Yang Peter H, Schneider John S, Chicoine Michael R, Kim Albert H, Limbrick David D
Departments of Neurological Surgery Washington University in St. Louis, St. Louis, Missouri.
Departments of Otolaryngology, Washington University in St. Louis, St. Louis, Missouri.
J Neurosurg Case Lessons. 2024 Jul 8;8(2). doi: 10.3171/CASE23559.
Congenital optic canal stenosis causing compressive optic neuropathy is a rare disorder that presents unique diagnostic and treatment challenges. Endoscopic endonasal optic nerve decompression (EOND) has been described for optic nerve compression in adults and adolescents but has never been reported for young children without pneumatized sphenoid sinuses. The authors describe preoperative and intraoperative considerations for three patients younger than 2 years of age with congenital optic canal stenosis due to genetically confirmed osteopetrosis or chondrodysplasia.
Serial ophthalmological examinations, with a particular focus on object tracking ability, fundoscopic examination, and visual evoked potential trends in preverbal children, are important for detecting progressive optic neuropathy. The lack of pneumatization of the sphenoid sinus presents unique challenges and requires the surgical creation of a sphenoid sinus with the use of neuronavigation to determine the limits of bony exposure given the lack of easily identifiable anatomical landmarks such as the opticocarotid recess. There were no perioperative complications.
EOND for congenital optic canal stenosis is safe and technically feasible even given the lack of pneumatization of the sphenoid sinus in young patients. The key operative step is surgically creating the sphenoid sinus through careful bony removal with the aid of neuronavigation. https://thejns.org/doi/10.3171/CASE23559.
先天性视神经管狭窄导致压迫性视神经病变是一种罕见疾病,存在独特的诊断和治疗挑战。内镜下经鼻视神经减压术(EOND)已被用于成人和青少年的视神经压迫,但从未有过针对蝶窦未气化的幼儿的报道。作者描述了3例年龄小于2岁、因基因确诊的骨质石化症或软骨发育异常导致先天性视神经管狭窄患者的术前和术中注意事项。
对于尚不能言语的儿童,连续进行眼科检查,尤其关注物体追踪能力、眼底检查和视觉诱发电位趋势,对于检测进行性视神经病变很重要。蝶窦未气化带来了独特挑战,鉴于缺乏如视神经颈动脉隐窝等易于识别的解剖标志,需要使用神经导航技术通过手术创建蝶窦以确定骨质暴露范围。围手术期无并发症发生。
即使年轻患者存在蝶窦未气化情况,先天性视神经管狭窄的内镜下经鼻视神经减压术也是安全且在技术上可行的。关键手术步骤是借助神经导航技术通过仔细去除骨质来手术创建蝶窦。https://thejns.org/doi/10.3171/CASE23559