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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. [ABSTRACT FROM AUTHOR]

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 cere- brospinal fluid (control solution) was stereotactically injected into the region of the NTS and the control nucleus gracilis in 1l9 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Lymphangiomas are congenital malformations of the lymphatic system. These lesions occur most often in the head and neck area, and their treatment continues to be a challenge. Fortunately, a number of advances have occurred in the diagnosis and management of lymphatic malformations in the past decade. The purpose of this article is to clarify the embryology, pathogenesis, histopathology, and classification of these lesions, as well as to describe their various forms of clinical presentation. We provide a complete review of the diagnostic measures available and thoroughly discuss new therapeutic interventions proposed to treat lymphangiomas. [ABSTRACT FROM AUTHOR]

Examines the effects of substance injection into the nucleus tractus solitarius of rats on cricothyroid and thyroarytenoid motor activity and cardiovascular and respiratory systems. Identification of central neurotransmitters; Injections of putative neurotransmitters in rats; Stimulation of the laryngeal or esophageal mucosa.

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. [ABSTRACT FROM AUTHOR]

Pediatric tongue lesions represent a rare, diverse group of pathologies related to congenital malformations, inflammatory changes, and neoplastic masses. We present the largest-to-date series of pediatric tongue lesions managed surgically. Charts of all pediatric patients with a tongue lesion surgically treated at a regional children's hospital from February 1st, 1997 to August 30th, 2019 were reviewed. After obtaining institutional review board approval, a retrospective review was completed of all pediatric patients with a tongue lesion surgically treated at a regional children's hospital from February 1st, 1997 through August 30th, 2019. Charts were evaluated for patient demographics as well as symptoms at presentation, surgical details, pathologic evaluation of the lesion, and rates of recurrence. Categorical variables were compared between various groups using Chi-square and/or Fisher's exact tests. Univariate and multivariable logistic regression analysis was performed unadjusted and adjusted to further explore these relationships. 159 patients were included in the study with ages distributed relatively even across infants (27.7%), children 1–5 years (27.7%), 6–10 years (23.3%), and greater than 10 years of age (21.4%). Patient/family reported race/ethnic background was most commonly Caucasian (34.8%) or Hispanic/Latino/Spanish Origin (34.0%) followed by Black or African American (21.3%). The most commonly reported symptoms were pain (23.3%), difficulty with oral intake (12.6%), bleeding (11.3%), and respiratory distress (11%). Patients with lesions of the base of tongue were significantly more likely to present with respiratory distress or difficulty with oral intake (p < 0.001). The likelihood of recurrence was observed to be higher with lesions managed in infancy (OR 3.85, CI 1.47–10.0, p = 0.005), those greater than 2 cm (OR 3.45, CI 1.34–8.71, p = 0.009), or with laser (OR 4.52, CI 1.25–15.00), p = 0.015). Pediatric patients with tongue lesions may present with a wide array of symptoms. Base of tongue lesions should be managed carefully as they are most likely to create barriers to the airway and oral intake. Larger lesions and those managed in infancy are at increased risk for recurrence. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives: A variety of sclerotherapy agents are used to treat macrocystic lymphatic malformations (LMs). This retrospective study at a single institution was performed to compare the outcomes of pediatric macrocystic LMs of the head and neck that were treated with doxycycline or with OK432. Methods: The outcomes measured included early response to therapy, number of treatments required, operating room time, and adverse events. Results: The rates of clinical success for OK432 and doxycycline were similar (83% and 82%, respectively; p > 0.05), although OK432-treated patients required more treatments than did doxycycline-treated patients (1.9 versus 1.0 injections; p = 0.01; 95% confidence interval, 1.57 to 0.27). The average operating room time for a single OK432 injection was significantly shorter than that for doxycycline (53.2 versus 98.1 minutes; p < 0.001); however, when the total number of treatments administered was considered, the overall times in the operating room were similar. Adverse events in the early postoperative period were more common in OK432-treated patients, who experienced marked postoperative swelling compared to doxycycline-treated patients. Conclusions: OK432 and doxycycline are both effective sclerosants for the treatment of predominantly macrocystic LMs. The administration time for OK432 is shorter than that for doxycycline, but OK432 required more treatments overall to achieve clinical success. Early adverse events were more common in OK432-treated patients, but longer follow-up is necessary to determine whether rates of recurrence and adverse events are similar, particularly in light of the risk of tooth discoloration in doxycycline-treated patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 ofthe head and neck along with congenital anomalies ofthe 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. [ABSTRACT FROM AUTHOR]

Objectives: Our primary objective was to review the current use of propranolol for treatment of infantile hemangioma (IH), specifically regarding 1) the age at initiation of therapy, 2) the method of initiation, 3) the use of other adjuvant therapy, 4) the duration of therapy and relapse rate, 5) the adverse events, and 6) the outcome. Our secondary objective was to describe a randomized, controlled, single-blinded trial comparing propranolol to prednisolone for treatment of IH. Methods: Ovid Medline and PubMed searches were completed for the MeSH keywords "propranolol" and "hemangioma." Forty-nine English-language articles were published between June 2008 and September 2010, and 28 of these reported data from a total of 213 patients. Only 6 studies treated more than 10 patients, and these were selected for review in detail (154 patients). Results: The treatment was initiated during infancy in 92.9% of patients (mean, 4.5 months). Sixty-five percent of patients were treated with 2 mg/kg per day, and 25.3% with 3 mg/kg per day. Patients were monitored overnight at initiation of treatment in 3 series (59 patients), for 4 to 6 hours as outpatients in 2 series (62 patients), and initially as inpatients but later as outpatients in 1 series (32 patients). Propranolol was used as sole therapy in about two thirds of patients (103 patients). Treatment was ongoing in 46% of patients at the time of publication. The average treatment duration in the remaining patients was 5.1 months. Rebound growth occurred in 21% of patients after a mean of 4.3 months of therapy. Adverse events occurred in 18.1% of patients and included hypotension in 6, somnolence in 6, wheezing in 4, insomnia, agitation, and/or nightmares in 6, cool hands or night sweats in 2, gastroesophageal reflux in 3, and psoriasis-like rash in 1. All authors reported a favorable outcome with propranolol, but the definition of efficacy was not standardized. Conclusions: Propranolol is an attractive alternative to other treatments for IH. Despite apparent widespread use of this medication, the data are limited, and prospective studies are lacking for this indication. The relatively high rate of adverse effects supports the need for careful monitoring of patients on this therapy. Fastidious reporting of adverse events and objective evaluation of early and late outcomes are necessary to improve our understanding of the use of propranolol for this indication. [ABSTRACT FROM AUTHOR]

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 aright 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives: Knowledge of the length between the upper esophageal sphincter (UES) and the lower esophageal sphincter (LES) in pediatric patients is essential for intraluminal impedance and dual pH probe recordings. Methods; We measured the vertical distance between the true vocal cords (TVCs) and the LES in chest x-rays (CXRs) of 118 children (ages, 6 weeks to 13 years) and measured the vertical distance between the UES and the LES during endoscopy in 31 patients (ages, 14 months to 17 years) and correlated the measurements to height, weight, and age. Results: Esophageal length correlated best with patient height (R = 0.96 by CXR, R = 0.88 by endoscopy) and less well with weight (R = 0.87, R = 0.67) and age (R = 0.94, R = 0.86). Linear regression analyses using radiographic measurements revealed that esophageal length (TVC to LES) can be estimated from a patient's height by the following equation: 1.048 + 0.167 × height (in centimeters). With the upper pH probe placed in the hypopharynx at the TVC level and the inferior probe placed in the esophagus 3 to 6 cm above the LES, the patients were divided into 6 groups corresponding to the currently available number of sizes of dual pH-impedance probes. With the patients" heights between 71.5 and 161.3 cm, 64.7% to 100% of patients were within I cm of the desired location with preselected probes. Confirmation of placement was performed with CXR. Conclusions: A pediatric patient s height can be used to estimate the esophageal length (TVC to LES) and facilitate the selection of dual pH-impedance probes. Our method decreases the risk of morbidity while increasing the accuracy of the study of extraesophageal reflux disease. [ABSTRACT FROM AUTHOR]

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 antiapoptotic 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. [ABSTRACT FROM AUTHOR]