Pain at the Cochlear Implant Site Requiring Device Removal in Pediatric Patients.

OBJECTIVES: Idiopathic pain at the cochlear implant (CI) site outside of the immediate postoperative period is an uncommon occurrence but may necessitate device explantation. Our objective was to describe the clinical course for pediatric patients with CI site pain who ultimately required device explantation. STUDY DESIGN: Retrospective chart review. METHODS: We performed a retrospective database review of CIs performed at a tertiary referral center for pediatric cochlear implantation. We specifically evaluated pediatric patients who presented with pain at or near the CI device site and ultimately required explantation. RESULTS: Fifteen patients (16 CIs) had pain at or near the CI site requiring device explantation. Cultures taken during site exploration or device explantation identified bacteria in 86% and 81% of procedures, respectively. Propionibacterium acnes and Staphylococcus non-aureus were the most commonly identified organisms. CONCLUSIONS: The majority of patients with idiopathic pain in this cohort ultimately requiring CI explantation had chronic bacterial colonization. LEVEL OF EVIDENCE: 4 (Case series) Laryngoscope, 132:2044-2049, 2022.

Common genetic susceptibility loci link PFAPA syndrome, Behçet's disease, and recurrent aphthous stomatitis.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. The disease appears to cluster in families, but the pathogenesis is unknown. We queried two European-American cohorts and one Turkish cohort (total n = 231) of individuals with PFAPA for common variants previously associated with two other oropharyngeal ulcerative disorders, Behçet's disease and recurrent aphthous stomatitis. In a metaanalysis, we found that a variant upstream of IL12A (rs17753641) is strongly associated with PFAPA (OR 2.13, P = 6 × 10-9). We demonstrated that monocytes from individuals who are heterozygous or homozygous for this risk allele produce significantly higher levels of IL-12p70 upon IFN-γ and LPS stimulation than those from individuals without the risk allele. We also found that variants near STAT4, IL10, and CCR1-CCR3 were significant susceptibility loci for PFAPA, suggesting that the pathogenesis of PFAPA involves abnormal antigen-presenting cell function and T cell activity and polarization, thereby implicating both innate and adaptive immune responses at the oropharyngeal mucosa. Our results illustrate genetic similarities among recurrent aphthous stomatitis, PFAPA, and Behçet's disease, placing these disorders on a common spectrum, with recurrent aphthous stomatitis on the mild end, Behçet's disease on the severe end, and PFAPA intermediate. We propose naming these disorders Behçet's spectrum disorders to highlight their relationship. HLA alleles may be factors that influence phenotypes along this spectrum as we found new class I and II HLA associations for PFAPA distinct from Behçet's disease and recurrent aphthous stomatitis.

Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome.

OBJECTIVE: To evaluate the long-term efficacy of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome. DESIGN: Prospective case series. SETTING: Tertiary care pediatric hospital. PATIENTS: Pediatric patients meeting the criteria for PFAPA syndrome. INTERVENTIONS: Tonsillectomy with adenoidectomy. MAIN OUTCOME MEASURES: Resolution of PFAPA symptoms. RESULTS: A total of 124 patients (75 boys and 49 girls) underwent adenotonsillectomy from 2004 to 2011 for relief of cyclical fevers due to PFAPA syndrome. Of the 124 patients, 22 did not meet criteria for inclusion in this study because (1) they had less than 6 months of follow-up after surgery or (2) they were unavailable for follow-up; therefore, 102 patients were included in the study. The mean age at the time of surgery was 58 months (range, 18-179 months). The average duration of follow-up after adenotonsillectomy was 43 months (range, 6-98 months). Of 102 patients, 99 had complete resolution of their symptoms immediately after surgery. CONCLUSIONS: Our findings showed complete resolution of symptoms in 99 of 102 patients with PFAPA syndrome who were treated surgically. Patients who meet the clinical criteria for PFAPA syndrome should be offered tonsillectomy and adenoidectomy as part of their treatment options.