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Objective: To evaluate the safety, immunogenicity, and efficacy of INO-3107, a DNA immunotherapy designed to elicit targeted T-cell responses against human papillomavirus (HPV) types 6 and 11, in adult patients with recurrent respiratory papillomatosis (RRP; NCT04398433)., Methods: Eligible patients required ≥2 surgical interventions for RRP in the year preceding dosing. INO-3107 was administered by intramuscular (IM) injection followed by electroporation (EP) on weeks 0, 3, 6, and 9. Patients underwent surgical debulking within 14 days prior to first dose, with office laryngoscopy and staging at screening and weeks 6, 11, 26, and 52. Primary endpoint was safety and tolerability, as assessed by treatment-emergent adverse events (TEAEs). Secondary endpoints included frequency of surgical interventions post-INO-3107 and cellular immune responses., Results: An initial cohort of 21 patients was enrolled between October 2020 and August 2021. Fifteen (71.4%) patients had ≥1 TEAE; 11 (52.4%) were Grade 1, and 3 (14.3%) were Grade 3 (none treatment related). The most frequently reported TEAE was injection site or procedural pain (n = 8; 38.1%). Sixteen (76.2%) patients had fewer surgical interventions in the year following INO-3107 administration, with a median decrease of 3 interventions versus the preceding year. The RRP severity score, modified by Pransky, showed improvement from baseline to week 52. INO-3107 induced durable cellular responses against HPV-6 and HPV-11, with an increase in activated CD4 and CD8 T cells and CD8 cells with lytic potential., Conclusion: The data suggest that INO-3107 administered by IM/EP is tolerable and immunogenic and provides clinical benefit to adults with RRP., Level of Evidence: 3 Laryngoscope, 133:3087-3093, 2023., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: The purpose of this study is to develop consensus on key points that would support the use of systemic bevacizumab for the treatment of recurrent respiratory papillomatosis (RRP), and to provide preliminary guidance surrounding the use of this treatment modality., Study Design: Delphi method-based survey series., Methods: A multidisciplinary, multi-institutional panel of physicians with experience using systemic bevacizumab for the treatment of RRP was established. The Delphi method was used to identify and obtain consensus on characteristics associated with systemic bevacizumab use across five domains: 1) patient characteristics; 2) disease characteristics; 3) treating center characteristics; 4) prior treatment characteristics; and 5) prior work-up., Results: The international panel was composed of 70 experts from 12 countries, representing pediatric and adult otolaryngology, hematology/oncology, infectious diseases, pediatric surgery, family medicine, and epidemiology. A total of 189 items were identified, of which consensus was achieved on Patient Characteristics (9), Disease Characteristics (10), Treatment Center Characteristics (22), and Prior Workup Characteristics (18)., Conclusion: This consensus statement provides a useful starting point for clinicians and centers hoping to offer systemic bevacizumab for RRP and may serve as a framework to assess the components of practices and centers currently using this therapy. We hope to provide a strategy to offer the treatment and also to provide a springboard for bevacizumab's use in combination with other RRP treatment protocols. Standardized delivery systems may facilitate research efforts and provide dosing regimens to help shape best-practice applications of systemic bevacizumab for patients with early-onset or less-severe disease phenotypes., Level of Evidence: 5 Laryngoscope, 131:E1941-E1949, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal., Study Design: Blinded modified Delphi consensus process., Setting: Tertiary care center., Methods: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items., Results: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus., Conclusions: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated., Level of Evidence: 5. Laryngoscope, 131:1168-1174, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Objectives: To provide recommendations to otolaryngologists, pulmonologists, and allied clinicians for tracheostomy decannulation in pediatric patients., Methods: An iterative questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group., Results: Twenty-six members completed the survey. Recommendations address patient criteria for decannulation readiness, airway evaluation prior to decannulation, decannulation protocol, and follow-up after both successful and failed decannulation., Conclusion: Tracheostomy decannulation recommendations are aimed at improving patient-centered care, quality and safety in children with tracheostomies., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives/hypothesis: Create a competency-based assessment tool for pediatric tracheotomy., Study Design: Blinded, modified, Delphi consensus process., Methods: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items., Results: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus., Conclusions: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure., Level of Evidence: 5 Laryngoscope, 130:2700-2707, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

The lingual frenulum is recognized as having the potential to limit tongue mobility, which may lead to difficulties with breastfeeding in some infants. There is extensive variation between individuals in the appearance of the lingual frenulum but an ambiguous relationship between frenulum appearance and functional limitation. An increasing number of infants are being diagnosed with ankyloglossia, with growing uncertainty regarding what can be considered "normal" lingual frenulum anatomy. In this study, microdissection of four fresh tissue premature infant cadavers shows that the lingual frenulum is a dynamic, layered structure formed by oral mucosa and the underlying floor of mouth fascia, which is mobilized into a midline fold with tongue elevation and/or retraction. Genioglossus is suspended from the floor of mouth fascia, and in some individuals can be drawn up into the fold of the frenulum. Branches of the lingual nerve are located superficially on the ventral surface of the tongue, immediately beneath the fascia, making them vulnerable to injury during frenotomy procedures. This research challenges the longstanding belief that the lingual frenulum is a midline structure formed by a submucosal "band" or "string" and confirms that the neonatal lingual frenulum structure replicates that recently described in the adult. This article provides an anatomical construct for understanding and describing variability in lingual frenulum morphology and lays the foundation for future research to assess the impact of specific anatomic variants of lingual frenulum morphology on tongue mobility. Clin. Anat. 32:824-835, 2019. © 2019 The Authors. Clinical Anatomy published by Wiley Periodicals, Inc. on behalf of American Association of Clinical Anatomists., (© 2019 The Authors. Clinical Anatomy published by Wiley Periodicals, Inc. on behalf of American Association of Clinical Anatomists.)

Surgical release of the lingual frenulum (frenotomy) has become an increasingly common procedure, performed from birth through to adulthood. Surprisingly, detailed anatomy of the in-situ lingual frenulum has never been described, and no anatomical basis has been proposed for the individual variability in frenulum morphology. The lingual frenulum is frequently referred to as a "cord" or "submucosal band" of connective tissue, yet there is no evidence to support this anatomical construct. This paper aims to describe the anatomy of the in-situ lingual frenulum and its relationship to floor of mouth structures. Fresh tissue microdissection of the lingual frenulum and floor of mouth was performed on nine adult cadavers with photo-documentation and description of findings. The lingual frenulum is a dynamic structure, formed by a midline fold in a layer of fascia that inserts around the inner arc of the mandible, forming a diaphragm-like structure across the floor of mouth. This fascia is located immediately beneath the oral mucosa, fusing centrally with the connective tissue on the tongue's ventral surface. The sublingual glands and submandibular ducts are enveloped by the fascial layer and anterior genioglossus fibers are suspended beneath it. Lingual nerve branches are located superficially on the ventral surface of the tongue, immediately deep to the fascia. The lingual frenulum is not a discrete midline structure. It is formed by dynamic elevation of a midline fold in the floor of mouth fascia. With this study, the clinical concept of ankyloglossia and its surgical management warrant revision. Clin. Anat. 32:749-761, 2019. © 2019 The Authors. Clinical Anatomy published by Wiley Periodicals, Inc. on behalf of American Association of Clinical Anatomists., (© 2019 The Authors. Clinical Anatomy published by Wiley Periodicals, Inc. on behalf of American Association of Clinical Anatomists.)

Objective: Oral cavity anomalies may contribute to breastfeeding problems. The objective of this study was to describe our experience in a high-volume breastfeeding difficulty clinic with a focus on posterior ankyloglossia and upper-lip ties., Methods: A retrospective review of patients from a dedicated breastfeeding difficulty clinic from January 2014 to December 2014 was performed. Those identified to have ankyloglossia and/or upper-lip ties underwent release procedures. Subjective breastfeeding changes were documented afterwards., Results: Of the 618 total patients, 290 (47%) had anterior ankyloglossia, 120 (19%) had posterior ankyloglossia, and 14 (2%) had upper-lip tie. Some patients had both anterior ankyloglossia and upper lip-tie (6%), or posterior ankyloglossia and upper-lip tie (5%). For those with anterior ankyloglossia, 78% reported some degree of improvement in breastfeeding after frenotomy. For those with posterior ankyloglossia, 91% reported some degree of improvement in breastfeeding after frenotomy. Upper lip-tie release also led to improved breastfeeding (100%)., Conclusions: Anterior and posterior ankyloglossia and upper-lip tie, or combinations thereof, were commonly recognized in our study population. Many newborns, however, also had no oral cavity anomalies. Although causation cannot be implied, these oral cavity anomalies may contribute to breastfeeding difficulties in some cases., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objectives/hypothesis: The authors sought to define the indications, administration, and adverse events associated with intralesional cidofovir use for recurrent respiratory papillomatosis (RRP)., Study Design: Cross-sectional study., Methods: A 21-question online survey was distributed to 115 selected adult and pediatric laryngeal surgeons internationally. Results were used to draft statements of best practice, which were approved by the full membership of the RRP Task Force., Results: Eighty-two surgeons, who altogether presently manage 3,043 patients with RRP, responded to the survey. Seventy-four surgeons previously used cidofovir, reporting 1,248 patients in the last decade (estimated 801 adults and 447 children). Single indications for adjuvant cidofovir included six or more surgeries per year, increasing frequency of surgery, and extralaryngeal spread (in children). Most adult surgeons use 20 to 40 mg in <4 mL; pediatric surgeons use <20 mg in <2 mL. Scheduled administration following an initiation trial of five injections is common; cidofovir is discontinued following a complete response. Most surgeons biopsy routinely, use special informed consent, and are willing to participate in multi-institutional clinical trials on cidofovir uses, efficacy, and safety., Conclusions: Eighteen statements were approved by the RRP Task Force after discussion of the survey results. Intralesional cidofovir may be initiated if surgical debulking is required every 2 to 3 months. The concept of an adjuvant regimen with regular biopsy is favored. Administration should remain below established safe limits of dosing (3 mg/kg) and volume. Informed consent, including discussion of off-label use and acute kidney injury in children, is important. A special consent form sample is included. There remains a need for high-quality data., (Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.)

Introduction: Ankyloglossia is a congenital condition in which tongue mobility is limited due to an abnormality of the lingual frenulum. The impact of ankyloglossia on breastfeeding is poorly understood but there is a recent trend toward more recognition of this condition and early intervention when needed. Currently, there lacks clear definition of ankyloglossia and different subtypes have been proposed with no clinical correlation., Objective: To determine the prevalence of anterior versus posterior ankyloglossia in a large series of consecutive patients and to assess clinical outcomes after frenotomy., Methods: Retrospective chart review of patients from July 2007 to July 2009 who were diagnosed with ankyloglossia and underwent office frenotomy. Baseline characteristics, specific feeding issues, type of ankyloglossia, and clinical outcomes after frenotomy were reviewed., Results: Of the 341 total patients, 322 (94%) had anterior ankyloglossia and 19 (6%) had posterior ankyloglossia. Median age at presentation was 2.7 weeks (range 1 day of life to 24 weeks); 227 were males and 114 were females. Revision frenotomy rates were significantly higher for the posterior ankyloglossia group (3.7% anterior and 21.1% posterior, p=0.008)., Conclusion: Anterior ankyloglossia is much more common and readily managed when compared to posterior ankyloglossia. Posterior ankyloglossia is a poorly recognized condition that may contribute to breastfeeding difficulties. The diagnosis is difficult due to the subtle clinical findings but relevant health care providers should be aware of this condition. Frenotomy is a simple, safe, and effective intervention for ankyloglossia which improves breastfeeding., (Copyright 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: To identify and characterize food items with high risk of airway obstruction in children younger than 15 years., Methods: This retrospective study collected injury data from 1989 to 1998 for 26 pediatric hospitals in the United States and Canada. Aspiration, choking, ingestion, and insertion injuries due to food items were analyzed. The data included 1429 infants and children. Results were compared with fatality data published by the American Association of Pediatrics in 1984., Results: The 10 food objects with the highest frequency for both injuries and fatalities were identified. Peanuts caused the highest frequency of injury, and hot dogs were most often associated with fatal outcomes. The severity of respiratory distress prior to hospital evaluation varied for different foods. Age younger than 3 years was the highest-risk factor. Key characteristics such as bite size, shape, and texture were analyzed and found to demonstrate relationships with severity of clinical outcomes., Conclusions: Children younger than 3 years remain at greatest risk of food injury and death. We found that hard, round foods with high elasticity or lubricity properties, or both, pose a significant level of risk. Consideration of the key characteristics of the most hazardous foods may greatly decrease airway obstruction injuries. Food safety education can help pediatricians and parents select, process, and supervise appropriate foods for children younger than 3 years to make them safer for this highest-risk population.

Purpose of Review: Subglottic hemangioma is a challenging congenital anomaly involving the larynx in children. Management is not uniform, and no single treatment modality has been accepted as ideal. During the last several years only a few articles have dealt with this topic. The purpose of this review is to examine the published literature and discuss the alternative treatments of SGH. The authors also describe their approach and the use of microdebrider as a new surgical tool., Recent Findings: Observation is an option for the rare case of SGH with minimal airway obstruction. However, most cases will require some additional intervention. Systemic steroids intralesional steroid injection, laser ablation with both the CO2 and KTP lasers, interferon (IFN) and open surgical excision have all been utilized., Summary: The authors found few technical innovations in this field in the last 5 years. All techniques utilized have some degree of success. However, all approaches have downsides and are associated with complications, some of which can be very serious. Ideally, more structured research comparing techniques would be helpful to best determine operative and postoperative management.

Nontuberculous mycobacteria are ubiquitous in the environment. Immunocompetent children are commonly infected by these resilient organisms. Cervical lymphadenitis, the most frequent head and neck manifestation of NTM infection, often presents as chronic, unilateral lymphadenopathy with characteristic violaceous overlying skin changes. Diagnosis is ultimately dependent on culture or histopathologic examination of specimen obtained through excisional lymph node biopsy or FNA. The principal treatment of NTM infection remains the surgical excision of diseased tissue. Antibiotics augment surgical therapy and their potential role as a single-modality therapy continues to be investigated.

Choanal atresia is postulated to be secondary to an abnormality of the rupture of the buccopharyngeal membrane during the embryological period. This condition usually occurs sporadically, but has been described in siblings and successive generations. The genetics remain unclear. We present monozygotic twins with identical findings of unilateral choanal atresia and no other associated anomalies. To our knowledge, this is the first report of such an occurrence.

Objectives: To continue assessment of the benefits and risks of intralesional administration of cidofovir, an acyclic nucleoside phosphonate, for treating severe recurrent respiratory papillomatosis (RRP) in pediatric patients, and to discuss guidelines for larger prospective multi-institutional studies of the use of cidofovir., Design: Prospective case series., Setting: Tertiary care children's hospital., Patients: A total of 10 patients with severe RRP (defined as requiring debulking procedures to maintain airway patency at least once a month) underwent intralesional cidofovir therapy. The original 5 patients have received more than 1 year of follow-up since their last cidofovir injection, and 5 subsequent patients have been treated with a revised injection protocol., Intervention: Microsuspension laryngoscopy with intralesional injection of cidofovir after repetitive carbon dioxide laser treatments and mechanical debulking of papillomas., Main Outcome Measures: Papilloma stage at the time of serial laryngoscopies. Histologic examination of biopsy specimens of laryngeal tissue obtained 1 year or more after last cidofovir injection., Results: There was evidence of marked improvement in the 4 of the 5 new patients enrolled under the revised injection protocol, continuation of a disease-free state in 1 of the original 5 patients, and sustained improvement in 4 of the 5 original patients, resulting in a significantly reduced interval of intervention., Conclusions: Intralesional cidofovir therapy continues to show benefit in the treatment of severe RRP in pediatric patients. Safety profiles have not been fully established, but current histologic data are reassuring.

Objective: To assess the potential benefit of intralesional administration of cidofovir, an acyclic nucleoside phosphonate with activity against several DNA viruses, for treating severe respiratory papillomas in pediatric patients., Design: Prospective case series., Setting: Tertiary care children's hospitals., Patients: Five pediatric patients with severe recurrent respiratory papillomatosis requiring laryngoscopy with carbon dioxide laser therapy more frequently than once a month to maintain airway patency. Each patient underwent between 12 and 33 laryngoscopies with laser treatment prior to being injected with cidofovir., Intervention: Microsuspension laryngoscopy with intralesional injection of cidofovir (Vistide) in conjunction with mechanical debulking and carbon dioxide laser of papillomas., Main Outcome Measure: Papilloma stage at time of serial laryngoscopies., Results: One patient was disease free and 3 patients demonstrated a dramatic response to adjuvant therapy with cidofovir at the 9-month follow-up visit after the last injection of cidofovir. One patient showed an improvement in papilloma stage that was possibly related to concurrent therapy with interferon., Conclusions: Intralesional injection of cidofovir seems to be of benefit in the treatment of severe respiratory papillomatosis in pediatric patients. Larger prospective studies with longer follow-up will be required before cidofovir can be considered an accepted means of managing this difficult disease.

With increasing pneumococcal resistance to penicillin and other antibiotics, use of antibiotic therapy for children with upper respiratory tract infections such as otitis media and sinusitis has become difficult. Selecting an appropriate treatment regimen has become more challenging due to frequent concomitant microbial resistance to multiple antibiotics. In a prospective, nonrandomized study, we obtained middle ear and sinus aspirate specimens from all children undergoing outpatient tympanocentesis or sinus lavage for any indication at our institution over two 4-week periods. One hundred fifty-four specimens were obtained. Of these, 12 grew Streptococcus pneumoniae, 7 of which were resistant to penicillin. A 6-month retrospective review of these patients' medical histories evaluated their antibiotic use prior to surgical intervention. An association between penicillin resistance and recent use of 2 or more antibiotics in children with positive S pneumoniae cultures was confirmed, as has been documented in prior reports. Those with penicillin-resistant S pneumoniae also demonstrated a higher incidence of multidrug-resistant organisms.

Postoperative management of the patient younger than 36 months undergoing adenotonsillectomy has been the subject of many debates. Concerns for early postoperative complications such as airway obstruction, emesis, dehydration, and hemorrhage have led many physicians to consider overnight hospitalization following adenotonsillectomy in very young children. Trends in health care management have had increasing focus on cost effective means of treating patients to limit unnecessary expenditure on the part of the patient, physician, and hospital facility. The purpose of this retrospective review was to analyze two methods of early postoperative management in children less than 36 months old undergoing adenotonsillectomy at the Children's Hospital, San Diego from 1992 to 1997. Three hundred and seven cases were reviewed. Same-day discharge was compared with overnight inpatient observation based on the cost analysis of these two methods of postoperative care. Postoperative care was based on length of stay in the recovery room and as an inpatient. Expense of postoperative care was based on cost calculation for the recovery room and overnight hospitalization. Of the 307 patients, 194 went home the day of surgery and 113 were observed overnight in the hospital. Average hospital cost was higher in the outpatient group than in the inpatient group (P < 0.001). This difference reflects longer recovery room stay (350 min) in the outpatient group compared to the inpatient group (108 min) (P < 0.001). Outpatient adenotonsillectomy in the patient under 36 months may be safe; however, prolonged recovery room stays may actually make outpatient surgery less cost-effective than overnight admission. Recovery room costs are significantly higher per unit time than costs of inpatient hospitalization. Further investigation of cost-effective outpatient observation units may improve cost containment in the outpatient surgical setting.

There is some evidence to suggest that the incidence and complications of ACM may be increasing. However, in the current era of widespread access to health care and broad-spectrum antibiotics, an intratemporal or intracranial complication from acute otitis media may not initially be suspected. The reported case is significant in that the patient was very young, had no underlying disease or immunocompromise, and did not have a known antecedent acute otitis media. With the emergence of resistant streptococcal species and prolonged survival in immunocompromised patients, the relative incidence of complications caused by acute otitis media will probably continue to rise, making it imperative that we raise our index of suspicion for previously rare infectious complications of relatively common diseases.

Objective: As we enter the new millennium, Otitis Media remains the most common cause for an acute care visit to the pediatrician. This paper discusses the various surgical approaches to children who do not respond to medical management for acute otitis, recurrent acute otitis and for chronic otitis media. In addition to discussing the relevant alternatives of intervention, attention is directed to special populations of patients who are likely to demand a great deal of care, the problems that are encountered with typanostomy tubes and the concept of a new era of a "post-tympanostomy tube legacy.", Conclusion: Suggestions are offered as to how we can alter our approach to these patients requiring surgical intervention and thereby reduce long term sequelae.

Objective: To limit endoscopic abscess drainage to the opening of the ethmoid cells involved, adjacent to the subperiosteal orbital abscess., Design: Prospective study., Patients: Twenty children with subperiosteal orbital abscess complicating acute sinusitis., Intervention: Endoscopic opening of the medial wall of the bulla ethmoidalis and of the lamina papyracea., Results: The limited endoscopic approach allowed subperiosteal orbital abscess drainage in all patients with positive clinical outcomes. Extensive ethmoidectomy was not necessary to achieve drainage., Conclusions: Endoscopic subperiosteal orbital abscess drainage does not require complete ethmoidectomy as was previously performed and can be limited to the opening of the bulla ethmoidalis and the lamina papyracea through the bulla ethmoidalis.

In cases of severe laryngomalacia, laser division of the aryepiglottic folds (AEFs) or endoscopic supraglottoplasty may be an ineffective solution. Failure of this technique is rare and the reasons for failure are not well established. The purpose of this study was to describe those cases of laryngomalacia in which endoscopic treatment did not reverse the clinical situation. We introduce the concept of discoordinate pharyngolaryngomalacia (DPLM). DPLM was defined as severe laryngomalacia with complete supraglottic collapse during inspiration, without shortened AEFs or redundant mucosa, and with associated pharyngomalacia. Twenty-seven of 82 children with severe laryngomalacia presented a DPLM. Endoscopic treatment was performed in 16 children and the surgical procedure was inadequate to reverse the clinical problem in these patients. In 10 children correction of additional sites of obstruction was required (uvulopharyngopalatoplasty, surgery of choanal atresia, aortopexy). Tracheostomy was necessary in 13 children. Bi-level positive airway pressure (BiPAP) was used successfully in 2 children and tracheotomy was avoided. Treatment still needs to be better defined.

Nager acrofacial dysostosis, first described by Nager and deReynier in 1948, is a rare syndrome characterized by mandibulofacial dysostosis with associated radial defects. The facial features include downward slanting palpebral fissures, absent eyelashes in the medial third of the lower lids, mandibular and malar hypoplasia, dysplastic ears with conductive deafness, and variable degrees of palatal clefting. Upper limb malformation is a constant feature of Nager syndrome and ranges from thumb hypoplasia to absence of the radial ray. The maxillo-mandibular hypoplasia and associated retroplaced tongue set the stage for early and significant upper airway obstruction. The craniofacial anomalies and associated trismus make emergent airway intubation challenging. We present a case of Nager syndrome with life threatening airway obstruction unresponsive to conservative management. This case and a review of the literature emphasize the importance of early tracheotomy for these patients.

A destructive granulomatous lesion of the temporal bone caused by Coccidioides immitis disseminated from a pulmonary lesion was found in a 4-year-old immunocompetent child. To our knowledge, it is the first case of coccidioidomycosis of the temporal bone reported in the world literature. The child presented with pain in her right ear and a 6-month history of intermittent fever, which partially responded to multiple courses of antibiotics. A tender erythematous postauricular swelling consistent with a subperiosteal abscess subsequently developed over 1 month. A mastoidectomy showed granulation tissue with pockets of purulence, and histologic evaluation of the specimen revealed spherules of C immitis, later verified by culture. The patient responded to intravenous amphotericin B therapy, without evidence of disease recurrence. Coccidioides immitis is endemic in regions of the Southwestern United States, with extremely infectious characteristics and relative resistance to medical therapy. Coccidioidomycosis should be considered in the differential diagnosis of a granulomatous lesion of the temporal bone.

Congenital tracheal stenosis (CTS) is a condition difficult to manage and associated with a high mortality rate. The principles of one-stage laryngotracheoplasty have been adapted to the surgical management of CTS. Three children underwent a one-stage anterior tracheoplasty with costal cartilage grafting and without cardiopulmonary bypass. Extubation was performed within 13 days. One child developed granulation tissue one month postoperatively, and this was removed endoscopically. The three children were free of respiratory obstruction signs during follow-up ranging from 8 months to 3 years. Postoperative endoscopies have shown growth of the grafted trachea.

The ethmoid sinuses play a key role in the health or disease of the entire paranasal sinus complex. Multiple underlying inflammatory processes may easily obstruct the narrow ostia of each ethmoid air cell, leading to infection. By virtue of its unique anatomic position and relationship to the ostiomeatal complex, the ethmoid labyrinth becomes the key to treatment of acute and chronic sinusitis. Surgical intervention for sinus disorders in children has grown in popularity. This article reviews the anatomy and pathophysiology of ethmoid sinusitis and discusses the role of surgery for these disorders.

Subglottic hemangioma is an unusual entity which may involute spontaneously without aggressive surgical intervention; although tracheotomy is sometimes necessary. The actual time course for involution is not clear by reviewing the literature [1,14]. Numerous treatment modalities are described for the treatment of this condition, including tracheotomy, CO2 laser ablation and intralesional steroid injection with or without endotracheal intubation. Surgical resection of subglottic hemangioma is an option which is described in the literature and may be utilized in certain selected cases. We present seven cases of subglottic hemangioma treated at three institutions which were resected via a crico-tracheotomy approach. Postoperative follow-up for these patients range from 6 months to 4.5 years. Although conservative measures are still advocated as the treatment of choice for subglottic hemangioma, open surgical resection may be indicated in selected cases resulting in a satisfactory outcome.

Respiratory distress in the neonate may be due to extrinsic tracheobronchial compression. We recently observed a neonate with left mainstem bronchus obstruction resulting in respiratory distress and mediastinal shift. Initial management required intubation and positive pressure ventilation. Bronchoscopic evaluation demonstrated compression of the proximal left main bronchus. The distal bronchus appeared normal. Bronchography, echocardiography and an MRI of the chest confirmed proximal compression of the bronchus and suggested that the cause was an aneurysmal left pulmonary artery and patent ductus arteriosus. With medical therapy there was resolution of the bronchial compression. Re-evaluation by bronchoscopy and echocardiography revealed a normal left main stem bronchus, a normal left pulmonary artery and a closed ductus arteriosus. This clinical scenario suggests that transient pulmonary hypertension may result in significant airway compression due to the proximity of the left main stem bronchus to the left pulmonary artery.

Nasal masses in the pediatric population present with nasal obstruction, epistaxis and chronic rhinorrhea. We report on a 6-year-old boy with long-standing nasal obstruction. A large left nasal mass was evident on physical examination and CT scan. Techniques of functional endoscopic sinus surgery were used to resect the mass, which, on pathologic examination, proved to be a hamartoma. Hamartomas are non-neoplastic malformations, or inborn errors of tissue development. They are characterized by an abnormal mixture of tissues indigenous to that area of the body. Review of the literature revealed 5 previously reported cases of nasal hamartomas, only 2 of these in children. Our case is unique in that the hamartoma arose from the ethmoid and maxillary sinuses; previously reported cases arose from the septum and vestibule. This is the first reported case treated using a functional endoscopic sinus surgery approach.

Neonatal otitis media may be an isolated infection or part of a more complex sepsis syndrome. Because early studies suggested that the microbiologic characteristics of neonatal otitis media included a high risk of gram-negative coliforms and Staphylococcus aureus, tympanocentesis was recommended routinely. Subsequent studies have supported empiric medical therapy in selected patients, reserving tympanocentesis for patients in whom medical treatment fails. Because of these conflicting recommendations, records from 37 neonates with otitis media who underwent tympanocentesis from 1986 through 1991 were studied retrospectively. Cultures in one outpatient (11%) and four inpatients (13%) yielded Escherichia coli, all of which were sensitive to amoxicillin. No cultures of S aureus were identified. Sterile cultures and pneumococcus isolates were found most frequently. No septic or intracranial complications were noted. All patients were discharged on regimens of routine antibiotics for otitis media.

An unusual case of fibrous hamartoma of infancy is reported. This lesion presented as a gradually enlarging, perspiring left neck mass in an eight month old male. Histologic examination revealed the characteristic features of this entity, with the additional finding of eccrine gland hyperplasia. In a review of the literature, we found no other reports of this unique histologic finding or clinical presentation.

The etiologies of pediatric neck masses are legion. Optimal management depends upon making the correct diagnosis. A detailed history to ascertain the duration of the illness, presence of systemic symptoms, animal exposures, tuberculous contacts, medications ingested, recent trauma, dental work or upper respiratory infection (URI), as well as a travel history, may yield important clues to establishing the diagnosis. The work-up for neck masses must be individualized based upon history and physical examination. Common diagnostic studies often obtained are listed in Table 1, and will be further clarified as individual diagnoses are discussed.

Five newborns presented with cystic lesions of the floor of mouth. Four of these patients proved to have congenitally imperforate submandibular salivary gland ducts and the other newborn proved to have a duplication anomaly of the submandibular gland duct and gland. The patients with imperforate Wharton's ducts underwent marsupialization with or without ductoplasty and have been without evidence of recurrence for up to three years. The duplication anomaly of the submandibular gland duct responded to simple excision. The diagnosis of congenital anomalies of the submandibular gland and duct can be made on physical examination. Magnetic resonance imaging can be helpful in differentiating congenital imperforate submandibular duct and duplication anomalies of the ductal system. Treatment of the former consists of duct marsupialization in the floor of mouth with or without ductoplasty. Treatment of the duplicated ductal system may best be treated with excision. A failure in diagnosis and treatment may result in ranula formation or sialoadenitis requiring more extensive therapy.

Gastroesophageal reflux (GER) in children may be classified as physiologic or pathologic, depending on its degree and consequences. There are many head and neck complications of GER in pediatric patients, but most numerous are the airway manifestations, including stridor, recurrent croup, exacerbation of subglottic stenosis, laryngeal irritation with or without laryngospasm, chronic cough, and obstructive apnea. Diagnosis may be difficult unless there is a high index of suspicion for GER and awareness of the concept of "silent" GER. We present the common pediatric airway manifestations of GER, illustrated by case reports, and provide a paradigm to assist in the diagnosis and management of children with airway compromise associated with GER.

Congenital rests of gastric epithelium have been reported in a variety of head and neck locations. Presenting symptoms of these lesions range from an asymptomatic cyst to one which is compromising the airway. The true diagnosis is rarely suspected prior to excision. A case of a gastric choristoma of the tongue is presented which was noted at birth as an intermittently bleeding ulcer. Complete excision of the mass was difficult to ascertain due to poor delineation of the tumor margins from the tongue musculature. A postoperative technetium-99m pertechnetate scan failed to demonstrate residual gastric mucosa. Conservative management resulted in only transient healing of the ulcer. Repeat excision demonstrated abundant residual gastric mucosa. Technetium-99m pertechnetate scanning may not be a reliable indicator of ectopic gastric mucosa in the head and neck region.

The use of corticosteroids to reduce the morbidity associated with laryngotracheobronchitis (croup) has been a controversial issue for many years. Recent literature, however, does support a decreased morbidity and increased clinical response when short-term steroids are used. As a prophylactic measure against bacterial superinfection, antibiotics are commonly utilized in the treatment of croup. We present the case of an otherwise healthy infant with severe croup who was hospitalized and treated with both steroids and antibiotics. A relapse in her symptoms led to the diagnosis of candida laryngotracheitis. We recommend close monitoring of patients with croup treated aggressively with steroids and antibiotics. Steroid use should be limited to 24 h with antibiotics reserved for patients with signs of bacterial infection.

A quantitative analysis of adenoid and tonsillar specimens from 460 patients was performed. The patients underwent tonsillectomy or adenotonsillectomy for either recurrent tonsillitis or obstructive tonsillar hypertrophy. Histologic preparations revealed Actinomycetes as statistically more prevalent in the obstructive population. The presence of Actinomycetes in the adenotonsillar core of this diagnostic group may indicate an etiologic role for this organism in tonsillar and adenoidal hypertrophy.

In an effort to determine if the endotracheal tube-leak pressure has prognostic value in relation to a successful outcome after one-stage laryngotracheal reconstruction or cricoid split surgery, a retrospective analysis was performed on 17 children who had undergone such surgery. The daily leak pressures, length of intubation, and ultimate outcome of attempts at extubation were noted. One hundred percent of patients with a leak pressure of less than 20 cm H2O on the day before extubation were successfully extubated. In contrast, the failure rate was 100% in children extubated with a leak of greater than 30 mm H2O. The difference between these two groups was statistically significant (chi 2, 13.03). Sixty percent of patients with leak pressures in the range of 21 to 30 cm H2O were successfully extubated. The endotracheal tube-leak pressure is a parameter that has prognostic value, and should be considered in determining when to extubate children who have undergone tracheal reconstructive surgery.

The management of subglottic hemangioma in infants remains controversial. We report two patients in whom an open surgical approach was utilized to completely excise the subglottic hemangioma. Both children have been successfully extubated and have remained symptom-free since that time.

Four case reports of infants with cleft palate and intractable otorrhea following the placement of pressure equalization tubes are presented. In one patient, liquids taken orally were noted to reflux through her ears. Otorrhea was refractory to medical management in all cases and was controlled only after closure of the soft palate. Persistent otorrhea may be an indication for early closure of the soft palate in these infants.

This article documents the evolution of our approach to the repair of subglottic stenosis in infants. With increasing experience in the management of subglottic stenosis using the anterior cricoid split procedure, we extended our procedure to include older children. This then led to the use of the one-stage procedure for repair of severe and mature subglottic stenosis. We present the results of surgical repair in 13 patients. Extubation was accomplished in 12 of 13 patients using one-stage laryngotracheoplasty.

More objective thinking regarding the indications for adenotonsillar surgery has gained new respect for these operations. Better understanding of the implications of adenoid disease in chronic otitis and sinusitis, along with appreciation of obstructive airway problems secondary to adenotonsillar hypertrophy, has changed the scope of the operation. Research is ongoing to determine the etiology of chronic antigenic stimulation as a cause for hypertrophy of these organs.

The complete surgical extirpation of branchial cleft abnormalities is essential in avoiding recurrent disease. The most technically difficult maneuver is often the localization and excision of associated sinus tracts and fistulae. The authors communicate a simple yet effective technique for dealing with this difficulty utilizing the Fogarty embolectomy catheter.