Langerhans cell histiocytosis causing acute optic neuropathy
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文摘
Purpose Langerhans cell histiocytosis (LCH) arising from the skull base is a rare acute optic neuropathy cause. We describe a clinical approach for the purpose of optic nerve decompression and simultaneous lesion excision. This is the first case of visual improvement following decompression operation via endoscopic endonasal approach for acute optic neuropathy that is produced by LCH. Methods An 11-year-old boy presented with a 2-week history of visual deterioration. Radiological evidence of compressive optic neuropathy by an expansile soft tissue mass around the optic canal was observed. The patient underwent an endoscopic endonasal optic nerve decompression and subtotal tumor removal. Results His visual impairment improved dramatically after the operation. Conclusions Acute optic neuropathy can be elicited by LCH arising in the sphenoid-ethmoid bone. Early decompression is the key to vision salvage. An endoscopic endonasal approach may be a good therapeutic option in this situation.
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