Prise en charge de l’hypertension intracrânienne bénigne : analyse de la série nancéenne

Abstract

Benign intracranial hypertension (BIH) is a rare condition, especially in childhood. The aim of this study was to analyze retrospectively pediatric cases that were diagnosed and managed in the same institution during the 2002-2006 period. Eight children (four girls and four boys) with a diagnosis of BIH were reviewed. The mean age at diagnosis was 10.5 years. Follow-up lasted a mean two years. The clinical features were those of intracranial hypertension. One child had previously had optic nerve sheath fenestration and another one occipitocervical decompression because of an associated Chiari I malformation. One child had a bilateral transverse sinus stenosis on angio-MRI. Seven children had a cerebrospinal fluid (CSF) pressure monitoring. Seven children were treated with acetazolamide. Three children are free of symptoms with the association of acetazolamide and depletive lumbar puncture (LP). For three others, a lumboperitoneal shunt had to be inserted. One child is in complete remission after depletive LP only. The clinical symptoms of BIH disappeared for all eight children, including normalization of the visual loss present in three children. BIH is a condition that threatens visual prognosis. Diagnosis is assessed by clinical, radiological, and raised CSF pressure criteria. First-line treatment is medical (acetazolamide at first intention) and surgery is recommended for refractory cases. The relationship between BIH and obesity is less clear than for adulthood. Depletion of CSF by LP is an important therapeutic factor.