Comprehensive preoperative staging system for endoscopic single and multicorridor approaches to juvenile nasal angiofibromas

BACKGROUND: Juvenile nasal angiofibromas (JNA) is a benign lesion with high vascularity and propensity of bone erosion leading to skull base invasion and intracranial extension. It is known to involve multiple compartments, which are often surgically difficult to access. With evolution in surgical expertise and technical innovations, endoscopic and endoscopic-assisted management has become the preferred choice of surgical management. Over the last four decades, various staging systems have been proposed, which are largely based on the extent of nasal angiofibroma. However, no clear guidelines exist for the stage-appropriate surgical management. In this study, we aim to formulate a novel staging system based on the analysis of high quality preoperative imaging and propose detailed surgical guidelines related to disease stages as observed in 242 primary cases of JNA. METHODS: A retrospective analysis of the case records of 242 primary JNA cases was performed at our center. Patients were staged according to various existing staging systems as well as our own new staging system, and outcome variables were compared with respect to intraoperative blood loss, multiple staged operations, and tumor recurrences. Operative records were studied and precise endoscopic surgical guidelines were formulated for each stage. RESULTS: Comparing the intraoperative blood loss seen in stages of various classifications, it was found that intraoperative blood loss correlated best and statistically significantly with stages in the newly proposed Janakiram staging system when compared to the existing staging systems. Staged operations were performed in a total of 7/242 patients, and there was a significant association between the requirement of a staged operation and tumor extent (Fischer's exact test, P < 0.001). Tumor recurrence was seen in 22 cases and the pterygoid wedge was found to be the most frequent site of recurrence initially. As the extent of resection improved with better surgical technique over time, recurrences were only found in superior orbital fissure, around the internal carotid artery, and in the middle cranial fossa. CONCLUSION: This new Janakiram staging system is based on preoperative imaging data from one of the largest JNA case series reported thus far. Respective guidelines reliably stratify patients into treatment groups with definite surgical approaches and predicts outcome. Improved surgical approaches in the modern endoscopic era have redefined JNA management with improved outcome. This study shows the importance of precise presurgical imaging and the choice of the most suitable surgical approach in reducing morbidity and mortality in JNA surgery.