Pseudopapilledema and association with idiopathic intracranial hypertension

Childs Nerv Syst. 2014 Jul;30(7):1197-200. doi: 10.1007/s00381-014-2390-y. Epub 2014 Feb 27.


Purpose: Diagnosing idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, can be challenging in children. Diagnosis is based on lumbar puncture, opening pressures, and appearance of the optic disk. Misdiagnosis of papilledema, a typical finding, may lead to unnecessary treatments and procedures. We report 52 children over a 6-year period to better identify the true incidence of pseudopapilledema and other factors that may confound the diagnosis of IIH.

Methods: A retrospective chart review approved by the Institutional Review Board was performed. Fifty-two children under the age of 21 referred to us based on suspected IIH or papilledema from 2007 to 2013 are included in this study. Patients were assessed by a pediatric ophthalmologist and a neurosurgeon.

Results: Fifty-two children were initially diagnosed with IIH and/or papilledema; 26 diagnoses were revised to pseudopapilledema after pediatric ophthalmological review. Out of those 26 patients with pseudopapilledema, 14 had undergone lumbar punctures, 19 had MRIs, 9 had CTs, and 12 were taking medications-these medications were discontinued upon revision of the diagnoses. The difference in the CSF opening pressure between children diagnosed with true IIH (32.7 cm H2O) and children diagnosed with pseudopapilledema (24.7 cm H2O) was statistically significant.

Conclusions: IIH diagnosis is heavily reliant on the appearance of the optic disk. Pediatric ophthalmological assessment is essential to carefully examine the optic disk and prevent further unnecessary investigation and treatments. Close communication between pediatricians, ophthalmologists, and neurosurgeons can avoid invasive procedures for children who do have pseudopapilledema, and not IIH or associated papilledema.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Eye Diseases, Hereditary / diagnosis*
  • Eye Diseases, Hereditary / epidemiology
  • Female
  • Humans
  • Incidence
  • Male
  • Optic Disk / pathology*
  • Optic Nerve Diseases / diagnosis*
  • Optic Nerve Diseases / epidemiology
  • Papilledema / diagnosis*
  • Pseudotumor Cerebri / diagnosis*
  • Retrospective Studies
  • Young Adult

Supplementary concepts

  • Pseudopapilledema