Transorbital Resection of an Optic Nerve Glioma with Severe Proptosis: Technical Note.

Abstract

Background and Importance Optic nerve gliomas (ONGs) are benign and slow-growing tumors with a variable clinical course that may result in significant morbidity. The variability in tumor burden, challenging anatomy, and associated symptomatology require an individualized and multidisciplinary approach. The relative paucity of cases and variation in surgical anatomy prompt the need for accrual of cases in literature to advance the understanding of surgical techniques to address this rare clinical entity. Clinical Presentation A 19-year-old patient presented with severe and globe-threatening left eye proptosis, negatively affecting quality of life, due to a sporadic intraorbital ONG that had been inexorably growing over the previous 9 years, despite chemotherapy. The patient was taken to the operating room for a single-stage globe-sparing transorbital resection of the intraorbital ONG. The surgical plan consisted of an endoscopic endonasal approach for medial and inferior orbital wall decompression combined with an open lateral orbitotomy, via a lateral canthal incision, for subtotal piecemeal tumor resection to relieve the proptosis, restore alignment of the globe, and yield tissue for molecular analysis. The patient experienced profound improvement in quality of life and opted for radiographic surveillance that has been stable at 5 years. Discussion and Conclusion Surgical approaches to intraorbital tumors have included both transcranial and/or transorbital routes, with the latter more recently encompassing endoscopic endonasal techniques. This report presents the use of a novel combination of transorbital techniques for resection of an intraorbital ONG with severe proptosis, including an endoscopic endonasal approach combined with an open lateral orbitotomy.