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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: 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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Picibanil (OK-432) is a sclerosing agent derived from a low-virulence strain of Streptococcus pyogenes that induces regression of macrocystic lymphangiomas. This report describes a prospective, nonrandomized trial to evaluate the efficacy of Picibanil in the treatment of 13 affected children ranging in age from 1 to 94 months. On average, 4.1 fluoroscopically guided intracystic injections were performed per child, with an average total dose of 0.56 mg of Picibanil. As judged by physical examination and radiographic studies, 5 children (42%) showed a complete or substantial response, and 2 children (16%) showed an intermediate response. No response was seen in 5 children (42%), 2 of whom had massive craniofacial lymphangioma. Factors that contribute to failure with Picibanil sclerotherapy are the presence of a significant microcystic component to the lesion, massive craniofacial involvement, and previous surgical resection. Macrocystic lymphangiomas of the infratemporal fossa or cervical area have the best response to therapy.

Importance Infantile hemangiomas (IHs) vary substantially in localization and extent of tissue involvement, but IH biological progression is remarkably unique and predictable. Propranolol is an effective treatment for symptomatic IH, but its mechanism of action remains unknown and understudied. Objective To compare excreted proteins in infants with IH being treated with propranolol vs prednisolone. Design, Setting, and Participants Exploratory urine proteomics profiling of patients with IH from July 2010 to September 2012 at a tertiary pediatric hospital. Participants were infants with IH treated at our institution who were participating in a blinded, randomized trial comparing prednisolone vs propranolol. They ranged in age from 14 days to 15 months at enrollment. Exclusion criteria included a history of diabetes mellitus, asthma, and/or cardiovascular disease including hypertension or hypotension. Urine samples were longitudinally collected from all participants. Specimens were desalted, concentrated, and gel fractionated, and the protein content was identified using liquid chromatography tandem mass spectrometry. Western blot analyses and enzyme-linked immunosorbent assays (ELISAs) were performed to validate mass spectrometry findings. Intervention Treatment with propranolol or prednisolone administered starting before the age of 6 months. Main Outcomes and Measures Proteins present in urine samples and change in urinary levels of proteins over time. Results Samples were obtained from 3 patients treated with prednisolone, 3 patients treated with propranolol, and 5 untreated controls with IH. More than 1000 urinary proteins were identified by proteomics. Patients treated with propranolol demonstrated attenuation of excreted matrix metalloproteinase 9 (MMP-9) in urine over the proliferative phase of the condition compared with prednisolone-treated patients. These findings were validated with Western blot analysis and quantified with ELISA, which confirmed mean urinary MMP-9 levels in the first year of life to be significantly lower in propranolol-treated patients with IH compared with prednisolone-treated patients with IH (0.118 vs 0.501 ng/mL; P = .03) or with nontreated patients with IH (0.118 vs 3.69 ng/mL; P = .02). Conclusions and Relevance Propranolol treatment decreases urinary excretion of MMP-9 in patients with IH. Matrix metalloproteinase 9 may be a biomarker for IH propranolol responsiveness, and its signaling pathways may represent the molecular target of this drug.

Objective: To assess the efficacy of OK-432 sclerotherapy in the treatment of lymphangiomas. Design: Nonrandomized trial; follow-up, 4 to 29 months. Setting: Academic tertiary referral medical center. Patients: Six children with presumed lymphangiomas; age range at initial treatment, 1 month to 7 years 10 months. Intervention: Fluoroscopically guided cyst aspiration, cystography, and injection of OK-432. Outcome Measures: Clinical and radiographic comparisons before and after therapy. Results: Complete response in 2 macrocystic lymphangiomas; no response in 3 microcystic lymphangiomas; and no response in 1 venous malformation. Conclusion: OK-432 sclerotherapy may be effective treatment for macrocystic lymphangiomas. Arch Otolaryngol Head Neck Surg. 1996;122:1195-1199

Laryngomalacia is a well-recognized cause of airway obstruction and inspiratory stridor in infants. As children grow and become more active, laryngomalacia may manifest in different, unexpected ways. Otherwise healthy athletes may generate enough inspiratory force to draw the aryepiglottic folds into the endolarynx, causing a subtotal glottic obstruction. This problem may be overlooked or attributed to asthma, lack of fitness, or functional abnormalities. The purpose of this report is to review the prevalence, diagnosis, and treatment of exercise-induced laryngomalacia (EIL) in children and young adults. To study the incidence and diagnosis of this disorder, we examined 10 healthy volunteers. Fiberoptic laryngoscopy was used to videotape each subject's larynx during active exercise on a stationary bicycle. All volunteers demonstrated altered laryngeal dynamics with exercise, and 1 of the 10 volunteers developed laryngomalacia. Anatomically, it appears that the aryepiglottic fold serves as the critical point of obstruction. When symptomatic, laryngomalacia may be treated with supraglottoplasty. We have had experience with 2 EIL patients in the last 12 months who have undergone carbon dioxide laser microlaryngoscopy with supraglottoplasty. Both patients benefited significantly from surgery. We conclude that EIL is underdiagnosed but responds well to treatment.

Respiratory manifestations of gastroesophageal reflux disease (GERD) are being recognized with increasing frequency. We present the evaluation and management of four infants and children with unusual respiratory symptoms attributed to GERD. The advantages and disadvantages of diagnostic studies of GERD are discussed, and an evaluation and treatment protocol is presented. Treatment must be tailored to the nature and severity of the patient's presenting symptoms and includes conservative, pharmacologic, and/or surgical management.

Importance Involuted infantile facial hemangiomas (IHs) may adversely affect the social skills of children. Objective To assess the social impact of involuted facial IHs, with or without prior treatment, in preteen children. Design, Setting, and Participants An observational, cross-sectional study of social anxiety and skills in preteen children with facial IHs diagnosed during infancy. The study took place in an academic institution and a community dermatology practice between January 1, 2013, and July 30, 2014. Records on 236 children with IHs located in a cosmetically sensitive area were identified; of those, 144 potential participants (parents) were reached by telephone and mailed study packets. Thirty completed questionnaires were returned. Data analysis was performed from August 1, 2014, to September 7, 2015. Interventions The questionnaires included the following psychiatric scales: (1) Social Anxiety Scale for Children–Revised (SASC-R), completed by parents and children, including the domains of Fear of Negative Evaluation and Social Avoidance/Distress in New Situations (SAD-New) (higher scores indicate greater social anxiety), and (2) Social Competency Inventory (SCI), completed by parents, including the domains of Prosocial Behavior and Social Initiative (lower scores indicate poorer social competency). Main Outcomes and Measures Demographics, clinical details, and survey responses were collected. Analysis was conducted using t tests to compare scores for each survey domain with established normative data and between sex as well as between treatment vs nontreatment groups. Results Of the 144 potential participants, 30 (21%) responded. The mean age of the preteen subjects was 10.0 years (range, 5.4-12.9 years) with a 2:1 female to male ratio. Twenty-five children (83%) had a single IH, and the remaining 5 participants (17%) had multiple IHs, with at least 1 IH in a cosmetically sensitive area. The periocular region was the most common site of the IH (10 [33%]), followed by the nose (6 [20%]), cheek (5 [17%]), forehead (4 [13%]), lip or perioral region (4 [13%]), and ear (1 [3%]). Eighteen children (60%) had received treatment for their IH. With results reported as mean (SD), the SASC-R test showed that social anxiety of the children was not increased over normative data; however, those who did not receive IH treatment had significantly greater anxiety for new situations compared with those who received treatment (SAD-New: 15.5 [5.1] vs 11.5 [3.8]; P = .02). Results of the SCI scale indicated that the Prosocial Orientation domain score for the children was similar to normative data (3.96 [0.48] vs 3.89 [0.55], P = .50). Social Initiative domain scores were significantly poorer in children who did not receive treatment vs those who received treatment (3.45 [0.43] vs 4.03 [0.55]; P = .006). Conclusions and Relevance Preteen children with involuted, untreated facial IHs have higher Social Anxiety domain scores in new situations and decreased Social Initiative domain scores compared with children who receive treatment for facial IH. Although this study is limited by a small sample size, it raises important considerations for whether early treatment of facial IHs in cosmetically sensitive areas has a beneficial effect on social skills in preteens.

We measured interarytenoid notch height (IANH) in 20 young children who required direct laryngoscopy under general anesthesia. By means of a specifically designed instrument, we found that the average IANH approximated 3 mm. Laryngeal incompetence on swallowing or in the presence of gastroesophageal reflux is associated with a low ianh (p < .0001). Therefore, ianh may be a measure of relative laryngeal incompetence.

To confirm and extend reported successful treatment of posterior glottic stenosis in pediatric patients using endoscopic laser division of the posterior cricoid plate with augmentation using costal cartilage.A retrospective chart review and case series.Medical records were examined to determine the surgical indications, outcomes, and postoperative complications of this procedure.Twelve patients underwent the procedure, six females and six males, with an average age of 7 years (range, 2-26 years). There were 8/12 (67%) patients successfully decannulated after being tracheostomy dependent. There were no consistent anatomic abnormalities or surgical findings predictive of failure to decannulate. Average hospital stay was 3.6 days (range, 2-9 days). There were no deaths or other major complications; one patient had extrusion.Endoscopic posterior cricoid grafting is a valuable surgical option for patients with posterior glottic stenosis. The procedure is associated with low morbidity and permits decannulation in the majority of patients.

Objectives: To determine the efficacy and safety of the immunostimulant OK-432 (Picibanil) as a treatment option in the management of children with cervicofacial lymphatic malformations. Study Design: A prospective, randomized, multi-institutional phase II clinical trial at 27 U.S. academic medical centers. Methods: 182 patients with lymphatic malformations (LM) were enrolled between January 1998 and November 2004. Of the 151 patients with complete case report forms, 117 patients were randomized into immediate or delayed treatment groups; 34 patients were nonrandomized and assigned to the open-label group. Treatment consisted of a four-dose intralesional injection series of OK-432 at eight-week intervals. Patients randomized into the delayed treatment group served as observational controls for spontaneous regression. Response to therapy was measured radiographically by quantitating change in lesion size and graded as complete (90%–100%), substantial (60%–89%), intermediate (20%–59%), or none (

Objectives:We review the diagnostic workup, associated disorders, surgical technique, and postoperative course of patients who underwent repair of H-type tracheoesophageal fistulas.Methods:We performed a retrospective chart review of patients who received a diagnosis of tracheoesophageal fistula at the University of Iowa.Results:Seven patients with an H-type tracheoesophageal fistula and a single patient with a missed proximal fistula associated with esophageal atresia were identified. Their symptoms included coughing with feeding, recurrent pneumonia, and episodic cyanosis. A delay in diagnosis was seen in 4 patients and ranged from 2.5 months to 5.9 years. In all patients, the diagnosis was made with an esophagogram. The level of the fistulas was between C5 and T3, and all were successfully repaired via a right cervical approach.Conclusions:A high index of suspicion for an H-type tracheoesophageal fistula should be maintained in the presence of neonatal respiratory symptoms, as the condition can be associated with a delay in diagnosis. Repeat esophagograms and bronchoscopy may be required for diagnosis. In the postoperative period, airway obstruction is a potential risk; however, long-term difficulty with swallowing, respiration, and phonation was not observed.

Facial swelling is a common clinical problem in pediatric patients. The causes of swelling are diverse, and knowledge of the typical clinical and imaging manifestations and the most common sites of occurrence of these conditions is needed to formulate a differential diagnosis. The general clinical manifestations may be classified into the following four groups: (a) acute swelling with inflammation, (b) nonprogressive swelling, (c) slowly progressive swelling, and (d) rapidly progressive swelling. Conditions that may account for acute swelling accompanied by inflammation include lymphadenitis, sinusitis, odontogenic infection, and abscess. Contrast-enhanced computed tomography is the modality of choice for detection of abscesses requiring surgical drainage. Nonprogressive midfacial swelling is suggestive of a congenital anomaly (eg, a cephalocele, nasal glioma, or nasal dermoid or epidermoid cyst). Slowly progressive swelling may indicate the presence of a neurofibroma, hemangioma, lymphangioma, vascular malformation, or pseudocyst, or of fibrous dysplasia. The differential diagnosis for rapidly progressive facial swelling in association with cranial nerve deficits should include rhabdomyosarcoma, Langerhans cell histiocytosis, Ewing sarcoma, osteogenic sarcoma, and metastatic neuroblastoma.

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.A retrospective chart review was performed on all patientsor =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.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; p0.0001), 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).A subset of patients with SPOAs can be managed medically. These patients tend to be younger and present with minimal restriction of ocular motility.

Identification of central neurotransmitters that mediate laryngeal adductor and/or tensor activity may prove useful in managing pathological laryngeal adduction as occurs in laryngospasm or apparent life-threatening events. The putative transmitter substance P (SP) is found in the nucleus tractus solitarius (NTS), in which laryngeal afferents terminate. Therefore, we studied the laryngeal, cardiovascular, and respiratory effects of SP injected into the NTS of rats. We completed bilateral stereotactic injections of 20 nL of SP (15 μmol) or control solution into the region of the NTS, the dorsal motor nucleus (DMN), or the nucleus gracilis (GR) in 30 anesthetized rats. Changes in diaphragm, cricothyroid (CT), and thyroarytenoid (TA) electromyography (EMG), as well as blood pressure (BP), were compared. The injection sites were verified histologically. Injection of SP into the NTS altered CT and/or TA EMG activity in all animals. The change ranged from complete inhibition, to a phasic increase, to a tonic increase. No change in laryngeal adductor EMG activity was seen in 8 of 9 animals after SP injections into the DMN (4/5) or GR (4/4), but 1 animal demonstrated brief inhibition of CT and TA EMG activity after SP injection into the DMN. Injection of SP into the NTS induced central apnea and a significant decrease in BP in all animals. The duration of apnea tended to be longer after NTS injections than after DMN or GR injections (p < .10 and p < .05, respectively). We conclude that stereotactic injections of putative neurotransmitters in rats may be accomplished to identify effects on laryngeal motor activity. Direct application of SP into the NTS consistently elicits a change in CT and/or TA EMG activity, ranging from inhibition to excitation. This model may prove useful in evaluating pharmacological targets of central reflex activity to manage life-threatening laryngeal reflex activity.

Objective To describe and to determine the robustness of our study evaluating the efficacy of OK-432 (Picibanil) as a therapeutic modality for lymphangiomas. Design and Setting Prospective, randomized trial and parallel-case series at 13 US tertiary care referral centers. Subjects Thirty patients diagnosed as having lymphangioma. Ages in 25 ranged from 6 months to 18 years. Twenty-nine had lesions located in the head-and-neck area. Intervention Every patient received a 4-dose injection series of OK-432 scheduled 6 to 8 weeks apart unless a contraindication existed or a complete response was observed before completion of all injections. A control group was observed for 6 months. Outcome Measures Successful outcome of therapy was defined as a complete or a substantial (>60%) reduction in lymphangioma size as determined by calculated lesion volumes on computed tomographic or magnetic resonance imaging scans. Results Overall, 19 (86%) of the 22 patients with predominantly macrocystic lymphangiomas had a successful outcome. Conclusions OK-432 should be efficacious in the treatment of lymphangiomas. Our study design is well structured to clearly define the role of this treatment agent.

Importance While propranolol is touted as superior to prednisolone for treating infantile hemangiomas (IH), a randomized clinical trial (RCT) comparing the outcome and tolerability of these medications for symptomatic, proliferating IH has not been reported. Objectives To determine if oral propranolol is more efficacious and better tolerated than prednisolone in treating symptomatic, proliferating IH and to determine the feasibility of conducting a multi-institutional, RCT comparing efficacy and tolerability of both medications. Design, Setting, and Participants Phase 2, investigator-blinded, multi-institutional RCT conducted in 3 academic vascular anomalies clinics on 19 of 44 eligible infants aged between 2 weeks and 6 months. All participating patients had symptomatic proliferating IH treated between September 1, 2010, and August 1, 2012. Interventions Treatment with oral propranolol vs prednisolone (2.0 mg/kg/d) until halted owing to toxic effects or clinical response. Main Outcomes and Measures Primary outcome was change in IH size after 4 months of therapy. Secondary outcomes were response rate and frequency and severity of adverse events (AEs). Results The primary outcome showed no difference in lesion size or affected skin area after 4 months of therapy: 41% and 1.32 mm 2 for prednisolone vs 64% and 0.55 mm 2 for propranolol ( P = .12 for lesion size, and P = .56 for affected skin area). Longitudinal analyses showed a faster response in total lesion outer dimension with prednisolone ( P = .03), but this advantage over time was not noted when central clearing and outer dimension were included in the analysis ( P = .91). The overall frequency of AEs was similar (44 for prednisolone vs 32 for propranolol) ( P = .84), but prednisolone-treated participants had more grade 3 severe AEs (11 vs 1) ( P = .01), particularly growth retardation resulting in size and weight below the fifth percentile. Early study withdrawal owing to AEs occurred in 6 (75%) of 8 patients in the prednisolone group but 0 of 11 propranolol-treated participants. The mean duration of therapy was shorter for prednisolone (141 vs 265 days), reflecting the higher rate of early withdrawals. Conclusions and Relevance Both medications show similar efficacy for reducing the area of symptomatic, proliferating IH. Although prednisolone showed a faster response rate, propranolol was better tolerated with significantly fewer severe AEs. Propranolol should be the first line of therapy for symptomatic IH unless contraindicated or unless future studies demonstrate severe AEs from propranolol. Recruiting participants for a phase 3 RCT would be difficult owing to safety profiles measured here and emerging trends favoring propranolol. Trial Registration clinicaltrials.gov Identifier:NCT00967226

Extended pH probe testing is often performed in patients believed to have extraesophageal symptoms of gastroesophageal reflux disease (GERD), although for this indication its diagnostic value is not well established. A retrospective review of all patients who underwent pH probe testing between 1994 and 1998 was conducted to determine the outcome of antireflux therapy in the subgroup with probable extraesophageal symptoms of GERD. Sixty-eight patients underwent antireflux therapy and had adequate follow-up after pH probe testing to be included in the study. Fifty-eight patients (85%) responded to antireflux therapy (improved, 44%; cured, 41%). The positive predictive value of distal pH probe testing was greater than 90%, but the negative predictive value was less than 50%. The reproducibility of pH probe testing on different study days was poor, but pH probe testing was helpful in assessing the adequacy of antireflux therapy. The presence of gastrointestinal symptoms did not correlate with the response of extraesophageal symptoms to antireflux therapy. Thirteen patients underwent double-probe pH studies. The mean percent time the pH was less than 4 in the upper esophagus was 2.6% (range, 1% to 9.6%). Twelve of these patients were improved or cured with antireflux therapy. Distal pH probe testing is of limited benefit in predicting whether patients with extraesophageal symptoms of GERD will respond to antireflux therapy. If extraesophageal symptoms of GERD are suspected, patients should undergo an empiric trial of antireflux therapy. Distal pH probe testing should be reserved for assessing the adequacy of antireflux therapy if symptoms persist. A prospective, randomized, controlled study will aid in determining the predictive value of double-probe pH studies in pediatric patients with probable extraesophageal symptoms of GERD.

A 1-day-old female full-term newborn had mild-to-moderate episodic inspiratory stridor. She had no retractions or cyanosis, her cry and feeding were normal, and the stridorous episodes typically occurred while she was crying. Genetic and orthopedic consultations were obtained for abnormal facies and knee and hip dislocations.

Objective: To determine 1) airway outcome of infants with laryngomalacia who do not undergo routine direct laryngoscopy (DL) and bronchoscopy (B), 2) the age at resolution of laryngomalacia, and, 3) outcome of supraglottoplasty as a function of the type of laryngomalacia and the presence of concomitant disease. Study Design: Retrospective chart review. Methods: The records of all infants diagnosed with laryngomalacia by flexible fiberoptic laryngoscopy (FFL) between 1990 and 1998 in the Department of Otolaryngology-Head and Neck Surgery, University of Iowa (Iowa City, IA) were reviewed. The type of laryngomalacia was designated by a new classification scheme (types 1-3) based on the site of supraglottic obstruction and the type of supraglottoplasty indicated, should the patient later require surgical intervention. The log rank test was used to compare age at resolution and outcome between types of laryngomalacia and between infants with isolated laryngomalacia versus those with additional congenital abnormalities and/or severe neurological compromise. Results: The type of laryngomalacia was evident in 48 of the 58 charts reviewed and included type 1 (57%), type 2 (15%), type 3 (13%), or combined types (15%). Twenty percent had severe neurological compromise and/or multiple congenital anomalies. The median time to resolution of stridor in these patients was not significantly delayed when compared with infants who had isolated airway anomalies (36 and 72 wk, respectively, vs. 36 wk for isolated laryngomalacia; P

Congenital head and neck anomalies can occur in association with vertebral anomalies, particularly of the cervical vertebrae. While the former are easily recognized, especially when part of a syndrome, the latter are often occult, thereby delaying their diagnosis. The presence of vertebral anomalies must be considered in pediatric patients with head and neck abnormalities to expedite management of select cases and to prevent neurologic injury. We present our experience with 5 pediatric patients who were referred to the Department of Otolaryngology-Head and Neck Surgery at the University of Iowa with a variety of syndromic anomalies of the head and neck. Each patient was subsequently also found to have a vertebral anomaly. The relevant embryogenesis of the anomalous structures is discussed, with highlighting of potential causes such as teratogenic agents and events and germ-line mutations. A review of syndromes having both head and neck and vertebral anomalies is presented to heighten awareness of otolaryngologists evaluating children with syndromic disorders. Finally, the findings on radiographic imaging studies, particularly computed tomography, are discussed to facilitate the prompt diagnosis of vertebral anomalies.

Paradoxical vocal cord motion (PVCM) is characterized by the inappropriate adduction of the true vocal cords during inspiration. Multiple causes have been proposed for this group of disorders, which share the common finding of mobile vocal cords that adduct inappropriately during inspiration and cause stridor by approximation. Management of this group of disorders has been complicated by the lack of a classification scheme to include all types of PVCM. We propose that PVCM be classified according to its underlying etiology and recognize the following causes of the disorder: 1. brainstem compression; 2. cortical or upper motor neuron injury; 3. nuclear or lower motor neuron injury; 4. movement disorder; 5. gastroesophageal reflux; 6. factitious or malingering disorder; 7. somatization/conversion disorder. Case reports are presented to illustrate the characteristic features and diagnostic evaluation used in assessing patients with PVCM. Management varies depending on the cause of PVCM and entails speech therapy, pharmacologic therapy, behavioral modification, and/or surgical intervention. Recognition of the multiple causes of PVCM allows otolaryngologists to formulate well-directed diagnostic evaluation and treatment.

To assess the response of massive, life-threatening, or function-impairing hemangiomas in pediatric patients receiving daily alpha(2a)-interferon subcutaneously.The effect of 3 or more months of subcutaneous alpha(2a)-interferon (3 mU/m2) was prospectively evaluated in 10 patients with hemangiomas necessitating medical intervention. Hemangioma characteristics and extent were initially assessed by radiographic imaging in all but one patient. alpha(2a)-Interferon tolerance was monitored, and reduction in hemangioma size was recorded as marked (50%), moderate (25% to 50%), or minimal (25%).Hemangiomas were apparent at birth in 8 of 10 patients, and alpha(2a)-interferon was initiated at a median age of 4.5 months. Symptoms necessitating therapeutic intervention included congestive heart failure, airway obstruction, dysphagia, infection, failure to thrive, external auditory canal occlusion, visual axis impairment, and severe facial deformity. Four patients received treatment before referral that included systemic steroids (n = 2), intralesional steroids (n = 1), or surgical/laser excision (n = 2). alpha(2a)-Interferon therapy was well tolerated. Most patients had a temporary elevation in body temperature during the first month of therapy. One patient with anorexia required nasogastric feedings and a temporary reduction in her alpha(2a)-interferon dose. An additional patient with irritability was withdrawn from the study at his parents' request even though this symptom persisted after drug cessation. Hemangioma response to alpha(2a)-interferon was marked in six patients, moderate in two, and minimal in one whose lesion had features suggestive of a vascular malformation. Early signs of involution were usually evident within 6 weeks and often heralded by cutaneous blanching. alpha(2a)-interferon therapy was concluded in four patients after a mean duration of 20 months.Daily subcutaneous alpha(2a)-interferon is well tolerated in pediatric patients and appears effective in hastening involution of symptomatic hemangiomas. A significant response is unlikely in lesions with features suggestive of a vascular malformation.

Single-stage laryngotracheoplasty (SSLTP) provides a method of correcting mild-to-moderate laryngotracheal stenosis while avoiding the risks of prolonged laryngeal sten ting. Pediatric patients are orally intubated for 5 to 7 days postoperatively to ensure an adequate airway while edema resolves and healing begins. During this period, continuous neuromuscular blockade has been advocated in infants and young children to avoid endotracheal tube trauma to the fresh graft and potentially life-threatening accidental decannulation. Pulmonary atelectasis is the most common morbidity associated with prolonged neuromuscular blockade. Neuromuscular weakness also may follow prolonged paralysis and prolong hospitalization. This paper compares the postoperative course of 17 patients who underwent 18 SSLTP procedures by the senior author. The first 8 patients received continuous neuromuscular blockade in the early postoperative period. To reduce perceived morbidity, the last 9 patients were managed with a protocol that incorporated daily 4- to 8-hour “interruptions” of paralysis. Seven patients tolerated this protocol modification. As a group, these patients had less postoperative pulmonary atelectasis prior to extubation (p < .05) and were extubated sooner than patients receiving continuous neuromuscular blockade (p < .05) without compromising the surgical success of the procedure. Intermittent paralysis permitted for more accurate assessment of pain control and protected against accidental drug accumulation. Although self-extubation did not occur, diligent nursing care with adequate sedation and analgesia is necessary to avoid the risk of accidental extubation.

Acquired subglottic ductal cysts following prolonged intubation in preterm infants have been reported with increasing frequency during the past two decades. This paper reviews the subglottic ductal cysts diagnosed in 15 pediatric patients between 1989 and 1993 — the largest such review. Eighty percent were observed in preterm low-birth weight infants following prolonged intubation, and all but 3 of the patients had major intubation trauma of the larynx — an association not previously reported. The cysts were usually multiple and arose in the posterolateral subglottis. Most were small and did not cause significant airway obstruction, but associated intubation changes were sufficiently severe to necessitate tracheotomy in 10 patients. The cysts observed in our study did not enlarge and in 4 patients were observed to spontaneously resolve or shrink in size — trends not previously reported. Subglottic ductal cysts noted in preterm infants after prolonged intubation most commonly occur in association with laryngeal intubation trauma and are likely a component of intubation trauma. Although these cysts may resolve without therapy, careful follow-up and treatment of potentially obstructing subglottic ductal cysts is recommended.

We report a case of proven thyroid medullary carcinoma, metastatic to the temporal bone. Review of the otolaryngology literature demonstrates that this is the first such report. The patient presented with symptoms, physical examination, and imaging studies suggestive of a glomus jugulare tumour. The pre-operative diagnosis of metastatic thyroid medullary carcinoma, however, was made, based on a history of medullary carcinoma of the thyroid with previous metastases as well as an elevation in calcitonin levels. Histological examination of the surgical specimen confirmed the diagnosis.

Exercise-induced laryngomalacia (EIL) is characterized by severe dyspnea, stridor, and mild wheezing unresponsive to prophylactic treatment with β-agonists and cromolyn sodium. Symptoms develop with extreme exertion, but resolve quickly as the degree of exercise is decreased. Diagnosis requires flexible fiberoptic laryngoscopy before, during, and after exercise. If the diagnosis of EIL is confirmed by laryngoscopy during maximal exercise, laser epiglottoplasty is effective in alleviating symptoms and improving the airway. However, because symptoms develop only during maximal exertion, EIL is unlikely to produce symptoms or functional disability in persons who lead relatively sedentary lives.

We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolution temporal bone computed tomography (HRCT) revealed bilateral findings of a short, malformed cochlea lacking an interscalar septum, a short and wide internal auditory canal, and an enlarged vestibular aqueduct, features diagnostic of bilateral Mondini dysplasia. To determine the importance of this observation, we completed HRCT in five additional children between 7 months and 9 years of age who had evidence of symptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but their HRCT scans were normal to visual inspection. When inner ear dimensions of these temporal bones were compared with norms established by Pappas and coworkers, however, seven of the eight ears had short cochleas and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalovirus infection may cause anomalies or growth disturbances of the temporal bone.

Objective To report the benefits and complications of subcutaneous interferon alfa-2a therapy for hemangiomas in children. Design Prospective nonrandomized trial. Setting Tertiary care pediatric referral center. Patients Twenty-four pediatric patients diagnosed with massive or life-threatening hemangiomas. Interventions Each patient received daily subcutaneous injections of interferon alfa-2a to a target dose of 3 million U/m 2 of body surface area for a minimum of 4 months. Nineteen patients completed therapy and have received adequate follow-up. Main Outcome Measures Clinical and radiographic comparisons before, during, and after therapy. Reduction in hemangioma size was graded as complete (>90%), substantial (50%-80%), intermediate (20%-40%), or no response ( Results Mean age at institution of therapy was 9.6 months, and mean duration of treatment was 10.2 months. Most patients (70%) had not received prior therapy. Responses were as follows: complete, 8 patients (42%); substantial, 3 patients (16%); intermediate, 5 patients (26%); and no response, 3 patients (16%) (n=19). During therapy, 5 patients (26%) developed neurological abnormalities: 3 had an unsteady gait, and 2 had fine motor deficits. Only 1 of these 5 patients required premature termination from the study, and the neurological abnormalities in all 5 patients resolved after treatment was discontinued. Two of the 4 patients with neurological findings who completed therapy demonstrated complete resolution of their hemangiomas. Patients who developed neurological abnormalities began interferon alfa-2a therapy at an earlier age (4.7 months) than patients without neurological difficulties (aged 11.1 months). The mean time from initiation of therapy to the appearance of neurological complications was 4.8 months. Conclusions In pediatric patients with massive or life-threatening hemangiomas, interferon alfa-2a therapy is an effective treatment option. However, neurological evaluation before and during therapy with interferon alfa-2a should be performed owing to a significant incidence of neurological abnormalities (28%). Although all children with neurological findings demonstrated neurological recovery after discontinuation of therapy, we have changed our protocol and now more gradually increase the dosage of interferon alfa-2a up to 3 million U/m 2 per day. The effect of this modification on the development of neurological abnormalities has not yet been determined.