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

The evidence for low cerebrospinal fluid pressure (CSFP) as a key parameter in the pathogenesis of glaucoma is increasing. Primate models have demonstrated the onset normal tension glaucoma (NTG) from experimentally induced chronic intrathecal hypotension; an approach not possible in human subjects.

A 27-year-old man presented with a central scotoma in his left eye. He had undergone 8 CSF shunt revision procedures over a 25-year period secondary to recurrent low CSFP following surgical excision of a pinealoblastoma, aged 2. A focal nerve fiber layer defect was detected in the left eye associated with reduced retinal sensitivity on microperimetry. Three adjacent optic disc hemorrhages had been documented in the same position over an 18-month period. A diagnosis of left-sided NTG was made; the patient was started on Latanoprost 0.005%. A new generation CSF shunting device (ProGAV)-which neutralizes CSFP fluctuations analogously to trabeculectomy surgery for intraocular pressure-was considered necessary in this patient to alleviate persistent headaches and reduce the risk of progressive glaucomatous visual loss.

This exceptional case illustrates how premature onset NTG may occur as a result of chronic, recurrent intrathecal hypotension-a "pure" human model. We describe an original management approach of implanting an adjustable, programmable CSF shunt valve (ProGAV) to reduce fluctuations in the translaminar cribrosa pressure difference, and reduce the risk of glaucomatous visual loss.