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Child's Nervous System
Published in: Child's Nervous System 4/2015

01-04-2015 | Case Report

Langerhans cell histiocytosis causing acute optic neuropathy

Authors: Jin Woo Bae, Yong Hwy Kim, Seung-Ki Kim, Kyu-Chang Wang, Hee-Young Shin, Hyoung Jin Kang, Sung-Hye Park, Ji Hoon Phi

Published in: Child's Nervous System | Issue 4/2015

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Abstract

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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Metadata
Title
Langerhans cell histiocytosis causing acute optic neuropathy
Authors
Jin Woo Bae
Yong Hwy Kim
Seung-Ki Kim
Kyu-Chang Wang
Hee-Young Shin
Hyoung Jin Kang
Sung-Hye Park
Ji Hoon Phi
Publication date
01-04-2015
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 4/2015
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-014-2610-5

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