Aldhahwani Bashaer, Shah Serena M, Jiang Hong, Lam Byron L
Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA.
King Abdulaziz Medical City, Jeddah, Saudi Arabia.
Am J Ophthalmol Case Rep. 2024 Oct 18;36:102201. doi: 10.1016/j.ajoc.2024.102201. eCollection 2024 Dec.
To report a case series of 4 patients with poor visual outcomes from concurrent fulminant idiopathic intracranial hypertension (IIH) and malignant arterial hypertension with bilateral optic disc edema. The diagnosis of fulminant IIH was delayed given the bilateral optic disc edema was attributed initially to hypertensive optic neuropathy.
All 4 patients (3 males, 3 African Americans, mean BMI 27.6 kg/m (range 19.5-36 kg/m) presented to the emergency department with bilateral vision loss, optic disc edema, and blood pressure (BP) of greater than 180/120. The patients were treated initially to control BP and the optic disc edema was either attributed to the hypertension or the ophthalmic examination was not performed. The patients were subsequently diagnosed with IIH with Brain MRI, MR venogram, and lumber puncture (mean cerebrospinal fluid (CSF) opening pressure 42 cm, range 40-43 cm). The mean time from presentation to diagnosis of IIH was 3.2 months (range 1-6 months). The final visual acuity ranged from 20/400 to hand motions in the better eye and count fingers to hand motions in the worse eye despite bilateral optic nerve sheath fenestrations (3 patients), ventriculoperitoneal shunts (3 patients), and treatments with acetazolamide (3 patients) and furosemide (1 patient).
Our case series underscores the need to promptly include IIH in the differential diagnosis in patients with bilateral optic disc edema including patients with malignant hypertension, particularly in those experiencing progressive visual loss, regardless of gender or BMI. Prompt work-up with brain MRI with contrast and MR or CT venogram to detect neuroimaging signs of intracranial hypertension followed by a lumbar puncture with CSF opening pressure are essential to initiate rapid treatment of fulminant IIH to avoid poor outcome.
报告4例同时患有暴发性特发性颅内高压（IIH）和恶性动脉高血压并伴有双侧视盘水肿且视力预后较差的病例系列。由于双侧视盘水肿最初被归因于高血压性视神经病变，暴发性IIH的诊断被延迟。
所有4例患者（3例男性，3例非裔美国人，平均体重指数27.6kg/m²（范围19.5 - 36kg/m²））因双侧视力丧失、视盘水肿和血压（BP）高于180/120而就诊于急诊科。患者最初接受治疗以控制血压，视盘水肿要么归因于高血压，要么未进行眼科检查。随后通过脑部磁共振成像（MRI）、磁共振静脉造影（MRV）和腰椎穿刺（平均脑脊液（CSF）初压42cm，范围40 - 43cm）诊断患者为IIH。从就诊到诊断IIH的平均时间为3.2个月（范围1 - 6个月）。尽管进行了双侧视神经鞘开窗术（3例患者）、脑室腹腔分流术（3例患者），并使用了乙酰唑胺（3例患者）和呋塞米（1例患者）进行治疗，但最终较好眼的视力范围为20/400至手动，较差眼的视力范围为指数至手动。
我们的病例系列强调，对于双侧视盘水肿的患者，包括患有恶性高血压的患者，尤其是那些视力逐渐丧失的患者，无论性别或体重指数如何，都需要在鉴别诊断中及时考虑IIH。及时进行增强脑部MRI和MR或CT静脉造影以检测颅内高压的神经影像学征象，随后进行测量脑脊液初压的腰椎穿刺，对于启动暴发性IIH的快速治疗以避免不良预后至关重要。
