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.

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.

Two reports of phacomatosis pigmentovascularis type IIb, one in association with Sturge-Weber syndrome and Klippel-Trenaunay syndrome.

We present two rare cases of phacomatosis pigmentovascularis type IIb, with one patient demonstrating concurrent Sturge-Weber syndrome and Klippel-Trenaunay syndrome. To the best of our knowledge, this is the second infantile case meeting diagnostic criteria for systemic phacomatosis pigmentovascularis type IIb, Sturge-Weber syndrome and Klippel-Trenaunay syndrome in the English language literature.

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.

Human papillomavirus vaccine: recommendations, issues and controversies.

PURPOSE OF REVIEW: The human papillomavirus vaccine is now Food and Drug Administration-approved for girls and young women ages 9-26 years and Advisory Committee on Immunization Practices-recommended for girls ages 11-12 years of age. Several issues have shrouded its debut and continue to provide controversy that may hinder effective delivery of this vaccine to young people in industrialized as well as third world countries. RECENT FINDINGS: The quadrivalent and divalent vaccines are compared, particularly with respect to juvenile recurrent respiratory papillomatosis. Recommendations for administration are summarized. Immunization of men and cost effectiveness are discussed. Issues regarding compulsory vaccination and attitudes of parents about human papillomavirus vaccination are reviewed. SUMMARY: Studies of the human papillomavirus vaccine are very promising, showing excellent efficacy and very few adverse events. It remains to be determined if it will be licensed in the United States for use in boys and men. Some stumbling blocks to implementing the vaccine in the United States need to be addressed in order to gain widespread acceptance.

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.

Surgery for pediatric sleep apnea.

Sleep-related breathing disorders (SRBD) in children are caused by a diverse group of anatomic and physiologic pathologies. These disorders share a common clinical presentation as stertor or sonorous breathing, occasionally accompanied by apneic events of variable duration. Successful management depends on accurate identification of the site of obstruction and the severity of obstruction. Intervention, both surgical and nonsurgical, is tailored to the disorder. In children with SRBD, such intervention may alter behavior and cognition, improve sleep and feeding, or even save a life.

The clinical relevance of the submucosal cricoid cleft.

PURPOSE: Although fairly uncommon and sometimes difficult to diagnose, laryngeal clefts have been well-defined in the literature. The submucosal cleft variant has been described anatomically, but not clinically. We describe a series of patients with a submucosal cricoid cleft variant and its clinical relevance. METHODS: This is a case series of patients presenting with submucosal cricoid. A submucosal cricoid cleft is defined as a dehiscent posterior cricoid plate with an intact mucosal bridge. RESULTS: Eight patients presented during a 4 year period from October 2002 to September 2006. Patient ages ranged from 6 days to 15 years, with a mean age of 40 months. Four patients (50%) had a submucosal cricoid cleft only while four children presented with a laryngotracheal cleft in addition to the submucosal cricoid cleft. Six patients were diagnosed with subglottic stenosis and had tracheotomies. Four patients (50%) failed laryngotracheal reconstruction (LTR) prior to submucosal cricoid cleft diagnosis. Two patients had their submucosal cricoid cleft diagnosed during initial airway reconstruction surgery. Two patients were diagnosed after feeding difficulties. The submucosal cricoid cleft was felt to be an important factor contributing to the failure of previous laryngotracheal reconstruction. Almost all of the patients (7/8) had co-morbidities. Two patients needed revision airway surgery, both successful. Four of the six patients with a tracheostomy have been decannulated. CONCLUSION: Because of the subtle nature of its clinical presentation, submucosal cricoid cleft can be a challenging diagnostic entity, and the results of surgical reconstruction may be compromised if the cleft is undiagnosed.

Post-tonsillectomy morbidity and quality of life in pediatric patients with obstructive tonsils and adenoid: microdebrider vs electrocautery.

OBJECTIVE: To prospectively compare outcomes in children over age 2 with obstructive adenotonsillar hypertrophy when tonsillectomy is performed utilizing an intracapsular microdebrider technique versus low-wattage electrocautery technique. STUDY DESIGN AND SETTING: Prospective, single-blind, randomized controlled trial at a tertiary care children's hospital. RESULTS: Among the 300 children, those treated with the microdebrider resumed normal activity faster, with a median recovery of 2.5 days, and stopped taking pain medication sooner, with the median time to the last dose of 4 days. The microdebrider group were 3 times more likely to no longer need pain medications at 3 days postoperatively and 2.5 times less likely to be still needing pain medication 7 days postoperatively. They were twice as likely to be at a normal activity level by 3 days postoperatively and were less likely to still not have attained normal activity and normal diet after 7 days. There was no difference between groups in median days to return to normal diet (3.0 to 3.5 days). At 1-month follow-up, children in the microdebrider group were 5 times more likely to have residual tonsil tissue. CONCLUSIONS: Intracapsular tonsillectomy in children with obstructive adenotonsillar hypertrophy results in improved peri-operative outcomes. Residual tonsil tissue is more common with use of the microdebrider; however, the incidence of future obstruction or infection is unknown. EBM RATING: A-1b.

Recurrent respiratory papillomatosis.

Recurrent respiratory papillomatosis is a frustrating and challenging disease for surgeons, patients, and patients' families. Although the voice and airway manifestations are managed surgically, a "cure" for this disease remains elusive. In this edition of the "Seminar Series," we endeavor to review the current literature regarding the epidemiology, etiology, clinical manifestations, and surgical and medical treatments of this disorder. The key to future management of recurrent respiratory papillomatosis may lie in its prevention, if current efforts to develop an effective vaccine come to fruition.

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.

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.

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.

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.

Indications for tonsillectomy and adenoidectomy.

OBJECTIVE: To review recent clinical trials that provide a foundation on which clinicians can base decisions regarding adenotonsillar surgery for their patients. STUDY DESIGN: Review. METHODS: An evidence-based approach was used to review recent clinical trials addressing indications for adenotonsillectomy, tonsillectomy, and adenoidectomy. RESULTS: Absolute indications for tonsillectomy and adenoidectomy include adenotonsillar hyperplasia with obstructive sleep apnea, failure to thrive, or abnormal dentofacial growth; suspicion of malignant disease; and (for tonsillectomy) hemorrhagic tonsillitis. Relative indications for both procedures are adenotonsillar hyperplasia with upper airway obstruction, dysphagia, or speech impairment, and halitosis. Otitis media and recurrent or chronic rhinosinusitis or adenoiditis are relative indications for adenoidectomy but not tonsillectomy. Recurrent or chronic pharyngotonsillitis, peritonsillar abscess, and streptococcal carriage are relative indications for tonsillectomy but not adenoidectomy. CONCLUSION: Good clinical evidence regarding indications for tonsillectomy and adenoidectomy is available. Clinicians should make recommendations for surgery on the basis of this evidence.

Effects of nasal saline spray on human neutrophils.

BACKGROUND: Nasal saline spray (NSS) used in the treatment of rhinitis and sinusitis often contains the preservative benzalkonium chloride (BKC). Previous studies have shown that corticosteroid nasal sprays and topical decongestants containing BKC damage respiratory mucosa, decrease mucociliary activity, and inhibit neutrophil functions in vitro. OBJECTIVE: To evaluate the effects of NSS with BKC on human neutrophils. DESIGN: Prospective, basic science observations. METHODS: Human neutrophils were exposed to NSS with BKC or to phosphate-buffered saline (PBS) at varying times and concentrations. The cells were examined for morphologic changes by light microscopy and for viability as determined by trypan blue exclusion. Lactate dehydrogenase levels were measured to quantify neutrophil cell lysis. In vivo morphologic changes were studied in neutrophils obtained from the oral mucosa in human volunteers who rinsed their mouths with NSS or PBS. RESULTS: Neutrophils exposed to NSS concentrations as low as 15% showed near-total cell lysis, and neutrophils exposed to 20% NSS demonstrated no cell viability by trypan blue staining. Phosphate-buffered saline-exposed cells were unaffected. The release of lactate dehydrogenase from lysed neutrophils increased sharply at NSS concentrations higher than 10% but remained stable in PBS-exposed cells. All neutrophils isolated from NSS oral rinses were lysed, while a mean of 78% of neutrophils from PBS rinses showed normal morphologic structure. CONCLUSIONS: Nasal saline spray with BKC is toxic to human neutrophils even at concentrations far lower than those found in commercially available preparations. Saline solutions without BKC appear to be safer alternatives, and additional studies are needed to determine the clinical significance of these findings.