Cornelia de Lange syndrome: What every otolaryngologist should know.

Cornelia de Lange Syndrome (CdLS) can be expressed in multiple organ systems requiring a variety of specialists, including pediatric otolaryngology. We present the case of a 20-month-old boy with CdLS actively managed by an aerodigestive team consisting of pediatric otolaryngology, pediatric pulmonology, pediatric gastroenterology, with support staff from audiology, speech, and nutrition. His presentation included mixed hearing loss, dysphagia, microaspiration, gastroesophageal reflux, and failure to thrive. We submit this challenging case of CdLS with a review of the literature to focus specific attention on the otolaryngic manifestations of the syndrome and to discuss the benefits of a multidisciplinary approach to these unique patients.

Use of a Novel Airway Kit and Simulation in Resident Training on Emergent Pediatric Airways.

Objective Development of a novel pediatric airway kit and implementation with simulation to improve resident response to emergencies with the goal of improving patient safety. Methods Prospective study with 9 otolaryngology residents (postgraduate years 1-5) from our tertiary care institution. Nine simulated pediatric emergency airway drills were carried out with the existing system and a novel portable airway kit. Response times and time to successful airway control were noted with both the extant airway system and the new handheld kit. Results were analyzed to ensure parametric data and compared with t tests. A Bonferroni adjustment indicated that an alpha of 0.025 was needed for significance. Results Use of the airway kit significantly reduced the mean time of resident arrival by 47% ( P = .013) and mean time of successful intubation by 50% ( P = .007). Survey data indicated 100% improved resident comfort with emergent airway scenarios with use of the kit. Discussion Times to response and meaningful intervention were significantly reduced with implementation of the handheld airway kit. Use of simulation training to implement the new kit improved residents' comfort and airway skills. This study describes an affordable novel mobile airway kit and demonstrates its ability to improve response times. Implications for Practice The low cost of this airway kit makes it a tenable option even for smaller hospitals. Simulation provides a safe and effective way to familiarize oneself with novel equipment, and, when possible, realistic emergent airway simulations should be used to improve provider performance.

Identifying predictive factors for long-term complications following button battery impactions: A case series and literature review.

OBJECTIVES: To complement a case series review of button battery impactions managed at our single military tertiary care center with a thorough literature review of laboratory research and clinical cases to develop a protocol to optimize patient care. Specifically, to identify predictive factors of long-term complications which can be used by the pediatric otolaryngologist to guide patient management after button battery impactions. METHODS: A retrospective review of the Department of Defense's electronic medical record systems was conducted to identify patients with button battery ingestions and then characterize their treatment course. A thorough literature review complemented the lessons learned to identify potentially predictive clinical measures for long-term complications. RESULTS: Eight patients were identified as being treated for button battery impaction in the aerodigestive tract with two sustaining long-term complications. The median age of the patients treated was 33 months old and the median estimated time of impaction in the aerodigestive tract prior to removal was 10.5 h. Time of impaction, anatomic direction of the battery's negative pole, and identifying specific battery parameters were identified as factors that may be employed to predict sequelae. CONCLUSION: Based on case reviews, advancements in battery manufacturing, and laboratory research, there are distinct clinical factors that should be assessed at the time of initial therapy to guide follow-up management to minimize potential catastrophic sequelae of button battery ingestion.

Delayed Facial Nerve Palsy After Open Reduction of an Isolated Zygomaticomaxillary Complex Fracture.

Facial nerve paralysis is a devastating complication which can occur after a variety of otolaryngic procedures, including facial trauma repair. The frontal and marginal branches are most often placed at risk. However, facial nerve paralysis is not typically described as a risk in most uncomplicated facial trauma repairs of the zygomaticomaxillary complex (ZMC). In particular, buccal branch injury has not been described in a delayed setting following repair of the ZMC. The authors present a patient of delayed buccal branch paralysis following a simple ZMC repair which has not been previously reported.The diagnosis, clinical course, and management strategies for delayed facial nerve paralysis in the setting of a ZMC repair are discussed. This rare complication after facial trauma repair should be discussed with patients as a possible complication.

Congenital stapes malformation: Rare conductive hearing loss in a patient with Waardenburg syndrome.

Waardenburg syndrome is a known autosomal dominant cause of congenital hearing loss. It is characterized by a distinctive phenotypic appearance and often involves sensorineural hearing loss. Temporal bone abnormalities and inner ear dysmorphisms have been described in association with the disease. However, middle ear abnormalities as causes of conductive hearing loss are not typically seen in Waardenburg syndrome. We discuss a case of an 8-year-old female who meets diagnostic criteria for Waardenburg syndrome type 3 and who presented with a bilateral conductive hearing loss associated with congenital stapes fixation. We discuss management strategy in this previously unreported phenotype.

Sublingual Immunotherapy for Allergic Fungal Sinusitis.

Allergic fungal sinusitis (AFS) is a condition that has an allergic basis caused by exposure to fungi in the sinonasal tract leading to chronic inflammation. Despite standard treatment modalities, which typically include surgery and medical management of allergies, patients still have a high rate of recurrence. Subcutaneous immunotherapy (SCIT) has been used as adjuvant treatment for AFS. Evidence exists to support the use of sublingual immunotherapy (SLIT) as a safe and efficacious method of treating allergies, but no studies have assessed the utility of SLIT in the management of allergic fungal sinusitis. A record review of cases of AFS that are currently or previously treated with sublingual immunotherapy from 2007 to 2011 was performed. Parameters of interest included serum IgE levels, changes in symptoms, Lund-McKay scores, decreased sensitization to fungal allergens associated with AFS, and serum IgE levels. Ten patients with diagnosed AFS were treated with SLIT. No adverse effects related to the use of SLIT therapy were identified. Decreases in subjective complaints, exam findings, Lund-McKay scores, and serum IgE levels were observed. Thus, sublingual immunotherapy appears to be a safe adjunct to the management of AFS that may improve patient outcomes.

Isolated adult lymphadenopathy: a rare presentation of Langerhans cell histiocytosis.

INTRO: Langerhans cell histiocytosis (LCH) is a rare disease involving the proliferation of histiocytes in one or more organ systems. The presentation of LCH is more common in the pediatric population and rarely occurs within the adult population. Isolated lymph node involvement is an extremely rare presentation of the disease. The authors present a case of isolated lymph node LCH in an otherwise healthy adult female and discuss management strategies for these unusual findings. METHODS: A case report of a patient at a tertiary care facility in June 2014 is discussed. RESULTS/DISCUSSION: The diagnosis, clinical course, and management strategies for isolated adult LCH are discussed and reviewed. CONCLUSION: Langerhans cell histiocytosis rarely presents with isolated lymph node involvement in adults. Management and surveillance algorithms are discussed in this unusual case of seemingly benign lymphadenopathy.

Hypoharyngeal injury in an infant from non-accidental trauma.

Non-accidental trauma is a sad but commonly described mechanism of injury in the pediatric literature. However, the otolaryngologist infrequently encounters the initial presentation of non-accidental trauma despite the fact that a significant percentage of injuries take place in the head and neck. This case report seeks to discuss otolaryngologic presentations of non-accidental trauma in the pediatric population as well as to discuss management strategies. The case of a 3 month old female with a hypopharyngeal injury and esophageal perforation is presented and discussed.

Effects of aging on current vocalization threshold in mice measured by a novel nociception assay.

BACKGROUND: Age-related changes in nociception have been extensively studied in the past decades. However, it remains unclear whether in addition to the increased incidence of chronic illness, age-related changes in nociception contribute to increased prevalence of pain in the elderly. Although a great deal of evidence suggests that nociception thresholds increase with aging, other studies yield disparate results. The aim of this investigation was to longitudinally determine the effect of aging on nociception. METHODS: The authors developed a nociception assay for mice using electrical stimuli at 2,000, 250, and 5 Hz that reportedly stimulate Abeta, Adelta, and C sensory nerve fibers, respectively. A system was designed to automate a method that elicits and detects pain-avoiding behavior in mice. Using a Latin square design, the authors measured current vocalization thresholds serially over the course of mice's life span. RESULTS: For 2,000-Hz (Abeta), 250-Hz (Adelta), and 5-Hz (C) electrical stimuli, current vocalization thresholds first decreases and then increases with aging following a U-shaped pattern (P < 0.001). In addition, average current vocalization thresholds at youth and senescence are significantly higher than those at middle age for the 250-Hz (Adelta) and 5-Hz (C fiber) electrical stimulus (P < 0.05). CONCLUSIONS: Using a novel and noninjurious nociception assay, the authors showed that over the life span of mice, current vocalization threshold to electrical stimuli changes in a U-shaped pattern. The findings support the notion that age-related changes in nociception are curvilinear, and to properly study and treat pain, the age of subjects should be considered.