Fulminant Intracranial Hypertension Managed With Temporary Lumbar Drain.

Abstract

In the Journal of Neuro-Ophthalmology, Jiramongkolchai et al (1) reported 2 patients with fulminant intracranial hypertension (FIH) who were managed with temporary lumbar drains in the setting of severe visual loss. We also cared for a pediatric patient with FIH because of minocycline and used a lumbar drain as part of the therapeutic regimen. A 15-year-old girl with a body mass index of 30 kg/m2 was evaluated for FIH. Visual acuity was hand motions, right eye, and 20/400, left eye. Automated visual fields (Humphrey 24-2) revealed mean deviations of −35.0 dB in both eyes with foveal sensitivities of 23 dB, right eye, and 13 dB, left eye. There was Frisen Grade 5 papilledema bilaterally, and optical coherence tomography showed peripapillary retinal nerve fiber layer thickening in both eyes (325 μm, right eye; 362 μm, left eye). Opening pressure on lumbar puncture was 50 cm of H2O with normal cerebrospinal fluid (CSF) constituents. The patient was initially managed with intravenous medications: acetazolamide 4 g/day, methylprednisolone 1 g/day, and furosemide 80 mg/day. Because of failure to improve, a temporary lumbar drain was placed on hospital Day 4 and titrated to drain 20 cc of CSF every 2 hours. On postlumbar drain Day 1, the drain was titrated to 12 cc of CSF every 2 hours, but on postlumbar drain Day 2, the patient developed lower-extremity paresthesias concerning for over drainage and the drain was removed. Over the next several weeks, the patient's vision began to gradually improve and at her 4-month visit, acuity was 20/50 bilaterally. Visual field mean deviation was 5.08 dB, right eye, and 6.07 dB, left eye, and retinal nerve fiber layer thickness was 87 μm, right eye and 86 μm, left eye. The patient was maintained on acetazolamide 2 g/day. To avoid severe vision loss, patients with FIH may require urgent surgical intervention. One study of patients with FIH indicated that better visual recovery occurred when surgery was undertaken within 4 days of initial evaluation (2). There are several aspects of our patient's case that are worthy of mention. First, in other reports, lumbar drains were left in place from 3 to 10 days (1,3). In our patient, the lumbar drain was in place for only 48 hours, yet she still experienced significant improvement in visual function. Our patient's drainage rate was initially titrated to 20 cc of CSF every 2 hours and then reduced, while Jiramongkolchai et al (1) set a rate of 10–15 cc every hour per the institutional protocol of their medical center. Finally, lumbar drains previously have been used as a temporizing measure before definitive surgery in patients with FIH. Yet, the few studies that have been reported raise the possibility that lumbar drains may have an important role to play in re-establishing normal intracranial pressure and possibly reduce the need for a surgical procedure. In addition to the lumbar drain, administering acetazolamide, methylprednisolone, or furosemide likely contributed to our patient's recovery. Yet, it was only after placement of a lumbar drain that our patient's clinical course began to show improvement. Further research is needed to determine the optimal duration and drainage rate required for a lumbar drain in the treatment of FIH.