100. Is Pseudotumor a Scapegoat for Biobehavioral Patients?

Authors: Ruth E. Bristol, MD, FAANS

Pediatric neurosurgeons are frequently asked to evaluate patients with elevated lumbar puncture (LP) pressures for potential long-term shunt placement. Pseudotumor cerebri is a poorly understood condition associated with elevated pressure in the intracranial space. In patients with biobehavioral problems and non-specific symptoms, LP is often part of the work up. As part of a larger study investigating the correlation of lumbar punctures with intracranial pressure (ICP), a subset of biobehavioral patients was identified.Methods: An IRB approved retrospective chart review was carried out of all patients undergoing ICP wire placement between 2012-2017 at our institution. Patients with a diagnosis of autism or other developmental delay were selected.
Out of 54 patients undergoing ICP monitoring for non-traumatic indications, 12 met study criteria. Many of these patients were non-verbal and exhibited behaviors interpreted to represent headaches. After trials on standard headache medications, a concern for elevated ICP was raised and 7 of 12 underwent lumbar punctures with mild to moderately elevated pressures (22-48 mmHg). The other 5 either had papilledema (2), poor growth trajectory after synostosis repair (2), or already had a CSF diverting system in place (2). Upon subsequent ICP monitoring via wire, none were confirmed to have high ICP, and 2 shunt systems were actually removed. Complete data including incidence of papilledema, BMI, drusen, and response to medical therapy is reviewed in the study.
Lumbar puncture in the pediatric population is a poor predictor of elevated ICP. Pediatric patients who cannot accurately report their symptoms often undergo a battery of tests for non-specific symptoms. The use of these tests in vulnerable populations, such as the developmentally delayed, should be considered carefully because a cascade of more invasive subsequent testing may be initiated. Neurosurgeons can educate other services that idiopathic intracranial hypertension is rare in pediatric patients.