Juvenile-onset Normal Tension Glaucoma From Chronic, Recurrent Low Cerebrospinal Fluid Pressure

Area of Science:

• Ophthalmology
• Neurosurgery
• Neurology

Background:

• Growing evidence links low cerebrospinal fluid pressure (CSFP) to glaucoma pathogenesis.
• Primate models suggest chronic intrathecal hypotension can induce normal tension glaucoma (NTG).

Observation:

• A 27-year-old male with a history of pinealoblastoma and multiple CSF shunt revisions presented with vision loss in his left eye.
• The patient exhibited signs of NTG, including a visual field defect, reduced retinal sensitivity, and optic disc hemorrhages, linked to recurrent low CSFP.
• This case represents a "pure" human model of premature NTG resulting from chronic intrathecal hypotension.

Findings:

• A diagnosis of left-sided NTG was made, and the patient was initiated on Latanoprost.
• A new-generation adjustable, programmable CSF shunt valve (ProGAV) was considered to manage CSFP fluctuations and mitigate glaucoma progression.

Implications:

• This case highlights the critical role of CSFP in glaucoma development, offering a unique human model for study.
• The management approach using a ProGAV shunt demonstrates a novel strategy to address CSFP fluctuations and reduce the risk of glaucomatous visual loss.