C Orssaud1, P Dureau, M Zerah, G Cinalli, C Sainte Rose, A P Kahn, J L Dufier. 1. Service d'Ophtalmologie du Professeur J.L. Dufier, Hôpital Necker - Enfants Malades, Assistance Publique-Hôpitaux de Paris, Faculté de médecine Necker-Enfants Malades, Paris-France.
Abstract
INTRODUCTION: Infrequent in children, benign intracranial hypertension (or pseudotumor cerebri) is most often observed in adults. Careful diagnosis requires eliminating all the other etiologies of intracranial hypertension. Most often medical, its treatment must be rapid to avoid permanent visual loss. However, a surgical procedure is necessary when vision is threatened. We present our experience with this pathology and discuss its clinical aspects, its etiologies, and the physiopathological mechanisms. PATIENTS AND METHODS: We conducted a retrospective study on children who presented benign intracranial hypertension confirmed by neuroradiological and neurosurgical examinations. These examinations also served to specify the responsible etiologies. The ophthalmologic examinations, adapted to the child's age and clinical status, included visual acuity testing, optic disc evaluation, 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 headache and visual disturbance such as visual loss and oculomotor nerve palsy. Papilledema was present in nearly all cases. Medical treatment was successful in 7 children; however, the remaining 15 patients required a lumboperitoneal shunt because of elevated intracranial pressure, no response to the medical therapy, or threatened vision. DISCUSSION: The physiopathological mechanisms of benign intracranial hypertension, an uncommon condition in children, are still unclear. It can be associated with severe visual loss. All other intracranial or medullary expansive lesions should be eliminated before diagnosis. The causes of this syndrome are not the same for pediatric and adult patients. Although medical therapy is usually sufficient to normalize the intracranial pressure, a lumboperitoneal shunt is at times required. The role of the ophthalmologist is important in detecting a possible visual loss or papilla abnormality and in ensuring proper treatment follow-up. CONCLUSION: Ophtalmologists are involved in the detection of pseudotumor cerebri and the monitoring of visual function, an important element in evaluating treatment efficacy.
INTRODUCTION: Infrequent in children, benign intracranial hypertension (or pseudotumor cerebri) is most often observed in adults. Careful diagnosis requires eliminating all the other etiologies of intracranial hypertension. Most often medical, its treatment must be rapid to avoid permanent visual loss. However, a surgical procedure is necessary when vision is threatened. We present our experience with this pathology and discuss its clinical aspects, its etiologies, and the physiopathological mechanisms. PATIENTS AND METHODS: We conducted a retrospective study on children who presented benign intracranial hypertension confirmed by neuroradiological and neurosurgical examinations. These examinations also served to specify the responsible etiologies. The ophthalmologic examinations, adapted to the child's age and clinical status, included visual acuity testing, optic disc evaluation, 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 headache and visual disturbance such as visual loss and oculomotor nerve palsy. Papilledema was present in nearly all cases. Medical treatment was successful in 7 children; however, the remaining 15 patients required a lumboperitoneal shunt because of elevated intracranial pressure, no response to the medical therapy, or threatened vision. DISCUSSION: The physiopathological mechanisms of benign intracranial hypertension, an uncommon condition in children, are still unclear. It can be associated with severe visual loss. All other intracranial or medullary expansive lesions should be eliminated before diagnosis. The causes of this syndrome are not the same for pediatric and adult patients. Although medical therapy is usually sufficient to normalize the intracranial pressure, a lumboperitoneal shunt is at times required. The role of the ophthalmologist is important in detecting a possible visual loss or papilla abnormality and in ensuring proper treatment follow-up. CONCLUSION: Ophtalmologists are involved in the detection of pseudotumor cerebri and the monitoring of visual function, an important element in evaluating treatment efficacy.
Authors: Daniel Tibussek; Dominik T Schneider; Nicola Vandemeulebroecke; Bernd Turowski; Martina Messing-Juenger; Peter H G M Willems; Ertan Mayatepek; Felix Distelmaier Journal: Childs Nerv Syst Date: 2009-11-10 Impact factor: 1.475