Child's Nervous System

, Volume 31, Issue 4, pp 615–619 | Cite as

Langerhans cell histiocytosis causing acute optic neuropathy

  • Jin Woo Bae
  • Yong Hwy Kim
  • Seung-Ki Kim
  • Kyu-Chang Wang
  • Hee-Young Shin
  • Hyoung Jin Kang
  • Sung-Hye Park
  • Ji Hoon PhiEmail author
Case Report



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.


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.


His visual impairment improved dramatically after the operation.


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.


Langerhans cell histiocytosis Skull base Acute optic neuropathy Decompression Endoscopic endonasal approach 


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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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Jin Woo Bae
    • 1
  • Yong Hwy Kim
    • 1
  • Seung-Ki Kim
    • 1
  • Kyu-Chang Wang
    • 1
  • Hee-Young Shin
    • 2
  • Hyoung Jin Kang
    • 2
  • Sung-Hye Park
    • 3
  • Ji Hoon Phi
    • 1
    Email author
  1. 1.Division of Pediatric NeurosurgerySeoul National University Children’s Hospital, Seoul National University College of MedicineSeoulRepublic of Korea
  2. 2.Department of PediatricsSeoul National University Children’s Hospital, Seoul National University College of MedicineSeoulRepublic of Korea
  3. 3.Department of PathologySeoul National University Children’s Hospital, Seoul National University College of MedicineSeoulRepublic of Korea

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