Clinical spectrum of the pseudotumor cerebri complex in children.
OBJECTIVE
Our aim was to improve diagnosis and management of pseudotumor cerebri (PTC; also known as idiopathic intracranial hypertension) in children.


METHODS
We performed a comprehensive analysis of epidemiology, diagnostic work-up, therapy, and clinical follow-up in 53 consecutive patients.


RESULTS
We identified several important aspects to be considered in the management of these children. First, patients may present without obvious symptoms at diagnosis. Second, bilateral papilledema might not or not yet be present in symptomatic patients. Third, measurement of cerebrospinal fluid (CSF) opening pressure may not always be reliable due to drugs used for sedation, which may alter intracranial pressure. Fourth, normal CSF pressure values in childhood are not well established and diagnosis might even be justified if pressure is <20 cm H(2)O. Fifth, associated conditions are frequent (at least in our cohort); however, in most cases, a causative link cannot be proven. Finally, disease relapse is a serious problem (20% in our group), which stresses the importance of standardized follow-up programs.


CONCLUSIONS
PTC constitutes an important and possibly underrecognized disorder in children and adolescents. Considering the high percentage of possibly associated conditions in our study, a detailed diagnostic work-up is crucial to identify treatable underlying conditions.
Tibussek
Daniel
D
Department of General Pediatrics, University Children's Hospital, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Germany.
Schneider
Dominik T
DT
Vandemeulebroecke
Nicola
N
Turowski
Bernd
B
Messing-Juenger
Martina
M
Willems
Peter H G M
PH
Mayatepek
Ertan
E
Distelmaier
Felix
F
D000293:Adolescent; D015170:Cerebrospinal Fluid Pressure; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007427:Intracranial Pressure; D008297:Male; D010211:Papilledema / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D011559:Pseudotumor Cerebri / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology / Q000628:therapy*; D012008:Recurrence; D012016:Reference Values; D016896:Treatment Outcome