Recurrent Pediatric Orbitofacial Plexiform Neurofibroma With Cavernous Sinus Extension: Craniofacial Debulking and Eyelid-Sparing Exenteration

Advanced pediatric orbitofacial plexiform neurofibroma may require coordinated decisions regarding globe sacrifice, cranial-base protection, soft-tissue coverage, and future rehabilitation. We describe a 5-year-old girl with recurrent orbitofacial plexiform neurofibroma involving the entire orbit, optic nerve, extraocular muscles, multiple cranial nerves, cavernous sinus, and deep craniofacial soft tissues. Management included craniofacial debulking, eyelid-sparing orbital exenteration, intentional residual disease in high-risk compartments, immediate orbital roof/temporal cranial-base reconstruction, and primary closure using the preserved native eyelid-facial envelope. At 12-month follow-up, compressive orbital pain remained resolved, and facial contour was improved, with no clinical evidence of progression. Follow-up MRI demonstrated marked reduction in tumor volume compared with preoperative imaging and persistent residual disease involving the left cavernous sinus.