Abstract
BACKGROUND: Optic nerve sheath hemangiomas (ONSHs) are rare vascular tumors from the blood vessels surrounding the optic nerve, accounting for <1% of optic tumors and 0.5% of vascular malformations. Although benign, their location near the optic nerve poses a risk of significant nerve damage and vision loss. Symptoms often include visual disturbances, eye pain, and strabismus, making it difficult to make a diagnosis. Advances in imaging, especially magnetic resonance imaging (MRI), have improved early detection and diagnosis. In addition, insights into the molecular mechanisms, including endothelial signaling and angiogenesis, have facilitated the development of more effective treatments, such as targeted therapies and minimally invasive surgical options. CASE DESCRIPTION: We present the case of a 57-year-old woman who experienced progressive left-eye swelling, increased tearing, and declining visual acuity. Imaging studies, including MRI and computed tomography scans, revealed a left intraconal mass suggestive of ONSH. A transcranial-modified orbitofrontal approach was employed for tumor resection due to its proximity to vital structures. Postoperative histopathology confirmed hemangioma. At 1-year follow-up, the patient exhibited significant improvement in visual function and resolution of orbital swelling. CONCLUSION: This case highlights the critical role of surgical intervention in managing ONSHs that threaten visual function and cause mass effects. The transcranial-modified orbitofrontal approach proved effective in providing optimal access for safe tumor resection and improving visual outcomes. Integrating advanced imaging techniques and intraoperative monitoring contributes significantly to enhancing prognosis in ONSH cases.