Multimodal Intraoperative Neurophysiological Monitoring in Neurofibromatosis Type 1-Related Craniofacial Tumor Surgery.

Objective: This study summarized the application effect and clinical experience of multimodal intraoperative neurophysiological monitoring (IONM) technology in the surgery of neurofibromatosis type 1 (NF1)-related craniofacial tumors.

Materials And Methods: A retrospective study was conducted on NF1 patients who were admitted between January 2019 and December 2024 and treated with craniofacial tumor resection surgery assisted by multimodal IONM technology. Data from this study were analyzed regarding sex, age at surgery, surgical procedures, IONM parameters, preoperative and postoperative symptoms, follow-up period, as well as oncological outcome.

Results: Sixty-three NF1 patients (34 male, 29 female; aged 31.1 ± 17.0 y) all successfully underwent surgery. IONM includes MEP and EMG (64 cases), SEP (12 cases), and VEP (19 cases). Sixty-two patients achieved a macroscopically complete resection, except for 1 patient who underwent palliative resection. All 9 cases of MPNST and 4 cases of AN achieved microscopically complete resection, confirmed by free tumor margins. Postoperative neurological deficits include 1 case of new facial paralysis, 5 cases of worsened preoperative facial paralysis, 3 cases of new postoperative hoarseness, and 11 cases of numbness. All but 1 somatic motor deficits were alarmed by IONM. The median length of postoperative follow-up has been 23 months (range: 13-59 mo). During follow-up, no significant changes in neurological symptoms or tumor recurrence were found except in 1 patient with MPNST, a local relapse occurred 25 months after the operation.

Conclusion: The clinical manifestations of NF1-related craniofacial tumors are complex. Multimodal IONM technology can provide real-time detection of nerve provocation and damage. Surgical treatment with multimodal IONM technology can be safe and effective.