Comprehensive Perioperative Management of PFAPA Syndrome: Insights From Clinical Cases

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, a common cause of recurrent fever in childhood, presents a challenge in both diagnosis and management. While initially considered a monogenic disorder, recent research has highlighted its complex genetic underpinnings, involving noncoding genome regions and immune-mediated cytokine dysregulation. This complexity underscores the need for comprehensive perioperative management strategies, particularly in surgical interventions such as tonsillectomy and adenoidectomy. This article presents two cases of PFAPA syndrome undergoing surgical procedures and outlines their perioperative management. Emphasizing meticulous preoperative assessment, including medication adjustments and fasting protocols, underscores the importance of multidisciplinary collaboration in tailoring individualized care plans. In this context, perioperative nurses play a crucial role in caring for pediatric patients diagnosed with PFAPA syndrome, contributing significantly to preoperative preparation, intraoperative monitoring, and postoperative recovery, ensuring patient safety and optimizing outcomes. Furthermore, the article discusses emerging prophylactic and therapeutic modalities, such as colchicine and interleukin-1 inhibitors, offering insights into potential avenues for optimizing patient outcomes. We present our article to improve care for PFAPA patients and contribute to nurses, residents, and anesthesiologists by explaining the basics and sharing our clinical experiences.