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Langerhans cell histiocytosis causing acute optic neuropathy

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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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Conflict of interest

This study was supported by a grant (no. 0420130930) from the Seoul National University Research Fund. The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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Authors and Affiliations

  1. 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

    Jin Woo Bae, Yong Hwy Kim, Seung-Ki Kim, Kyu-Chang Wang & Ji Hoon Phi

  2. Department of Pediatrics, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu,, 110-744, Seoul, Republic of Korea

    Hee-Young Shin & Hyoung Jin Kang

  3. Department of Pathology, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu,, 110-744, Seoul, Republic of Korea

    Sung-Hye Park

Authors
  1. Jin Woo Bae
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  2. Yong Hwy Kim
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  3. Seung-Ki Kim
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  4. Kyu-Chang Wang
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  5. Hee-Young Shin
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  7. Sung-Hye Park
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  8. Ji Hoon Phi
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Correspondence to Ji Hoon Phi.

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Bae, J.W., Kim, Y.H., Kim, SK. et al. Langerhans cell histiocytosis causing acute optic neuropathy. Childs Nerv Syst 31, 615–619 (2015). https://doi.org/10.1007/s00381-014-2610-5

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  • DOI: https://doi.org/10.1007/s00381-014-2610-5

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