Periodic fever and pharyngitis in young children -: A new disease for the otolaryngologist?

Objective: A clinical entity consisting of periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis termed "PFAPA syndrome" in young children (<5 years old) may be unfamiliar to otolaryngologists. We present our 5-year experience of PFAPA syndrome. Design: Case series. Setting: Tertiary academic. Patients: A 5-year retrospective chart review for children (<5 years old) who have undergone tonsillectomies with and without adenoidectomies was conducted. Medical records from subjects who underwent the procedures for recurrent pharyngitis were reviewed with reference to a history of periodic fever and stomatitis associated with pharyngitis. Interventions: Tonsillectomy with and without adenoidectomy. Main Outcome Measure: The objective measure was a comparison of the number of visits to the primary care physician for pharyngitis associated with fever in a 3-month period before and after the surgical intervention. The subjective measure was a telephone interview evaluating preoperative and postoperative symptoms. Results: Of the 117 patients identified, 22 (19%) underwent surgery for recurrent pharyngitis. Five subjects (average age, 2.5 years) were identified as having PFAPA syndrome. The average number of preoperative PFAPA-related complaints was 11.6 compared with 0.2 for the number of postoperative PFAPA-related complaints (P=.03). Conclusions: Our experience suggests that PFAPA syndrome is an uncommon disease. Most of these children have undergone workup(s) for sepsis performed by their pediatricians because of the associated high fever. The clinical history of this cohort was quite distinctive. This small sample suggests a significant decrease if not cessation of pharyngitis following surgical intervention.