Jalkh Rita Maria, Abou Raji Feghali Patrick, Lababidi Ghena, Darwish Houssein, Korban Zeina
Department of Otolaryngology, American University of Beirut Medical Center, Beirut, Lebanon.
Department of Neurosurgery, American University of Beirut Medical Center, Beirut, Lebanon.
J Neurol Surg Rep. 2025 Mar 20;86(1):e36-e40. doi: 10.1055/a-2544-3435. eCollection 2025 Jan.
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.
骨质石化症是一种罕见的遗传性疾病，其特征为破骨细胞功能受损和骨密度过高，常因视神经管骨质过度生长导致压迫性视神经病变。及时进行手术干预对于防止受影响患者永久性视力丧失至关重要。本综述总结了关于骨质石化症患者内镜下经鼻视神经减压术（EEOND）和经泪阜入路手术技术的现有文献。EEOND是一种微创手术技术，具有良好的视野、先进的器械，可通过鼻腔通道进入视神经，且需要掌握蝶骨解剖结构才能取得成功。然而，骨质石化症患者的骨骼致密且脆弱，给手术带来了挑战。经泪阜入路为进入内侧视神经管提供了一条有针对性的途径，外部瘢痕最小。然而，在严重病例中，其有限的范围可能不足以进行广泛减压。早期手术干预与更好的视觉效果相关，尤其是在视力丧失快速进展风险较高的儿科患者中。整合先进成像技术和混合手术技术可能会提高减压效果。总之，EEOND和经泪阜入路对于治疗骨质石化症中的视神经压迫都很有价值，各有其独特的优点和局限性。手术技术的不断进步和多学科方法对于优化患者预后至关重要。
