Advertisement

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

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.

Keywords

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

Notes

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.

References

  1. 1.
    Amit M, Fliss DM, Gil Z (2011) Fibrous dysplasia of the sphenoid and skull base. Otolaryngol Clin N Am 44(4):891–902CrossRefGoogle Scholar
  2. 2.
    Badalian-Very G, Vergilio JA, Degar BA, MacConaill LE, Brandner B, Calicchio ML, Kuo FC, Ligon AH, Stevenson KE, Kehoe SM, Garraway LA, Hahn WC, Meyerson M, Fleming MD, Rollins BJ (2010) Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 116(11):1919–1923CrossRefPubMedCentralPubMedGoogle Scholar
  3. 3.
    Balcer LJ (2006) Optic neuritis. N Engl J Med 354:1273–1280CrossRefPubMedGoogle Scholar
  4. 4.
    Belen D, Colak A, Ozcan OE (1996) CNS involvement of Langerhans cell histiocytosis: report of 23 surgically treated cases. Neurosurg Rev 19(4):247–252CrossRefPubMedGoogle Scholar
  5. 5.
    Gangwani V, Walker M, El-Defrawy H, Nicoll JA, Reck A, Pathmanathan T (2007) Langerhans cell histiocytosis of the optic chiasm. Clin Experiment Ophthalmol 35(1):66–68Google Scholar
  6. 6.
    Guy WM, Soparkar CN, Alford EL, Patrinely JR, Sami MS, Parke RB (2014) Traumatic optic neuropathy and second optic nerve injuries. JAMA Ophthalmol 132(5):567–571CrossRefPubMedGoogle Scholar
  7. 7.
    Hervey-Jumper SL, Ghori A, Ziewacz JE, Mckeever PE, Chandler WF (2011) Langerhans cell histiocytosis of the optic chiasm: case report. Neurosurgery 68(2):556–561CrossRefGoogle Scholar
  8. 8.
    Komotar RJ, Starke RM, Raper DM, Anand VK, Schwartz TH (2012) Endoscopic skull base surgery: a comprehensive comparison with open transcranial approaches. Bj J Neurosurg 26(5):637–648Google Scholar
  9. 9.
    Kong DS, Shin HJ, Kim HY, Chung SK, Nam DH, Lee JI, Park K, Kim JH (2011) Endoscopic optic nerve decompression for compressive optic neuropathy. J Clin Neurosci 18(11):1541–1545Google Scholar
  10. 10.
    Lee JW, Shin HY, Kang HJ, Kim H, Park JD, Park KD, Kim HS, Park SH, Wang KC, Ahn HS (2014) Clinical characteristics and treatment outcome of Langerhans cell histiocytosis: 22 years’ experience of 154 patients at a single center. Pediatr Hematol Oncol 31(3):293–302Google Scholar
  11. 11.
    Li KK, Lucarelli MJ, Bilyk JR, Joseph MP (1997) Optic nerve decompression for compressive neuropathy secondary to neoplasia. Arch Otolaryngol Head Neck Surg 123(4):425–429CrossRefPubMedGoogle Scholar
  12. 12.
    Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ (2004) MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 25(5):880–891PubMedGoogle Scholar
  13. 13.
    Ropposch T, Stegar B, Meco C, Emerz M, Reitsamer H, Rasp G, Moser G (2013) The effect of steroids in combination with optic nerve decompression surgery in traumatic optic neuropathy. Laryngosope 123(5):1082–1086CrossRefGoogle Scholar
  14. 14.
    Shields CL, Shields JA, Honavar SG, Demirci H (2001) Primary ophthalmic rhabdomyosarcoma in 33 patients. Trans Am Ophthalmol Soc 99:133–143PubMedCentralPubMedGoogle Scholar
  15. 15.
    Stromberg JS, Wang AM, Huang TE, Vicini FA, Nowak PA (1995) Langerhans cell histiocytosis involving the sphenoid sinus and superior orbital fissure. AJNR Am J Neuroradiol 16:964–967PubMedGoogle Scholar
  16. 16.
    Yano S, Hide T, Shinojima N, Hasegawa Y, Kawano T, Kuratsu J (2014) Ensodscopic endonasal approach for parasellar lesions: Initial experences, results, efficacy, and complications. Surg Neurol Int 5:51CrossRefPubMedCentralPubMedGoogle Scholar

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

Personalised recommendations