Langerhans cell histiocytosis causing acute optic neuropathy

Area of Science:

• Neuro-oncology
• Ophthalmology
• Endoscopic Skull Base Surgery

Background:

• Langerhans cell histiocytosis (LCH) is a rare condition that can affect the skull base.
• Skull base LCH can lead to acute optic neuropathy, causing significant visual impairment.

Observation:

• An 11-year-old boy presented with rapidly progressing visual deterioration.
• Radiological imaging revealed a soft tissue mass compressing the optic canal, indicative of compressive optic neuropathy.

Findings:

• The patient underwent successful endoscopic endonasal optic nerve decompression and subtotal tumor removal.
• Postoperatively, the patient experienced dramatic improvement in visual function.

Implications:

• This case highlights LCH as a potential cause of acute optic neuropathy.
• Early surgical decompression via an endoscopic endonasal approach may be crucial for vision salvage in such cases.
• This approach offers a minimally invasive option for managing optic nerve compression due to skull base LCH.