Benign childhood intracranial hypertension

Introduction: Infrequent in children, benign intracranial hypertension (or pseudotumor cerebri) is most often observed in adults. Careful diagnosis re quires eliminating all the other etiologies of intracranial hypertension. M ost often medical, its treatment must be rapid to avoid permanent visual lo ss. However, a surgical procedure is necessary when vision is threatened. W e present our experience with this pathology and discuss its clinical aspec ts, its etiologies, and the physiopathological mechanisms. Patients and methods: We conducted a retrospective study on children who pr esented benign intracranial hypertension confirmed by neuroradiological and neurosurgical examinations. These examinations also served to specify the responsible etiologies. The ophthalmologic examinations, adapted to the chi ld's age and clinical status, included visual acuity testing, optic disc ev aluation, ocular motility testing, and visual field evaluation. Progression of visual acuity and the topic disc was analyzed after treatment. Results: The diagnosis of benign intracranial hypertension was confirmed in 22 children (12 boys and 10 girls). Clinical presentation included headach e and visual disturbance such as visual loss and oculomotor nerve palsy. Pa pilledema was present in nearly all cases. Medical treatment was successful in 7 children; however, the remaining 15 patients required a lumboperitone al shunt because of elevated intracranial pressure, no response to the medi cal therapy, or threatened vision. Discussion: The physiopathological mechanisms of benign intracranial hypert ension, an uncommon condition in children, are still unclear. It can be ass ociated with severe visual loss. All other intracranial or medullary expans ive lesions should be eliminated before diagnosis. The causes of this syndr ome are not the same for pediatric and adult patients. Although medical the rapy is usually sufficient to normalize the intracranial pressure, a lumbop eritoneal shunt is at times required. The role of the ophthalmologist is im portant in detecting a possible visual loss or papilla abnormality and in e nsuring proper treatment follow-up. Conclusion: Ophthalmologists are involved in the detection of pseudotumor c erebri and the monitoring of visual function, an important element in evalu ating treatment efficacy.