朗格汉斯细胞组织细胞增多症导致急性视神经病变。
Langerhans cell histiocytosis causing acute optic neuropathy.
作者信息
Bae Jin Woo, Kim Yong Hwy, Kim Seung-Ki, Wang Kyu-Chang, Shin Hee-Young, Kang Hyoung Jin, Park Sung-Hye, Phi Ji Hoon
机构信息
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, 110-744, Seoul, Republic of Korea.
出版信息
Childs Nerv Syst. 2015 Apr;31(4):615-9. doi: 10.1007/s00381-014-2610-5. Epub 2015 Jan 8.
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.
目的
起源于颅底的朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的急性视神经病变病因。我们描述了一种用于视神经减压和同时切除病变的临床方法。这是首例通过鼻内镜经鼻入路对LCH所致急性视神经病变进行减压手术后视力改善的病例。
方法
一名11岁男孩，有2周视力恶化病史。观察到影像学证据显示视神经管周围有一个扩张性软组织肿块压迫视神经。该患者接受了鼻内镜经鼻视神经减压和肿瘤次全切除术。
结果
术后他的视力障碍显著改善。
结论
蝶筛骨出现的LCH可引发急性视神经病变。早期减压是挽救视力的关键。在这种情况下，鼻内镜经鼻入路可能是一种良好的治疗选择。
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