Langerhans cell histiocytosis causing acute optic neuropathy
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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.
KeywordsLangerhans 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.
- 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
- 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
- 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.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.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