retrospective chart review was performed on all patients ≤18 years of age who were admitted to the University of Iowa between 1984 and 2001 with findings consistent with an SPOA on computed tomography imaging. Patients were divided into two groups: group I received medical treatment only while group II underwent surgical drainage of the abscess. Presenting features were compared between the two groups. Results Forty-two patients were identified with 17 group I patients and 25 group II patients. All patients had resolution of their SPOA and favorable outcomes. The following variables attained significance: group I patients were younger than group II patients (5.1 years versus 11 years; p < 0.0001), had less restriction of ocular motility (—1.0 versus —2.3), and were hospitalized for fewer days (6.5 days versus 9.6 days; p = 0.011). The following clinical variables did not vary significantly between the groups: gender, side of abscess, temperature, total white blood cell count, neutrophil count, chemosis, visual acuity, and proptosis. Culture results predominantly showed growth of anaerobic organisms (7/23). With increasing age, there was an increase in the number of organisms cultured (p = 0.005). Conclusion A subset of patients with SPOAs can be managed medically. These patients tend to be younger and present with minimal restriction of ocular motility. (American Journal of Rhinology 18, 321–327, 2004)

Objective: To determine how frequently the use of -interferon (-IFN) is associated with the development of spastic diplegia. Study Design and Methods: Meta-analysis of 600 English manuscripts published January 1991 to June 2002 reporting -IFN use in infants/children. We identified 3,113 children 18 years of age or younger and an estimated 3,055 children 12 years of age or younger who received -IFN therapy. Sixty-nine percent were treated for chronic hepatitis and 14% for vascular neoplasms. Outcome Measure: Neurologic examination to confirm spastic diplegia or a motor developmental disturbance other than spastic diplegia such as hyperactive deep tendon reflexes, gait disturbances, or impaired fine motor control. Results: Including our index case, 11 of 441 children with vascular lesions developed spastic diplegia and an additional 16 of 441 developed a motor developmental disturbance. All of these children were less than 1 year of age at initiation of therapy. Mean age of initiation and duration of -IFN therapy were not significantly different between groups (P > .05); however, motor developmental disturbances improved with cessation of therapy, whereas spastic diplegia did not. No child receiving treatment for chronic hepatitis developed neurologic complications; however, only 49 children were less than 1 year of age at initiation of therapy. Conclusion: -IFN should not be used in infants under 1 year of age unless life-threatening conditions do not respond to any other form of treatment. If -IFN must be used, children should have monthly neurologic examinations. If a motor developmental disturbance is detected and -IFN therapy can be discontinued, it should be.

Pediatric airway stenosis challenges the treating surgeon, from the initial evaluation and decision-making process to the final postoperative care and follow-up setting. As our approach to these cases evolves, we must critically evaluate our outcomes in reference to our stated goals. We describe our process in treating this population and critically examine our outcome data from 1990 to the present. Emphasis is placed on selection of procedure and postoperative management.

Surgical drainage of localized infections in deep neck spaces in children is often completed without a high degree of technical difficulty. However, abscess drainage within the superior parapharyngeal space medial to the great vessels is particularly challenging for otolaryngologists. Drawbacks to both the intraoral and external approaches to this area have led us to develop a new adjunctive technique that utilizes intraoperative image-guided technology to augment the intraoral approach. Here we present a case series of 12 children in whom this technique was successfully used.

Purpose To determine whether there is a role for surgery in Group III and IV patients with residual parameningeal rhabdomyosarcoma (PM-RMS) after radiotherapy (RT). Materials and Methods From 1965 to 2000, 29 patients with PM-RMS (Group III 27, Group IV 2) were diagnosed and treated with RT at the University of Iowa. All patients received chemotherapy consisting of vincristine, dactinomycin, and cyclophosphamide (VAC) in 17 (59%), VAC + doxorubicin in six (21%), VC + doxorubicin in three (10%), and other in three. RT was given to the primary site. Median dose was 50.4 Gy (range, 41.4–65 Gy). Two had hyperfractionated RT (59.4 Gy in 54 fractions). Median follow-up time for surviving patients was 17.9 years (range, 1.5–31.5 years). Results The 2- and 5-year overall survival rates were 78.9% and 45.7% while the 2- and 5-year freedom from local progression rates were 56.8% and 42.1%. For the 11 patients who did not achieve a complete response to chemoradiotherapy at the primary site, eight underwent surgical resection 1.5–7 months after RT. The 2- and 5-year survival rates for the eight who had a surgical salvage were 100% and 60%. None of the other three survived. Six of 18 patients (33%) relapsed at the primary site after a complete response to chemoradiation and all died. Conclusion Surgical salvage after an incomplete response to chemoradiation in PM-RMS is feasible and can be curative in some cases. Med Pediatr Oncol 2003;41:118–122. © 2003 Wiley-Liss, Inc.

Recurrent respiratory papillomatosis (RRP), caused by the human papillomavirus, is characterized by unregulated growth of wartlike neoplasms on laryngeal mucosa. Apoptosis is important in normal cellular homeostasis, and dysregulation of this process is thought to govern the behavior of certain neoplasms. This study evaluates the expression of several pro-apoptotic and anti-apoptotic factors in papillomas of patients with RRP, with a specific interest in survivin, a cell cycle-regulated anti–apoptotic factor. Three anti-apoptotic and 6 pro-apoptotic messenger RNA (mRNA) species were quantified by ribonuclease protection assay in 11 RRP papilloma specimens and 5 normal laryngeal specimens. Anti-apoptotic and pro-apoptotic mRNA ratios were quantified by normalizing to the ribosomal protein L32 and compared between specimens. Protein expression of survivin in tissue samples was also evaluated. The mean (±SD) expression of survivin was almost fivefold greater in the RRP papillomas than in normal tissue (14.2% ± 2.5% versus 3.0% ± 0.8% of L32, p = .003). The RRP specimens also had greater expression of XIAP, Fas, and p53 than did the normal tissue. Survivin protein was differentially expressed in the papilloma specimens, and was greatest in a papilloma that underwent malignant transformation. Survivin was absent in all normal laryngeal tissue tested. Apoptotic factors in general appear to be ***upregulated in papillomatous tissue as compared to normal laryngeal tissue and may suggest a higher proliferation rate and cell turnover. Survivin is abundant in papillomas and absent in normal laryngeal tissue. Dysregulation of apoptosis as determined by abnormal expression of anti-apoptotic factors like survivin and XIAP probably favors papilloma growth and survival. Such factors may represent potential targets in the treatment of this disease.

Lymphatic malformations are usually congenital in origin and often arise in the head and neck region. The size of lymphatic malformations varies markedly and treatment is tailored ascending to the degree of function and cosmetic compromise. Current common treatment options include observation, sclerosant therapy, somnoplasty, laser reduction/excision, and surgical removal. The classification, clinical presentation, diagnosis and treatment of lymphatic malformations are further discussed in this article.

Nontraumatic atlantoaxial rotary subluxation (NAARS) is a relatively uncommon entity, with inconsistent presentations. It most commonly follows infectious processes or operative procedures. We present our experience with 4 pediatric otolaryngology patients with NAARS who were treated at the University of Iowa Hospitals and Clinics during a 2-year period beginning in 1997. A review of the symptoms, physical findings, and radiographic abnormalities is presented. Treatment options, varying from muscle relaxants to surgical fusion, are discussed. A high index of suspicion in evaluating children with a stiff neck or pain on attempted motion is essential in order to facilitate prompt diagnosis and appropriate intervention.

Objectives We compare the success of different surgical options in the treatment of laryngeal webs. Methods We performed a retrospective study spanning the years 1980 to 2005. Results Eighteen patients were identified. The average age at diagnosis was 6 months (range, 1 day to 2.5 years). The presenting symptoms included weak cry, stridor, airway obstruction, and difficulty breathing. Associated cardiac defects consistent with the diagnosis of 22q-syndrome were present in 7 patients. Webs were classified as grade I (5 patients), grade II (2 patients), grade III (10 patients), or grade IV (1 patient) according to the Cohen classification. In 5 patients, only endoscopic lysis was required. The remaining 13 patients underwent open procedures; 9 patients in this group required tracheotomy. An average of 1.3 open airway procedures was necessary to achieve a decannulation rate of 89%. After operation, 34% of patients had residual webbing and 20% had a weak or aphonic voice. Conclusions Management of laryngeal webs is dependent on the severity of airway obstruction. Grade I and II webs can be treated endoscopically; more severe laryngeal webs usually require tracheotomy and open airway reconstruction.

Juvenile onset recurrent respiratory papillomatosis is the most common cause of laryngeal tumors in children. This disease is caused by infection of the human papillomavirus, a virus whose complete genetic structure is now known. New, more directed agents show promise for improved control of papillomatosis in preliminary studies. Concurrently, there is an increasing awareness of methods to reduce surgical morbidity. At present, the role of preventive efforts including elective caesarian section remains uncertain.

Laryngomalacia is the most common cause of stridor in infants and occasionally also manifests in older patients. Although a self-limited condition a small group of patients with laryngomalacia require surgical intervention for relief of their airway obstruction. A careful preoperative evaluation is necessary to determine the type of laryngomalacia present so that the appropriate type of supraglottoplasty is performed. The overall success of supraglottoplasty is excellent and prevents the necessity of tracheotomy for patients requiring surgical intervention.