title: Pseudotumor cerebri as an important differential diagnosis of papilledema in children.
abstract: BACKGROUND
  Primary pseudotumor cerebri (PTC) in childhood is a rare but important differential diagnosis in children presenting with papilledema. It is defined as elevated cerebrospinal fluid (CSF) pressure of more than 20 cm H(2)O, normal CSF composition, and exclusion of underlying structural or systemic causes. Visual loss is a serious complication, which requires careful monitoring and management.
  
  
  METHODS
  We conducted a retrospective chart review of 12 patients with primary PTC. The mean age at presentation was 8212 years, and there was a male-to-female ratio of 7:5. The aim of this study was to investigate the clinical features of primary PTC in children, and to highlight the different treatment options in normalizing intracranial pressure in these patients.
  
  
  RESULTS
  In the majority of cases, children presented with headache. Four patients had no obvious symptoms and papilledema was found on routine eye examination. Obesity was uncommon and there was no distinct sex predilection. Acetazolamide was our drug of choice for the initial treatment. Furosemide and prednisone were used as second-line agents. Treatment was gradually decreased after resolution of the papilledema with exception of the two youngest children, who remained symptomatic. One child underwent ventricular-peritoneal shunting.
  
  
  CONCLUSIONS
  The treatment goals of PTC are the relief of symptoms, and preservation of visual function. Acetazolamide is an effective first-line method of lowering raised intracranial pressure. In our study group especially the young children were difficult to treat. This might indicate an age-related difference in the etiology of PTC. When medical treatment remains ineffective and visual function deteriorates, surgical treatment should be considered.
authors.affiliation: Department of General Pediatrics, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, D-40225 Düsseldorf, Germany. distelmaier@med.uni-duesseldorf.de
authors.forename: Felix
authors.initials: F
authors.lastname: Distelmaier
authors.forename: Ulrike
authors.initials: U
authors.lastname: Sengler
authors.forename: Martina
authors.initials: M
authors.lastname: Messing-Juenger
authors.forename: Birgit
authors.initials: B
authors.lastname: Assmann
authors.forename: Ertan
authors.initials: E
authors.lastname: Mayatepek
authors.forename: Thorsten
authors.initials: T
authors.lastname: Rosenbaum
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005451:Fluorescein Angiography; D006261:Headache / Q000209:etiology; D006801:Humans; D007223:Infant; D010211:Papilledema / Q000175:diagnosis*; D011559:Pseudotumor Cerebri / Q000175:diagnosis*; D012189:Retrospective Studies; D014786:Vision Disorders / Q000209:etiology