Undifferentiated recurrent fevers in pediatrics are clinically distinct from PFAPA syndrome but retain an IL-1 signature.

Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined hereditary fever disorders, many patients lack a clear diagnosis. Many pediatric patients are often grouped with patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome despite failing to meet diagnostic criteria.

Immune Dysregulation in the Tonsillar Microenvironment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome.

Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical adenitis, and leukocytosis. While the mechanism is unclear, previous studies have shown that tonsillectomy can be a therapeutic option with improvement in quality of life in many patients with PFAPA, but the mechanisms behind surgical success remain unknown. In addition, long-term clinical follow-up is lacking.

Airway characteristics of infants with Pierre Robin sequence who undergo mandibular distraction osteogenesis.

Newborn upper airway obstruction secondary to micrognathia and Pierre Robin sequence can be managed with conservative and surgical interventions. Mandibular distraction osteogenesis has been used to relieve micrognathia associated with severe airway obstruction. We conducted a retrospective chart review to identify patients with Pierre Robin sequence who underwent mandibular distraction osteogenesis during a 2-year period.

The long-term effects of mandibular distraction osteogenesis on developing deciduous molar teeth.

Background. Many studies have demonstrated the effectiveness of mandibular distraction osteogenesis (MDO) in alleviating the micrognathia-associated upper airway obstruction but very few studies have focused on long-term dental outcomes. Objective.

Tracheostomy versus mandibular distraction osteogenesis in Canadian children with Pierre Robin sequence: a comparative cost analysis.

Introduction: Upper airway obstruction in newborns with Pierre Robin sequence (PRS) may be severe enough to require a surgical intervention. Tracheostomy has been the traditional gold standard, but mandibular distraction osteogenesis (MDO) has been proven to be an effective alternative procedure.

Objective: The objective of the present study was to conduct the first comparative cost analysis between tracheostomy and MDO in Canada.

Mandibular distraction osteogenesis in children with Pierre Robin sequence: impact on health-related quality of life.

Background: Upper airway obstruction in children with Pierre Robin sequence (PRS) may be severe enough to require surgical intervention. Although many studies have demonstrated the effectiveness of mandibular distraction osteogenesis (MDO) in relieving airway obstruction, no study has reported health-related quality of life (QOL) outcomes. The objective of the present study was to conduct the first health-related QOL assessment post-MDO.

Work environment discomfort and injury: an ergonomic survey study of the American Society of Pediatric Otolaryngology members.

Background: Workplace-related musculoskeletal pain has been studied in various occupations, but it is rarely reported in the surgical literature.

Objective: The aim of this study was to examine work-related discomfort and injury among pediatric otolaryngologists and to assess their knowledge of workplace ergonomic principles.

Methods: We surveyed current North American members of the American Society of Pediatric Otolaryngology.

Chronic esophageal foreign bodies and secondary mediastinitis in children.

Objectives: The purpose of this study was to review the clinical presentation, diagnosis, and management of chronic esophageal foreign bodies complicated by mediastinitis in children.

Methods: A retrospective study of children with a chronic esophageal foreign body and secondary mediastinal complications diagnosed at Rady Children's Hospital in San Diego over a 12-month period is reported.

Results: Three patients received a diagnosis of an esophageal foreign body, retained from 1 to 12 months, and mediastinitis.

Nasal dermoids.

Purpose Of Review: Midline congenital nasal lesions are rare, occurring in one out of every 20,000-40,000 births. Of these midline lesions, nasal dermoids are the most common. This review centers on diagnosis of nasal dermoids, the role of imaging in diagnosis and surgical planning and the various approaches to surgical management of these lesions.

Professional diversity and personal commitments of pediatric otolaryngologists.

Objective: To characterize the time demands and practice patterns of pediatric otolaryngologists.

Design: Prospective survey of members from the American Society of Pediatric Otolaryngology.

Results: The survey response rate was 54% (n = 136) of practicing members of the American Society of Pediatric Otolaryngology.

The otolaryngological manifestations of mitochondrial disease and the risk of neurodegeneration with infection.

Objective: To report the nature and extent of hearing loss and other otolaryngological problems in patients with mitochondrial disease, and to document the risk of neurodegeneration with infection.

Design: Medical chart review and telephone interview of 40 patients with documented mitochondrial disease.

Setting: An international referral center for the diagnosis and management of mitochondrial disorders.

Imaging and surgical approach of nasal dermoids.

Objective: Determine the most accurate and cost effective radiographic evaluation for nasal dermoids. Determine the best surgical approach for excision of nasal dermoids.

Design: Retrospective chart review.