Otolaryngologic manifestations of klippel-feil syndrome in children

IMPORTANCE: Children with Klippel-Feil syndrome (KFS), characterized principally by abnormal fusion of 2 or more cervical vertebrae, may have many additional congenital anomalies. The overall prevalence of otolaryngologic manifestations among patients with KFS has not been previously characterized. OBJECTIVE: To define the otolaryngologic diagnoses made and procedures performed in95 patients with KFS, which, to our knowledge, is the largest series of this challenging patient population published to date. DESIGN, SETTING, AND PARTICIPANTS: For this retrospective review, all patients with KFS who underwent otolaryngology consultation at our institution over a 26-year period (January 1989 to December 2015) were included. Patients were identified using International Classification of Diseases, Ninth Revision (ICD-9) codes and were confirmed through individual medical record review. Relevant otolaryngologic diagnoses and procedures were extracted using ICD-9 and Current Procedural Terminology codes, respectively. Selected demographics included age, sex, number of clinic visits, and number of procedures. MAINOUTCOMES ANDMEASURES: The primary outcomes were the otolaryngologic diagnoses and procedures associated with the KFS patient population; the secondary outcome was Cormack-Lehane classification documented during airway procedures. RESULTS: Overall, 95 patients with KFS were included in this study (55 males [58%] and 40 females [42%]); mean (range) age at time of presentation to the otorhinolaryngology clinic was 5.8 (birth-23.0) years. Each patient with KFS averaged 8 visits to the otorhinolaryngology office and 5 otolaryngologic diagnoses. The most common diagnosis was conductive hearing loss (n = 49 [52%]), followed by sensorineural hearing loss (n = 38 [40%]), and dysphagia (n = 37 [39%]). Sixty-two (65%) patients underwent otolaryngologic procedures, with 44 (46%) undergoing multiple procedures. The most common procedure was tympanostomy tube placement (n = 36 [38%]), followed by office flexible endoscopy (n = 23 [24%]). Twelve of the20 patients who underwent direct laryngoscopy had documented Cormack-Lehane classification; 5 of 12 patients (42%) had a compromised view (grade 2, 3, or 4) of the larynx. Three patients required tracheotomies at this institution for airway stabilization purposes; each had severe upper airway obstruction leading to respiratory failure. CONCLUSIONS AND RELEVANCE: Patients with KFS require consultation for a variety of otolaryngologic conditions. Among these, hearing loss is the most common, but airway issues related to cervical spine fusion are the most challenging. Formulating an appropriate care plan in advance is paramount, even for routine otolaryngology procedures

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient

OBJECTIVE To provide recommendations for the workup of hearing loss in the pediatric patient. METHODS Expert opinion by the members of the International Pediatric Otolaryngology Group. RESULTS Consensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss. CONCLUSION The workup of children with hearing loss can be guided by the recommendations provided herei

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient

Objective To provide recommendations for the workup of hearing loss in the pediatric patient. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group. Results Consensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss. Conclusion The workup of children with hearing loss can be guided by the recommendations provided herein

International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations

Objective To provide recommendations for the comprehensive management of young infants who present with signs or symptoms concerning for laryngomalacia. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results Consensus recommendations include initial care and triage recommendations for health care providers who commonly evaluate young infants with noisy breathing. The consensus statement also provides comprehensive care recommendations for otolaryngologists who manage young infants with laryngomalacia including: evaluation and treatment considerations for commonly debated issues in laryngomalacia, initial work-up of infants presenting with inspiratory stridor, treatment recommendations based on disease severity, management of the infant with feeding difficulties, post-surgical treatment management recommendations, and suggestions for acid suppression therapy. Conclusion Laryngomalacia care consensus recommendations are aimed at improving patient-centered care in infants with laryngomalacia

International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations

Objective To provide recommendations for the comprehensive management of young infants who present with signs or symptoms concerning for laryngomalacia. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results Consensus recommendations include initial care and triage recommendations for health care providers who commonly evaluate young infants with noisy breathing. The consensus statement also provides comprehensive care recommendations for otolaryngologists who manage young infants with laryngomalacia including: evaluation and treatment considerations for commonly debated issues in laryngomalacia, initial work-up of infants presenting with inspiratory stridor, treatment recommendations based on disease severity, management of the infant with feeding difficulties, post-surgical treatment management recommendations, and suggestions for acid suppression therapy. Conclusion Laryngomalacia care consensus recommendations are aimed at improving patient-centered care in infants with laryngomalacia

ICAR: endoscopic skull‐base surgery

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Opioid prescribing practices following elective surgery in Otolaryngology-Head & Neck Surgery

Abstract Background Prescription opioid abuse has become a major issue across the world and especially in North America. Canada has the second highest number of opioid prescriptions per capita in the world, second only to the United States, with numbers continuing to rise in recent years. Surgeons play a critical role in this discussion as they are responsible for the management of post-operative pain in their patients. The objective of this study is to evaluate the opioid prescribing practices of Otolaryngologists-Head and Neck Surgeons in Canada and determine factors that may influence these practices. Methods The online survey was distributed to members of the Canadian Society of Otolaryngology-Head and Neck Surgery. Questions surveyed the respondents’ demographics and opioid prescribing practices for common pediatric and adult elective surgeries. Results The survey was sent to 670 surgeons and trainees and 121 responses were received (18%). There was representation across all subspecialties with a mix of community and academic surgeons. The most commonly prescribed opioid was Codeine/Acetaminophen, 48.2% (n = 53), in the adult population, and Morphine, 47.1% (n = 41), in the pediatric population. The median total oral morphine equivalents prescribed across all adult surgeries was 123.75 mg (24.75 doses). The surgery with the highest oral morphine equivalents prescribed was tonsillectomy/adenoidectomy for both adult and pediatric patients, with a median of 150 mg (30 doses) for adults and 4.5 mg/kg (23 doses) for pediatrics. Gender, training years, year in residency, or reported level of conservatism did not predict the dose prescribed to either adult or pediatric patients. Due to the relatively low response rate, the generalizability of these results is unclear. Conclusions Our study demonstrates a wide variability in opioid prescriptions across procedures and within each individual procedure. This variability reflects the lack of guidelines available for post-operative opioid prescribing and suggests that some Otolaryngologists may be prescribing higher doses of opioids than required. Opportunities for improving patient safety and resource stewardship regarding optimal prescribing practices should be explored