Orssaud C, Dureau P, Zerah M, Cinalli G, Sainte Rose C, Kahn A P, Dufier J L
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.
J Fr Ophtalmol. 2001 Jan;24(1):54-9.
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.
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.
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.
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.
Ophtalmologists are involved in the detection of pseudotumor cerebri and the monitoring of visual function, an important element in evaluating treatment efficacy.
良性颅内高压（或假性脑瘤）在儿童中并不常见，多见于成人。准确诊断需要排除颅内高压的所有其他病因。其治疗大多采用药物治疗，必须迅速进行以避免永久性视力丧失。然而，当视力受到威胁时，则需要进行手术。我们介绍我们在这种病症方面的经验，并讨论其临床特征、病因及病理生理机制。
我们对经神经放射学和神经外科检查确诊为良性颅内高压的儿童进行了一项回顾性研究。这些检查还用于明确病因。根据儿童年龄和临床状况进行的眼科检查包括视力测试、视盘评估、眼球运动测试和视野评估。治疗后分析视力和视盘的进展情况。
22名儿童（12名男孩和10名女孩）确诊为良性颅内高压。临床表现包括头痛和视觉障碍，如视力丧失和动眼神经麻痹。几乎所有病例均出现视乳头水肿。7名儿童药物治疗成功；然而，其余15名患者因颅内压升高、对药物治疗无反应或视力受到威胁而需要进行腰大池腹腔分流术。
良性颅内高压在儿童中并不常见，其病理生理机制仍不清楚。它可能与严重视力丧失有关。在诊断前应排除所有其他颅内或髓内占位性病变。该综合征在儿童和成人患者中的病因不同。虽然药物治疗通常足以使颅内压恢复正常，但有时需要进行腰大池腹腔分流术。眼科医生在检测可能的视力丧失或视乳头异常以及确保适当的治疗随访方面发挥着重要作用。
眼科医生参与假性脑瘤的检测和视觉功能的监测，这是评估治疗效果的一个重要因素。
