Surgical Approaches to Optic Nerve Decompression in Osteopetrosis: A Review of Endoscopic Endonasal and Transcaruncular Techniques.

Abstract

Osteopetrosis is a rare genetic disorder characterized by impaired osteoclast function and excessive bone density, often leading to compressive optic neuropathy due to bony overgrowth of the optic canal. Timely surgical intervention is critical for preventing permanent vision loss in affected patients. This review summarizes the available literature on the surgical techniques of endoscopic endonasal optic nerve decompression (EEOND) and the transcaruncular approach in osteopetrosis patients. EEOND is a minimally invasive technique that offers excellent visualization, advanced instrumentation, and access to the optic nerve through the nasal corridor and requires mastering the anatomy of the sphenoid bone to achieve success. However, challenges arise from the dense and brittle nature of the bone in osteopetrosis, complicating the procedure. The transcaruncular approach provides a targeted route to the medial optic canal with minimal external scarring. However, its limited scope may not suffice for extensive decompression in severe cases. Early surgical intervention correlates with better visual outcomes, particularly in pediatric patients who are at higher risk for rapid progression of vision loss. Integrating advanced imaging and hybrid surgical techniques may enhance decompression efficacy. In conclusion, both EEOND and the transcaruncular approach are valuable for managing optic nerve compression in osteopetrosis, each with distinct advantages and limitations. Ongoing advancements in surgical techniques and a multidisciplinary approach are essential to optimize patient outcomes.