External lumbar drainage in progressive pediatric idiopathic intracranial hypertension

OBJECTIVE Pediatric idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure despite normal cerebrospinal fluid and neuroimaging findings. Initial management is typically medical; however, nearly 10% of children will eventually require surgery for persistent headache and/or vision loss. External lumbar drainage, which is a considerably safer treatment option, has not been adequately analyzed in children with medically refractory IIH. METHODS The authors conducted a single-institution retrospective analysis of children with medically refractory IIH who had undergone external lumbar drain (ELD) placement because of worsening papilledema, reflected as increased retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT) testing. The main outcome measures were effects of external lumbar drainage on papilledema resolution, symptoms, and vision. RESULTS The authors analyzed the medical records of 13 children with IIH (11 girls, mean age 15.0 ± 2.3 years) whose mean CSF opening pressure was 45.5 ± 6.8 cm H2O. In all children, the average global RNFL thickness in both eyes significantly increased at ELD placement (right eye 371.8 ± 150.2 μm, left eye 400.3 ± 96.9 μm) compared with presentation thickness (right eye 301.6 ± 110.40 μm, left eye 350.2 ± 107.7 μm) despite acetazolamide medical therapy (20-30 mg/kg/day), leading to ELD placement after 9.5 ± 6.9 days (range 3-29 days). After ELD insertion, there was headache resolution, gradual and continuous improvement in optic disc thickness, and preservation of good vision. CONCLUSIONS ELD placement in children with medically refractory IIH who demonstrated worsening papilledema with increased RNFL thickening on OCT testing typically results in symptom relief and disc edema resolution with good visual outcome, often preventing the need for additional definitive surgeries that carry greater failure and morbidity risks.