Systemic Bevacizumab for Treatment of Respiratory Papillomatosis: International Consensus Statement.

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.

Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis.

BACKGROUND: Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. METHODS: A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included. Exploratory data analysis was conducted followed by inferential analyses with frequentist and Bayesian statistics. RESULTS: We examined 339 subjects: 82% were diagnosed prior to the age of 18 years, 65% were infected with HPV6, and 69% had an aggressive clinical course. When comparing age at diagnosis with clinical course, the probability of aggressiveness is high for children under five years of age then drops rapidly. For patients diagnosed after the age of 10 years, an indolent course is more common. After accounting for confounding between HPV11 and young age, HPV type was minimally associated with aggressiveness. Fast and Frugal Trees (FFTs) were utilized to determine which algorithms yield the highest accuracy to classify patients as having an indolent or aggressive clinical course and consistently created a branch for diagnostic age at ~5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors. CONCLUSION: In the largest cohort of its type, we have identified a critical age at diagnosis which demarcates a more aggressive from less aggressive clinical course.

Microbial flora and antibiotic resistance in odontogenic abscesses in Upstate New York.

Abscesses in the head and neck frequently have odontogenic sources. As bacterial pathogens and antibiotic resistance patterns may change over time and based on location, we describe the current common bacteria found in odontogenic abscesses, the prevalence of antibiotic resistance, and differences in each between pediatric and adult patients in Upstate New York. This is a retrospective review of patients who underwent drainage of odontogenic abscesses (n = 131) from 2002 to 2012 at an academic institution. The medical records were reviewed for results of abscess cultures, comorbidities, and drainage procedures. Polymicrobial sources were identified in 60.3% and monomicrobial in 33.6%. Overall, the most common bacteria were alpha hemolytic Streptococci (33.6%), Streptococcus milleri (32.1%), Prevotella (16.8%), and coagulase-negative Staphylococcus (14.5%). Candida and Morganella spp were more common in children than in adults. Overall, antibiotic resistance was observed in seven different pathogens. The most common antibiotic resistances were to clindamycin and erythromycin, which should be considered when deciding initial antibiotic therapy, especially in adult patients, who trended in this study toward having pathogens with higher rates of resistance.

Transoral resection of extensive pediatric supraglottic neurofibroma.

This case report describes the novel use of transoral robotic surgery (TORS) with delayed transoral laser microsurgery (TLM) for residual disease in the resection of a pediatric neurofibroma with supraglottic and parapharyngeal space extension. Postoperatively, the patient had excellent functional outcomes. This case demonstrates that pediatric TORS is feasible, safe, and complementary to TLM. Neurofibroma is just one of the diverse pathologic processes of the pediatric airway that can be managed via transoral surgery, and applications of TORS with TLM will continue to broaden as our experience develops. Laryngoscope, 2525-2528, 2018.

Changes in Tonsil B Cell Phenotypes and EBV Receptor Expression in Children Under 5-Years-Old.

BACKGROUND: Palatine tonsils are principally B cell organs that are the initial line of defense against many oral pathogens, as well as the site of infection for others. While the size of palatine tonsils changes greatly in the first five years of life, the cellular changes during this period are not well studied. Epstein Barr virus (EBV) is a common orally transmitted virus that infects tonsillar B cells. Naïve B cells are thought to be the target of primary infection with EBV in vivo, suggesting that they are targeted by the virus. EBV enters B cells through CD21, but studies of older children and adults have not shown differences in surface CD21 between naïve B cells and other tonsil B cell populations. METHODS: In this study, we used an 11-color flow cytometry panel to detail the changes in B cell subpopulations in human tonsils over the first five years of life from 33 healthy US children. RESULTS: We provide reference ranges for tonsil B cell subpopulations over this age range. We show that the frequency of naïve tonsil B cells decreases over the early years of life, and that naïve B cells expressed higher surface levels of CD21 relative to other tonsil B cell populations. CONCLUSIONS: We show that young children have a higher frequency of naïve tonsil B cells, and importantly that these cells express increased surface EBV receptor, suggesting that young children have a larger pool of cells that can be infected by the virus. © 2017 International Clinical Cytometry Society.

Incremental change in cross sectional area in small endotracheal tubes: A call for more size options.

OBJECTIVE: To elucidate the relatively large incremental percent change (IPC) in cross sectional area (CSA) in currently available small endotracheal tubes (ETTs), and to make recommendation for lesser incremental change in CSA in these smaller ETTs, in order to minimize iatrogenic airway injury. METHODS: The CSAs of a commercially available line of ETTs were calculated, and the IPC of the CSA between consecutive size ETTs was calculated and graphed. The average IPC in CSA with large ETTs was applied to calculate identical IPC in the CSA for a theoretical, smaller ETT series, and the dimensions of a new theoretical series of proposed small ETTs were defined. RESULTS: The IPC of CSA in the larger (5.0-8.0 mm inner diameter (ID)) ETTs was 17.07%, and the IPC of CSA in the smaller ETTs (2.0-4.0 mm ID) is remarkably larger (38.08%). Applying the relatively smaller IPC of CSA from larger ETTs to a theoretical sequence of small ETTs, starting with the 2.5 mm ID ETT, suggests that intermediate sizes of small ETTs (ID 2.745 mm, 3.254 mm, and 3.859 mm) should exist. CONCLUSION: We recommend manufacturers produce additional small ETT size options at the intuitive intermediate sizes of 2.75 mm, 3.25 mm, and 3.75 mm ID in order to improve airway management for infants and small children.

Microbial Flora and Antibiotic Resistance in Peritonsillar Abscesses in Upstate New York.

OBJECTIVES: To identify the common bacteria in recent peritonsillar abscesses and the prevalence of antibiotic resistance and compare both between adults and children. METHODS: This is a retrospective chart review at a single academic institution of patients who underwent either incision and drainage or tonsillectomy for a peritonsillar abscess between 2002 and 2012 (n=69). Medical records were reviewed for cultures, comorbidities, and drainage procedures. RESULTS: Cultures obtained from 62.32% of peritonsillar abscesses were polymicrobial, and 34.78% were monomicrobial. The most common pathogens were ß-hemolytic Streptococcus (31.88%), α-hemolytic Streptococcus (21.74%), Neisseria (14.49%), and Streptococcus milleri (13.04%). Group A ß-hemolytic streptococcus was more common in children and Streptococcus milleri was more common in adults. Alpha-hemolytic streptococcus was resistant to clindamycin (6.67%) and erythromycin (6.67%). Streptococcus milleri was resistant to clindamycin (11.11%) and erythromycin (11.11%). Staphylococcus was resistant to penicillin (37.5%), oxacillin (25%), erythromycin (25%), and clindamycin (12.5%). CONCLUSIONS: ß- and α-hemolytic Streptococci, Neisseria, and Streptococcus milleri are the most common pathogens. Streptococcus milleri is more common in adults, and ß-hemolytic streptococcus is more common in children. Resistance to clindamycin and erythromycin is common in Streptococci and Staphylococci, and penicillin resistance is common in Staphylococci.

Acute chest syndrome following adenotonsillectomy in a pediatric patient with Hemoglobin SC disease.

Hemoglobinopathies involving Hemoglobin S, like Hemoglobin SC disease, are characterized by anemia and vaso-occlusive crises. Vaso-occlusive crises can range in severity from localized pain to acute chest syndrome and myocardial infarction. These crises are usually brought on by stressors that induce hypothermia, hypoxia or acidosis, such as surgery [1]. Here we report a case of acute chest syndrome in a child with Hemoglobin SC disease following adenotonsillectomy and review of the literature regarding acute chest syndrome following adenotonsillectomy.

Impact on hearing of routine ear suctioning at the tympanic membrane.

OBJECTIVE: Patient and equipment safety has become increasingly scrutinized in today's medical care. Routine otolaryngologic evaluation often involves suctioning with Frazier-type suction devices in the ear canal for improved visualization, but data are limited on the potential acoustic trauma from ear canal suction devices. This study intends to document the objective and subjective effects of ear canal suctioning to identify any risk for hearing threshold shifts or other potential negative effects. PATIENTS AND METHODS: Prospective study on 21 healthy volunteers enlisted for evaluation. Presuctioning tympanogram, audiogram, and otoacoustic emissions data were obtained. Spectrum analyses were recorded during ear canal suctioning with a probe microphone placed lateral to the tympanic membrane. Subjective data were recorded, and a follow-up audiogram and otoacoustic emissions were obtained to identify any temporary threshold shifts. RESULTS: Spectrum analyses revealed a high degree of variability between subjects. A peak intensity of 111 dB sound pressure level was recorded. All patients tolerated suctioning, and none reported hearing loss. No threshold shifts were observed. Subjective data failed to correlate with the objective recorded intensities. CONCLUSIONS: Clinicians and patients need to be acutely aware of potential risks and benefits from any medical intervention. Routine ear canal suctioning can be extremely loud and uncomfortable for patients. This study failed to document objective proof of hearing detriment from ear canal suctioning, although the possibility exists during office and surgical intervention. Further study and potential alternative suctioning methods deserve attention.

Postcricoid hemangioma of childhood: report of four cases.

Hemangiomas are the most common tumor of infancy, and the vast majority occur in the head and neck region. In children, laryngeal hemangiomas typically occur below the level of the true vocal folds, in the region of the subglottis, and other sites are exceedingly rare. We present four cases of hemangiomas located in the postcricoid region of the hypopharynx. Because of the location of these lesions, children may present with obstructive symptoms such as dysphagia, intermittent aspiration, hypersalivation, or recurrent respiratory infections. Clinical diagnosis is relatively easily made with flexible laryngoscopy, as the lesions have a propensity to enlarge with crying or straining. When these patients are examined under general anesthesia in a relaxed state, however, the lesions are typically much smaller, and can even go unnoticed. Unlike other reported cases, the postcricoid hemangiomas in our patients were not causing any symptoms and were simply incidental findings. Thus, we believe that the true incidence of postcricoid hemangiomas is likely higher than reports suggest. To our knowledge, we report the longest follow-up (6 years) of a patient with a postcricoid hemangioma and are the first to describe the natural course of such a lesion.

Velo-cardio-facial syndrome: the pediatric otolaryngologist's perspective.

PURPOSE OF REVIEW: The understanding of velo-cardio-facial syndrome has grown markedly since the initial descriptions of this common genetic disorder nearly 30 years ago. Our knowledge of the syndrome has advanced in part from opportunities to monitor many patients into adulthood because of advances in the fields of cardiothoracic surgery and immunology. Longitudinal study has brought to light psychiatric and behavioral features of the syndrome that are often not apparent until late adolescence or the early adult years. Certain endocrine and immunologic features of the syndrome thought to be resolved in childhood are now witnessed in older patients. Variable expression and lack of disease awareness are two major factors that contribute to the delays in diagnosis in many cases. To address this, there has been a call to delineate screening parameters for patients at risk of carrying the deletion. RECENT FINDINGS: Several areas are highlighted in this review, reflecting the focus of scholarly work on velo-cardio-facial syndrome in the past year. Molecular genetics has shown smaller deletions in many families with the syndrome. The gene TBX1 has been found to be important to the phenotype. Surgical outcomes data reveal the greater challenges involved in correcting velopharyngeal insufficiency. SUMMARY: Defining the genetic basis of velo-cardio-facial syndrome will allow clinicians and basic scientists to make further inroads into understanding the variable expressivity of this syndrome. It is also important to be aware of the continued diagnostic challenges encountered by clinicians in attempts to improve the detection of patients with this syndrome.

Management of pediatric hemoptysis: review and a case of isolated unilateral pulmonary artery agenesis.

OBJECTIVES: Management of hemoptysis in the pediatric patient requires an otolaryngologist knowledgeable and skilled in the causes and management of this potentially life-threatening condition. This paper will discuss the management of pediatric hemoptysis by the otolaryngologist through an index case of massive hemoptysis seen in a child with isolated unilateral pulmonary artery agenesis (IUPAA). STUDY DESIGN: Literature review and index case. METHODS: Through a Medline search and the experience of the author (A.J.M.), we reviewed the causes and treatment options for hemoptysis in the pediatric patient. RESULTS: Management of hemoptysis by the otolaryngologist requires prompt diagnosis and patient stabilization. This allows the patient access to multiple treatment options which may include diagnostic or therapeutic bronchoscopy, angiography with embolization, and surgical intervention such as resection or revascularization. IUPAA is an unusual, and potentially life-threatening cause of hemoptysis in the pediatric patient. CONCLUSION: Hemoptysis in the pediatric patient requires prompt and thorough evaluation and treatment. An approach for the management of pediatric hemoptysis, including massive hemoptysis, is described.