Dept. of Radiology, University of Alabama - Birmingham, Birmingham, AL.
Curr Probl Diagn Radiol. 2020 May-Jun;49(3):205-214. doi: 10.1067/j.cpradiol.2019.02.012. Epub 2019 Mar 2.
Idiopathic intracranial hypertension (IIH) is a syndrome of unknown cause that is increasing in frequency. Patients who are typically women of childbearing age and obese present with headaches and may also present with visual changes that may become chronic. The purpose of this review is to describe the possible mechanisms for this disease and also to illustrate the ever increasing role of imaging in the diagnosis of this disorder. In addition, the various methods of treatment including medical and surgical will be reviewed. The fact that idiopathic intracranial hypertension has undergone many name changes over the years serves as a reminder that the underlying mechanism is still not well understood. Although there are only several possible mechanisms that can cause increased intracranial pressure, it is still not certain which of these mechanisms is involved. The role of imaging has significantly changed in the evaluation of patients with possible IIH. First, it is involved in ruling out secondary causes of increased intracranial pressure. In addition, there is now ample evidence that the previously held belief that imaging of patients with IIH should be normal is incorrect but rather that there are several subtle findings that radiologists need to look for. These findings include a partially empty sella, flattening of the posterior globe, cupping of optic disks and distension of the optic nerve sheaths. In addition, the role of intracranial venography is playing an ever increasing role due to the finding that a very high percentage of patients have dural venous sinus stenoses. It is becoming clear that there is potentially true morbidity associated with idiopathic intracranial hypertension. The earlier the disease can be diagnosed, the earlier treatment can be started to minimalize permanent visual changes including blindness. Treatment varies from institution to institution due to the fact that multiple specialists with different perspectives treat these patients. Knowledge of subtle imaging features associated with idiopathic intracranial hypertension can help radiologists establish the diagnosis earlier and potentially prevent complications of this disorder. However imaging has not as of yet been shown to be beneficial in managing patients with idiopathic intracranial hypertension.
特发性颅内高压(IIH)是一种病因不明且发病率不断上升的综合征。该病患者多为育龄期肥胖女性,主要表现为头痛,也可能出现慢性视觉改变。本文旨在描述这种疾病的可能发病机制,并说明影像学在诊断该病中的作用日益凸显。此外,本文还将对包括药物和手术治疗在内的各种治疗方法进行综述。多年来,特发性颅内高压这个名称几经变化,这提醒我们其潜在发病机制仍不明确。虽然能够引起颅内压升高的潜在机制只有几种,但仍不能确定具体是哪种机制在起作用。影像学在疑似 IIH 患者的评估中的作用已发生显著变化。首先,它可用于排除颅内压升高的继发性病因。此外,目前已有充分证据表明,之前认为 IIH 患者的影像学检查应该正常的观点是不正确的,而是存在一些细微的发现,需要放射科医生寻找。这些发现包括部分空蝶鞍、眼球后段变平、视盘杯状凹陷和视神经鞘扩张。此外,由于发现相当高比例的患者存在硬脑膜静脉窦狭窄,颅内静脉造影的作用也越来越大。目前越来越清楚的是,特发性颅内高压可能导致真正的发病率。该病越早诊断,就能越早开始治疗,使永久性视觉改变(包括失明)最小化。由于有不同视角的多个专科医生对这些患者进行治疗,因此各个机构的治疗方法也有所不同。了解与特发性颅内高压相关的细微影像学特征,有助于放射科医生更早地做出诊断,从而可能预防该病的并发症。但是,影像学在管理特发性颅内高压患者方面尚未显示出有益作用。