Diagnosis and Management of Riga-Fede Disease: A Case Series.

In this case series, we present four unique cases of Riga-Fede disease (RFD), a rare disorder characterized by mucosal trauma as a result of repetitive tongue protrusion against the incisors, leading to the development of a large oral mass/ulceration. Due to the rapid development and growth of these lesions mimicking malignancy, it is important for the general and pediatric otolaryngologist to correctly diagnose and treat this benign disorder. This series highlights the variable clinical presentations, along with comorbidities of RFD, as well as the importance of interdisciplinary care between the pediatric otolaryngologist and pediatric dentist in its management. Laryngoscope, 2024.

Clinical Consensus Statement: Ankyloglossia in Children.

OBJECTIVE: To identify and seek consensus on issues and controversies related to ankyloglossia and upper lip tie in children by using established methodology for American Academy of Otolaryngology-Head and Neck Surgery clinical consensus statements. METHODS: An expert panel of pediatric otolaryngologists was assembled with nominated representatives of otolaryngology organizations. The target population was children aged 0 to 18 years, including breastfeeding infants. A modified Delphi method was used to distill expert opinion into clinical statements that met a standardized definition of consensus, per established methodology published by the American Academy of Otolaryngology-Head and Neck Surgery. RESULTS: After 3 iterative Delphi method surveys of 89 total statements, 41 met the predefined criteria for consensus, 17 were near consensus, and 28 did not reach consensus. The clinical statements were grouped into several categories for the purposes of presentation and discussion: ankyloglossia (general), buccal tie, ankyloglossia and sleep apnea, ankyloglossia and breastfeeding, frenotomy indications and informed consent, frenotomy procedure, ankyloglossia in older children, and maxillary labial frenulum. CONCLUSION: This expert panel reached consensus on several statements that clarify the diagnosis, management, and treatment of ankyloglossia in children 0 to 18 years of age. Lack of consensus on other statements likely reflects knowledge gaps and lack of evidence regarding the diagnosis, management, and treatment of ankyloglossia. Expert panel consensus may provide helpful information for otolaryngologists treating patients with ankyloglossia.

Stridor in the Newborn.

Stridor in the newborn period may result from numerous causes, both congenital and acquired. Its presentation is diverse, and understanding the subtleties of that diversity is the key to determining the likely cause of the stridor, as well as the urgency for specialist evaluation. This article presents a framework for evaluating the quality of stridor in the newborn, as well as a review of the characteristics of stridor associated with entities commonly encountered in the neonatal airway.

Clinical Practice Guideline for the Management of Infantile Hemangiomas.

Infantile hemangiomas (IHs) occur in as many as 5% of infants, making them the most common benign tumor of infancy. Most IHs are small, innocuous, self-resolving, and require no treatment. However, because of their size or location, a significant minority of IHs are potentially problematic. These include IHs that may cause permanent scarring and disfigurement (eg, facial IHs), hepatic or airway IHs, and IHs with the potential for functional impairment (eg, periorbital IHs), ulceration (that may cause pain or scarring), and associated underlying abnormalities (eg, intracranial and aortic arch vascular abnormalities accompanying a large facial IH). This clinical practice guideline for the management of IHs emphasizes several key concepts. It defines those IHs that are potentially higher risk and should prompt concern, and emphasizes increased vigilance, consideration of active treatment and, when appropriate, specialty consultation. It discusses the specific growth characteristics of IHs, that is, that the most rapid and significant growth occurs between 1 and 3 months of age and that growth is completed by 5 months of age in most cases. Because many IHs leave behind permanent skin changes, there is a window of opportunity to treat higher-risk IHs and optimize outcomes. Early intervention and/or referral (ideally by 1 month of age) is recommended for infants who have potentially problematic IHs. When systemic treatment is indicated, propranolol is the drug of choice at a dose of 2 to 3 mg/kg per day. Treatment typically is continued for at least 6 months and often is maintained until 12 months of age (occasionally longer). Topical timolol may be used to treat select small, thin, superficial IHs. Surgery and/or laser treatment are most useful for the treatment of residual skin changes after involution and, less commonly, may be considered earlier to treat some IHs.

Management of Infantile Hemangiomas of the Airway.

Infantile hemangiomas (IHs) of the airway are far less common than their cutaneous counterparts, and their symptoms mimic those of viral croup. As a result, by the time these lesions are diagnosed, they are often advanced and causing airway compromise. Fortunately, the evolution of propranolol as an effective and safe pharmacotherapy has simplified management of IH and reduced the likelihood of complications previously seen with steroid therapy and surgery. Nevertheless, the otolaryngologist must be prepared with an alternate plan to manage lesions refractory to pharmacotherapy. This article reviews the clinical presentation and current management of IHs of the airway.

Education on, Exposure to, and Management of Vascular Anomalies During Otolaryngology Residency and Pediatric Otolaryngology Fellowship.

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

Diagnosis and Management of Infantile Hemangioma.

Infantile hemangiomas (IHs) are the most common tumors of childhood. Unlike other tumors, they have the unique ability to involute after proliferation, often leading primary care providers to assume they will resolve without intervention or consequence. Unfortunately, a subset of IHs rapidly develop complications, resulting in pain, functional impairment, or permanent disfigurement. As a result, the primary clinician has the task of determining which lesions require early consultation with a specialist. Although several recent reviews have been published, this clinical report is the first based on input from individuals representing the many specialties involved in the treatment of IH. Its purpose is to update the pediatric community regarding recent discoveries in IH pathogenesis, treatment, and clinical associations and to provide a basis for clinical decision-making in the management of IH.

Multicenter Evaluation of the Effectiveness of Systemic Propranolol in the Treatment of Airway Hemangiomas.

OBJECTIVE: To evaluate the effectiveness of systemic propranolol in airway infantile hemangioma (AIH) treatment. DESIGN: Case series with chart review. PARTICIPANTS: Patients with AIH treated with propranolol between 2009 and 2012 with at least a 1-year follow-up. OUTCOMES: Presenting age, treating institution, syndrome presence, presenting AIH stage, endoscopy status, propranolol initiation location/dose/duration, time to stridor resolution, adjunctive medical and surgical therapy, and treatment-associated complications. RESULTS: Twenty-seven patients met inclusion criteria. Average age of diagnosis was 2.3 months (range, 1-5 months). The AIHs were stage 1 in 7 of 27 (26%), stage 2 in 12 of 27 (44%), and stage 3 in 8 of 27 (30%). Propranolol initiation was inpatient in 25 of 27 (93%) and outpatient in 2 of 27 (7%). Propranolol dose was maintained at 2 mg/kg/d in all patients for a minimum of 7 months (range, 7-34 months; median, 15 months). Stridor was eliminated within 24 hours or less of propranolol initiation in 23 of 27 (85%). At diagnosis, staging and propranolol initiation in 11 of 27 (41%) were managed with propranolol alone; the remaining 16 of 27 (59%) also had a steroid injection. The use of adjuvant therapy at the time of propranolol initiation and the size of the AIH were not statistically correlated. Twelve patients had additional treatments after the initiation of propranolol due to recurrence of respiratory symptoms, 1 of 27 (4%) of whom was considered a nonresponder. No complications related to propranolol use were noted. CONCLUSIONS: This multisite study of AIH treatment with propranolol demonstrates similar effectiveness to surgical treatment modalities. Propranolol therapy for AIH had no complications, had potentially lower resource utilization, and should be considered a first-line AIH treatment.

Laryngoscope Illuminance in a Tertiary Care Medical Center: Industry Standards and Implications for Quality Laryngoscopy.

OBJECTIVE: To test the hypothesis that a substantial proportion of laryngoscopes exhibit substandard illuminance by comparing laryngoscope illuminance in a tertiary-level medical center to established standards and identifying features associated with poor illuminance. STUDY DESIGN: Cross-sectional observational study. SETTING: Academic tertiary care medical center (level 1 trauma center, specialty cardiac hospital, and general hospital). SUBJECTS AND METHODS: Laryngoscopes from main, cardiac, and outpatient operating rooms; emergency department; and code carts were tested using a standard technique. Illuminance (lux) was chosen as the outcome measure. Benchmarks were derived from the International Standards Organization and medical literature. Light types included incandescent bulb, light-emitting diode, and xenon. Personnel were surveyed regarding maintenance practices. RESULTS: Across all hospitals, 691 laryngoscopes were tested. Mean (SD) illuminance was 810 (700) lux for incandescent bulb-on-blade designs (n = 237), 1860 (1220) lux for incandescent bulb in-handle designs (n = 79), 4730 (3210) lux for LED (n = 354), and 28,800 (34,500) lux for xenon (n = 21). Seven percent of units failed to turn on (n = 45). Using an established threshold of 867 lux, 28% of devices (47% of incandescent, 12% of LED, and 10% of xenon) were substandard. All laryngoscopes were cleaned according to standard protocols following use; no preventive maintenance was reported. CONCLUSION: Twenty-eight percent of laryngoscopes in a tertiary care hospital exhibit substandard illuminance; these results corroborate the findings of our inaugural study on this subject. Consequently, our hospital is instituting changes to reduce the likelihood of substandard performance by laryngoscopes in circulation.

Standardized outcome and reporting measures in pediatric head and neck lymphatic malformations.

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

Laryngoscope illuminance in a tertiary children's hospital: implications for quality laryngoscopy.

IMPORTANCE: Laryngoscopes are used by otolaryngologists in a variety of hospital emergency and critical care settings. However, only rarely have quality-related aspects of laryngoscope function and application been studied. OBJECTIVES: To compare the illuminance of laryngoscopes commonly used in a hospital setting to established standards and to assess the potential effects of maintenance practices on laryngoscope illuminance. DESIGN, SETTING, AND PARTICIPANTS: Observational study of laryngoscope light output and cross-sectional survey of individuals charged with laryngoscope maintenance in a tertiary care children's hospital. INTERVENTIONS: Illuminance was chosen as the unit of measurement (lux). Laryngoscopes in the operating room, emergency department, and pediatric intensive care unit were tested according to a standard technique. Illuminance standards for laryngoscopes, published by the International Organization for Standardization (ISO) (500 lux) and in the medical literature (867 lux) were used as benchmarks. MAIN OUTCOMES AND MEASURES: Mean laryngoscope illuminance by type of laryngoscope and light source and percentage of laryngoscopes with illuminance below established standards as well as nonfunctioning units. Maintenance practices were evaluated as a secondary outcome. RESULTS: A total of 319 laryngoscopes were tested; 283 were incandescent bulb units used by anesthesiologists, emergency physicians, and intensivists and 36 were xenon light units used by otolaryngologists. Mean (SD) illuminance was 1330 (1160) lux in the incandescent group and 16,600 (13,000) lux in the xenon group (P < .001). Substandard illuminance was observed only in the incandescent group, in 29% to 43% of laryngoscopes; 5% of the incandescent group did not turn on at all. Maintenance of laryngoscopes was performed on a reactive rather than a preventive basis. CONCLUSIONS AND RELEVANCE: At our facility, approximately one-third of incandescent laryngoscopes exhibited substandard light output. On the basis of these findings, our hospital has converted all of its incandescent laryngoscopes to light-emitting diode (LED) devices. Such changes, as well as the institution of a quality-control program including scheduled laryngoscope inspection and battery and bulb replacement for incandescent laryngoscopes, may reduce adverse events associated with poor-quality direct laryngoscopy.

Clinical practice guideline for the diagnosis and management of acute bacterial sinusitis in children aged 1 to 18 years.

OBJECTIVE: To update the American Academy of Pediatrics clinical practice guideline regarding the diagnosis and management of acute bacterial sinusitis in children and adolescents. METHODS: Analysis of the medical literature published since the last version of the guideline (2001). RESULTS: The diagnosis of acute bacterial sinusitis is made when a child with an acute upper respiratory tract infection (URI) presents with (1) persistent illness (nasal discharge [of any quality] or daytime cough or both lasting more than 10 days without improvement), (2) a worsening course (worsening or new onset of nasal discharge, daytime cough, or fever after initial improvement), or (3) severe onset (concurrent fever[temperature ≥39°C/102.2°F] and purulent nasal discharge for at least 3 consecutive days). Clinicians should not obtain imaging studies of any kind to distinguish acute bacterial sinusitis from viral URI, because they do not contribute to the diagnosis; however, a contrast-enhanced computed tomography scan of the paranasal sinuses should be obtained whenever a child is suspected of having orbital or central nervous system complications. The clinician should prescribe antibiotic therapy for acute bacterial sinusitis in children with severe onset or worsening course. The clinician should either prescribe antibiotic therapy or offer additional observation for 3 days to children with persistent illness. Amoxicillin with or without clavulanate is the firstline treatment of acute bacterial sinusitis. Clinicians should reassess initial management if there is either a caregiver report of worsening(progression of initial signs/symptoms or appearance of new signs/symptoms) or failure to improve within 72 hours of initial management.If the diagnosis of acute bacterial sinusitis is confirmed in a child with worsening symptoms or failure to improve, then clinicians may change the antibiotic therapy for the child initially managed with antibiotic or initiate antibiotic treatment of the child initially managed with observation. CONCLUSIONS: Changes in this revision include the addition of a clinical presentation designated as "worsening course," an option to treat immediately or observe children with persistent symptoms for 3 days before treating, and a review of evidence indicating that imaging is not necessary in children with uncomplicated acute bacterial sinusitis.

The financial value of fellowship training in otolaryngology.

OBJECTIVE: To evaluate the financial impact of pursuing a fellowship in otolaryngology. STUDY DESIGN: Retrospective financial analysis using American Academy of Otolaryngology-Head and Neck Surgery survey data. SUBJECTS AND METHODS: The American Academy of Otolaryngology-Head and Neck Surgery report, entitled Socioeconomic Study among Members April 2011, gives a financial profile of respondents who reported their primary area of specialization as either general otolaryngology or a specific area of subspecialization. Weighted averages were calculated from the reported data. The weighted averages were used to calculate a net present value (NPV) over a 30-year contiguous career. RESULTS: The NPV for general otolaryngology was $4.73 million. The NPV for the following subspecialties in relation to general otolaryngology were (in hundred thousands) as follows: otolaryngologic allergy (-$1153), sleep medicine (-$677), otology/neurotology (-$339), laryngology (-$288), head and neck (-$191), pediatric otolaryngology (-$176), facial plastic surgery (-$139), skull base surgery ($122), rhinology ($285), and allergy and immunology ($350). Ninety-four percent of general otolaryngology respondents were in private practice. Most subspecialists worked in an academic setting. CONCLUSION: Fellowship training in otolaryngology will affect career earnings of prospective fellows. The overall financial impact of fellowship training, calculating in the delay in receiving a full clinical salary, should be factored into the decision to pursue fellowship training.

Propranolol use for infantile hemangiomas: American Society of Pediatric Otolaryngology Vascular Anomalies Task Force practice patterns.

IMPORTANCE: This study provides multi-institutional practice guidelines for the initiation of propranolol hydrochloride treatment of routine infantile hemangiomas. OBJECTIVE: To provide information on current propranolol treatment practices for infantile hemangiomas among a cohort of pediatric otolaryngologists. DESIGN AND SETTING: A survey for initiation of propranolol therapy was created by the American Society of Pediatric Otolaryngology Vascular Anomalies Task Force Subcommittee. After an initial pilot of the survey by 4 task force members, the survey was modified and then distributed by e-mail. Results were transferred to spreadsheet format and analyzed. PARTICIPANTS: All 51 members of the task force. RESULTS: A total of 18 respondents from 15 institutions submitted completed surveys. Data from respondents at the same institution were aggregated and/or averaged to minimize regional bias. Fourteen of 15 responding institutions (93%) treat patients with infantile hemangioma as part of a multidisciplinary vascular anomalies team. Ten institutions (67%) routinely consult cardiology before initiation of propranolol therapy. The median propranolol hydrochloride initiation dosage is 2.00 (mean [SD], 1.65 [0.64]; range, 0.45-2.50) mg/kg/d. Postinitiation monitoring for propranolol therapy includes blood pressure (15 of 15 respondents [100%]), serum glucose levels (7 of 15 [47%]), and pulse oximetry (2 of 15 [13%]). Only 2 institutions routinely admit all patients for initiation of propranolol therapy. Typical duration of therapy ranges from 4 to 8 (5 of 15 [33%]) or 8 to 12 months (10 of 15 [67%]), and cessation of therapy in most cases is based on the clinical response (7 of 14 [50%]) or the age of the patient (6 of 14 [43%]). CONCLUSIONS AND RELEVANCE: Propranolol is a commonly used medication for the treatment of infantile hemangiomas among otolaryngologists in the Vascular Anomalies Task Force. Propranolol therapy is commonly initiated in the outpatient setting and continued for as long as 12 months.

Initiation and use of propranolol for infantile hemangioma: report of a consensus conference.

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

Antibiotics for Otitis Media with Effusion in Children.

The "Cochrane Corner" is a quarterly section in the journal that highlights systematic reviews relevant to otolaryngology-head and neck surgery, with invited commentary to aid clinical decision making. This installment features a Cochrane Review, "Antibiotics for Otitis Media with Effusion in Children," that does not support the routine use of antibiotics for treating otitis media with effusion in children. Although children treated with antibiotics had higher rates of effusion resolution, there was no impact on hearing levels or the need for tympanostomy tubes.

Orodental manifestations of facial port-wine stains.

BACKGROUND: Patients with facial port-wine stains (PWS) often demonstrate oral manifestations of their disorder; however, the spectrum and prevalence of such findings among a cohort of patients with PWS has not been established. As a result, dermatologists and oral health specialists may be uncertain how to counsel their patients with PWS regarding oral hypervascularity, bony oral changes, and oral hygiene. OBJECTIVES: We sought to identify physical findings and complications involving the teeth, oral cavity, and perioral structures in individuals with facial PWS. METHODS: This was a cross-sectional study of 30 patients with facial PWS. Descriptive data were collected through anonymous paired surveys completed by patients and their dentists, and analyzed (Fisher exact test) for trends based on physical findings and stage of the PWS. RESULTS: The most common orodental manifestations according to patients were enlargement of the lip (53.3%), stained gums (46.7%), abnormal bite (30%), and spontaneous bleeding of the gums (26.7%). Staining of the gingiva correlated significantly with gingival hyperplasia (P = .006), maxillary hyperplasia (P = .014), and widened interdental spaces (P = .002), and in all cases gingival staining predated these findings. Lip hyperplasia was reported more frequently by patients than by their dentists (50% vs 18.2%, P = .008). Orodental manifestations were more common among patients with darker and thicker PWS. Hemorrhage after dental procedures was rare (4.5%). LIMITATIONS: Modest sample size and difficulty recruiting control subjects are limitations. CONCLUSIONS: Facial PWS commonly affect the orodental structures, and intraoral staining may predict future complications.