Kawalec Philip, Del Bigio Marc R, Kaufmann Anthony M
1Section of Neurosurgery, Department of Surgery, University of Manitoba, Health Sciences Centre, Winnipeg, Manitoba, Canada.
2Department of Pathology, University of Manitoba, Health Sciences Centre, Winnipeg, Manitoba, Canada; and.
J Neurosurg Case Lessons. 2024 Feb 26;7(9). doi: 10.3171/CASE23746.
A cavernous malformation of the optic nerve (CMON) is a rare condition that often presents with an abrupt decline in vision. Acute management of ruptured optic nerve cavernous malformations is generally surgical, although the timing of surgery is controversial.
A 47-year-old female experienced the sudden loss of vision in her left eye. Examination showed that this eye was nearly blind, and her right eye had a temporal field defect. Neuroimaging showed hemorrhage in her left optic nerve and optic chiasm. She was taken to the operating room on an emergent basis where the optic canal was decompressed, the hemorrhage was evacuated, and a vascular malformation with features of a cavernoma was removed from the optic nerve. Over the next 2 days, the vision in her right eye significantly recovered.
CMONs remain rare, and it is unlikely that enough cases can be gathered to form a larger trial to compare the role and timing of surgery. On the basis of our experience with this case, the authors recommend that acute CMON-related hematomas should be treated as a surgical emergency and managed with acute optic nerve decompression, hematoma evacuation, and cavernoma resection to improve chances of vision recovery and prevent further vision loss.
视神经海绵状畸形（CMON）是一种罕见疾病，常表现为视力突然下降。尽管手术时机存在争议，但破裂性视神经海绵状畸形的急性处理通常为手术治疗。
一名47岁女性左眼突然失明。检查显示该眼几乎失明，右眼有颞侧视野缺损。神经影像学检查显示其左侧视神经和视交叉出血。她被紧急送往手术室，术中对视神经管进行减压，清除出血，并从视神经上切除了具有海绵状血管瘤特征的血管畸形。在接下来的2天里，她右眼的视力显著恢复。
CMON仍然罕见，不太可能收集到足够多的病例来进行更大规模的试验以比较手术的作用和时机。基于我们对该病例的经验，作者建议与急性CMON相关的血肿应作为外科急症处理，通过急性视神经减压、血肿清除和海绵状瘤切除来提高视力恢复的机会并防止视力进一步丧失。
