Faculty Bibliography

Currently, mal de débarquement syndrome (MdDS) has been reported only among adults. This case series describes three pediatric MdDS patients. MdDS presentation in children is similar to that of adults, although frequency of comorbid conditions is greater. Diagnostic delays are common and likely due to under recognition of MdDS among children.

BACKGROUND:Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a clinical syndrome of unclear etiology. PFAPA has generally been considered a non-hereditary fever syndrome; however, this has been called into question with recent reports of family clustering. Few reports have been published describing siblings with PFAPA. To our knowledge, this is the first report of siblings with near simultaneous onset of disease followed by synchronous disease flares. CASE PRESENTATION/METHODS:We describe the case of near simultaneous onset of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis in siblings followed by synchronous disease flares of clear frequency and nearly identical character. Flares were characterized predominantly by fever, aphthous ulceration, cervical lymphadenitis, and the absence of infection. The fever episodes demonstrated a robust response to glucocorticoids and recurred in the same staggered manner every four weeks, with complete absence of symptoms and normal growth and development between episodes. Nine months after onset, the older sibling, a 5-year-old female, underwent tonsillectomy resulting in dramatic resolution of episodes. At the same time, her 2-year-old sister experienced resolution of her fever episodes, though she did not undergo tonsillectomy herself. CONCLUSION/CONCLUSIONS:This is an unusual case of simultaneous onset PFAPA followed by synchronous disease flares. PFAPA is an uncommon clinical syndrome, and it is rarely diagnosed in siblings. The etiology of PFAPA remains unclear. Though the disease is classically considered sporadic, there is a growing body of evidence to suggest that PFAPA may be heritable.

OBJECTIVE/UNASSIGNED:This report describes a new observation of hyperglycemia in a child with Type 1 diabetes after off-label use of otic ciprofloxacin/dexamethasone drops in the nasal passage and reviews previous reports of adverse endocrine effects from intranasal corticosteroids in pediatric patients. METHODS/UNASSIGNED:We describe the clinical case and conducted a literature review of MEDLINE (PubMed) and EMBASE. RESULTS/UNASSIGNED:A 9-month-old female with a history of Type 1 diabetes who underwent unilateral choanal atresia repair was started on 1 week of ciprofloxacin 0.3%/dexamethasone 0.1% otic drops twice a day for choanal obstruction with granulation tissue. While the patient's airway patency improved, average daily blood glucose increases by 40 to 50 points were noted on the patient's continuous glucose monitor. The hyperglycemia resolved within 2 days after switching to mometasone furoate 0.05% spray. We also review 21 pediatric otolaryngology cases of iatrogenic Cushing's syndrome associated with on- and off-label use of topical steroid suspensions in the airway. Patients ranged from 3 months to 16 years in age and used doses of 50 μg/day to 2 mg/day. CONCLUSION/UNASSIGNED:This is the first reported pediatric case of increased blood glucose levels associated with intranasal steroid suspensions, to the best of our knowledge. Counseling families on precise dose administration and potential endocrine disturbances is critical when prescribing these medications for off-label use in infants and small children, particularly among patients with underlying endocrine disorders such as diabetes.

BACKGROUND:Pediatric otolaryngologists have seen an increased focus on upper lip frenum as a possible culprit for feeding difficulties and the development of maxillary midline diastema (MMD). This increase may be encouraged by parents' exposure to medical advice over the internet about breastfeeding and potential long-term aesthetic concerns for their children. Subsequently, there has been increased pressure on pediatric otolaryngologists to perform superior labial frenectomies. There has been a reported 10-fold increase in frenectomies since the year 2000. However, there is no consensus within the literature regarding the benefit of superior labial frenectomy in preventing midline diastema. OBJECTIVE:To provide physicians and parents with the most updated information by systematically reviewing the available literature for the association between superior labial frenum and midline diastema. METHODS:A literature search was performed in MEDLINE (PubMed), EMBASE, Web of Science, the Cochrane Library and Dental and Oral Sciences Source (DOSS). Using the Covidence platform, a systematic review was conducted. The initial 314 articles identified underwent systematic review and 11 studies were included in the final review. RESULTS/DISCUSSION/CONCLUSIONS:Available data, primarily from the dental literature, showed that two subtypes of frenum: papillary and papillary penetrating frenum, are associated with maxillary midline diastema. Superior labial frenectomy should be delayed until permanent lateral incisors have erupted, as this can spontaneously close the physiological MMD. Current literature recommends against frenectomy before addressing the diastema with orthodontics, which helps to prevent diastema relapse. It is also imperative to rule out other odontogenic and oral cavity causes of diastema, such as thumb sucking, dental agenesis, and other causes. Online information may not always be fully representative and should be interpreted in the full context of the patient's medical history before referral for surgical intervention.

IMPORTANCE/OBJECTIVE:Foreign body (FB) aspiration into the airway is a significant cause of pediatric morbidity and mortality, yet the clinical presentation is diverse and dynamic. There are conflicting recommendations which pre-procedural findings support performing a bronchoscopy, the gold standard for diagnosis and removal of FBs, however a procedure that entails general anesthesia and possible risks. OBJECTIVE:Decision whether to proceed to a bronchoscopy may be challenging. Our goal was to enhance decision-making by analyzing the diagnostic values of the different pre-procedural findings in this setting. DATA SOURCES/METHODS:A comprehensive search was performed in PUBMED, EMBASE and Cochrane Review databases to find studies from the last 19 years that reported pre-procedural history, physical examination and radiological findings in patients who had bronchoscopies. STUDY SELECTION/METHODS:Studies were included of pediatric populations if they contained bronchoscopy results (positive and negative for foreign body) with a breakdown according to pre-intervention findings. DATA EXTRACTION AND SYNTHESIS/METHODS:Titles and abstracts retrieved from our search were screened. Thereafter, full-texts were carefully reviewed and selected for inclusion if the aforementioned criteria were met. PRISMA guidelines for systematic review and meta-analyses were followed. MAIN OUTCOME(S) AND MEASURE(S)/UNASSIGNED:Cumulative weighted prevalence, sensitivity, specificity, positive and negative predictive values of each pre-procedural finding were calculated, as well as for the "classic triad" (history of an acute event, wheezing, and unilateral decreased breath sounds). Calculation for other combinations of findings, or optimally, constructing a weighted score based on all the findings for each specific patient were not possible to perform, as the specific data breakdown is rarely reported. RESULTS:Fifteen studies met inclusion criteria, totaling 5606 patients who underwent bronchoscopies. All studies but one were single center based and all except one were retrospective. No single finding has both positive and negative predictive values over 50%. The "classic triad" has 90% specificity, however only 35% sensitivity. CONCLUSIONS:The data is very heterogeneous with regard to pre-procedural findings and how best to guide treatment according to them. This meta-analysis provides cumulative weighted metrics for each finding, to optimize decision-making for the individual patient. Future reporting of data should be enhanced, so that combinations of findings for a specific patient can be used to optimize management. LEVEL OF EVIDENCE/METHODS:4.

OBJECTIVE:Laryngopharyngeal and Gastroesophageal reflux (LPR and GER) are distinct clinical entities that present with a range of non-specific symptoms. The exact prevalence in the pediatric population is unknown. While there has been an increase in the use of PPIs, lack of clear guidelines, conflicting evidence regarding efficacy and safety concerns with long-term use require physicians to use their own anecdotal experience and clinical judgement when treating patients. The goal of this study was to evaluate practice patterns among pediatric otolaryngologists regarding the use of proton-pump inhibitors for reflux-related conditions. METHODS:A survey was submitted to American Society of Pediatric Otolaryngology (ASPO) members to determine practice patterns regarding use of PPIs for reflux-related conditions in the newborn and infant population. Statistical analysis using Fisher's exact test was performed. RESULTS:37% of respondents would not prescribe PO PPIs in neonates, with 50% not prescribing IV PPIs. 60% would prescribe a PPI as second or third-line treatment for infants (10 weeks to 1-year). Only 10% would prescribe as first-line in this age group. 48% would prescribe PPIs once daily and 19% as BID. No significant practice differences exist based on years of experience, number of relevant patients seen, and setting of practice. CONCLUSION/CONCLUSIONS:There was no agreement regarding dosage, frequency and duration of PPI treatment for reflux disease in neonates and infants. There was also no correlation with experience or practice setting. This emphasizes the need for a multidisciplinary approach and consensus statement to guide management of GER and LPR in this population.

OBJECTIVE:Review otolaryngology literature for awareness of neurotoxicity from general anesthesia in children. Recently, there has been increasing focus in anesthesia literature on the long-term effects of general anesthesia on neurodevelopment. Multiple animal models have demonstrated evidence of neurotoxicity from both inhalational and intravenous anesthetics. Cohort studies also have revealed modestly increased risk of adverse neurodevelopmental outcomes in children exposed to a single episode of general anesthesia prior to 3 to 4 years of age, with stronger evidence for multiple exposures in this age range. Otolaryngologists may subject children to general anesthesia via procedures or tests, including computed tomography, magnetic resonance imaging, and auditory brainstem response. DATA SOURCES:PubMed, Embase, Scopus, and Web of Science Review. METHODS:A scoping review using the above databases was performed limited to January 2005 through December 2015. Articles were screened and reviewed based on predefined inclusion and exclusion criteria. RESULTS:Initial search generated 3,909 articles. After 72 full text articles were reviewed, only seven articles mentioned neurotoxicity as a risk of general anesthesia in pediatric patients. CONCLUSION:Despite the high volume of pediatric otolaryngologic procedures performed annually, there remains limited awareness in our literature discussing neurotoxicity as an outcome. Prospective data from anesthesia literature is still pending; therefore, specific recommendations cannot be made at this time. Otolaryngologists should be aware of the concerns and work toward defining elective procedures, combining surgical procedures with other procedures or imaging, and reassessing the timing and frequency of various interventions under general anesthesia in young children. Laryngoscope, 127:1930-1937, 2017.