Almotairi Fawaz S, Alanazi Aued I, Alokayli Sherin Hamad, Maghrabi Sarah, Elwatidy Sherif M
Neurosurgery Division, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Department of surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Asian J Neurosurg. 2024 May 27;19(2):179-185. doi: 10.1055/s-0044-1779447. eCollection 2024 Jun.
Idiopathic intracranial hypertension (IIH) is a condition in which intracranial pressure (ICP) increases without an apparent cause. Typically, patients present with headaches, dizziness, pulsatile tinnitus, visual disturbances, blurred vision, diplopia, photophobia, visual field defects, and papilledema on fundoscopy. The association between IIH, spontaneous cerebrospinal fluid (CSF) rhinorrhea, and arachnoid cysts has been discussed in the literature; however, there is no clear explanation for this association. We aimed to present a series of four patients with a confirmed diagnosis of IIH with atypical presentations, discuss the management of each case, and provide an explanation for this association to alert clinicians to the atypical presentation of IIH and facilitate early diagnosis and proper treatment of this condition by CSF diversion. This was a retrospective case series of all patients who were diagnosed with IIH and showed improvement after ventriculoperitoneal shunt insertion after failure of at least one operative intervention resulting from primary radiological and clinical findings in 2001 to 2022. Data on demographics, clinical presentation, radiological findings, surgical management, and diagnostic criteria for IIH were recorded. We identified four patients with a confirmed diagnosis of IIH who presented with atypical presentations as follows: intracranial arachnoid cyst, cervical spine arachnoid cyst, giant Virchow perivascular space, and spontaneous CSF (CSF) rhinorrhea. All patients responded to CSF diversion after failure of surgical treatment targeting the primary pathology. IIH should be suspected after the failure of primary surgical treatment in cases of spontaneous CSF rhinorrhea, spinal and cranial arachnoid cysts, and symptomatic ventriculoperitoneal shunt. Treatment in such situations should be directed toward IIH with CSF diversion.
特发性颅内高压（IIH）是一种颅内压（ICP）升高但无明显病因的病症。通常情况下，患者会出现头痛、头晕、搏动性耳鸣、视觉障碍、视力模糊、复视、畏光、视野缺损以及眼底镜检查可见视乳头水肿。文献中已讨论过IIH、自发性脑脊液（CSF）鼻漏和蛛网膜囊肿之间的关联；然而，对于这种关联尚无明确解释。我们旨在介绍一系列4例确诊为IIH但表现不典型的患者，讨论每例患者的治疗方法，并对这种关联作出解释，以提醒临床医生注意IIH的非典型表现，并通过脑脊液分流促进对该病症的早期诊断和恰当治疗。这是一项回顾性病例系列研究，研究对象为2001年至2022年期间所有因原发性影像学和临床检查结果，在至少一次手术干预失败后接受脑室腹腔分流术并显示病情改善的IIH确诊患者。记录了患者的人口统计学数据、临床表现、影像学检查结果、手术治疗情况以及IIH的诊断标准。我们确定了4例确诊为IIH但表现不典型的患者，具体如下：颅内蛛网膜囊肿、颈椎蛛网膜囊肿、巨大的维尔肖血管周围间隙以及自发性脑脊液（CSF）鼻漏。所有患者在针对原发性病变的手术治疗失败后，对脑脊液分流术均有反应。对于自发性脑脊液鼻漏、脊柱和颅内蛛网膜囊肿以及有症状性脑室腹腔分流术的病例，在原发性手术治疗失败后应怀疑IIH。在这种情况下应采用脑脊液分流术针对IIH进行治疗。
