Bilateral Buried Optic Nerve Head Drusen Mimicking Idiopathic Intracranial Hypertension

Background The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. Objective To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. Methods Systematic collection of demographics, neuro-ophthalmic and magnetic resonance imaging evaluation of spontaneous skull base CSF leak patients seen pre- and post-leak repair in one neuro-ophthalmology service. Patients with preexisting IIH were diagnosed with definite IIH if adequate documentation was provided; otherwise, they were categorized with presumed IIH. Classic radiographic signs of intracranial hypertension and bilateral transverse venous sinus stenosis were recorded. Results Thirty six patients were included (age [interquartile range]: 50 [45;54] years; 94% women; body mass index: 36.8 [30.5;39.9] kg/m2), among whom six (16.7%, [95% confidence interval, CI]: [6.4;32.8]) had a preexisting diagnosis of definite or presumed IIH. Of the remaining 30 patients, four (13.3%, 95%CI: [3.8;30.7]) had optic nerve head changes suggesting previously undiagnosed IIH, while one was newly diagnosed with definite IIH at initial consultation. One out of 29 patients with normal findings of the optic nerve head at presentation developed new onset papilledema following surgery (3.4%, 95%CI: [0.1;17.8]) and was ultimately diagnosed with definite IIH. Overall, the prevalence of definite IIH was 19.4% (95%CI: [8.2;36.0]). Conclusion Striking demographic overlap exists between IIH patients and those with spontaneous CSF leak. Definite IIH was present in approximately 20% of our patients. However, its true prevalence is likely higher than identified by using classic criteria. We therefore hypothesize that an active CSF leak serves as an auto-diversion for CSF, thereby “treating” the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation.

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Contrast-Enhanced 3D-FLAIR Imaging of the Optic Nerve and Optic Nerve Head: Novel Neuroimaging Findings of Idiopathic Intracranial Hypertension

American Journal of Neuroradiology ◽

10.3174/ajnr.a5937 ◽

2019 ◽

Vol 40 (2) ◽

pp. 334-339 ◽

Cited By ~ 4

Author(s):

E. Golden ◽

R. Krivochenitser ◽

N. Mathews ◽

C. Longhurst ◽

Y. Chen ◽

...

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension ◽

Flair Imaging ◽

Contrast Enhanced ◽

3D Flair

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OCT assessment of morphological changes of the optic nerve head and macula in idiopathic intracranial hypertension

Clinical Neurology and Neurosurgery ◽

10.1016/j.clineuro.2011.05.015 ◽

2011 ◽

Vol 113 (10) ◽

pp. 839-843 ◽

Cited By ~ 10

Author(s):

Michael Waisbourd ◽

Igal Leibovitch ◽

Dafna Goldenberg ◽

Anat Kesler

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension ◽

Morphological Changes

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Imaging Features of Idiopathic Intracranial Hypertension in Children

Journal of Child Neurology ◽

10.1177/0883073816671855 ◽

2016 ◽

Vol 32 (1) ◽

pp. 120-126 ◽

Cited By ~ 20

Author(s):

Alexander J. P. W. Hartmann ◽

Bruno P. Soares ◽

Beau B. Bruce ◽

Amit M. Saindane ◽

Nancy J. Newman ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension ◽

Empty Sella ◽

Imaging Features ◽

Mri Findings ◽

Sinus Stenosis ◽

Magnetic Resonance Imaging Mri

Magnetic resonance imaging (MRI) signs of elevated intracranial pressure and idiopathic intracranial hypertension have been well characterized in adults but not in children. The MRIs of 50 children with idiopathic intracranial hypertension and 46 adults with idiopathic intracranial hypertension were reviewed for optic nerve head protrusion, optic nerve head enhancement, posterior scleral flattening, increased perioptic cerebrospinal fluid, optic nerve tortuosity, empty or partially empty sella, tonsillar herniation, enlargement of Meckel’s cave meningoceles, and transverse venous sinus stenosis(TSS). Compared to adolescents (11-17 years, n = 40) and adults (>17 years, n = 46), prepubescent children (<11 years, n = 10) had lower frequencies of scleral flattening (50% vs 89% and 85%, P = .02), increased perioptic cerebrospinal fluid (60% vs 84% and 89%, P = .08), optic nerve tortuosity (20% vs 46% and 59%, P = .07), empty or partially empty sella (56% vs 78% and 93%, P = .007), and TSS (67% vs 93% and 96%, P = .04). Children with idiopathic intracranial hypertension have similar MRI findings as adults, but they are less frequent in prepubescent children.

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Optical coherence tomography assessment of structural changes in the optic nerve head and peripapillary retina in idiopathic intracranial hypertension

Archives of the Balkan Medical Union ◽

10.31688/abmu.2019.54.2.07 ◽

2019 ◽

Vol 54 (2) ◽

pp. 267-273

Author(s):

Corina I. MERTICARIU ◽

◽

Florian BALTA ◽

Andrei MERTICARIU ◽

Radu CIULUVICA ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension ◽

Structural Changes ◽

Optical Coherence ◽

Peripapillary Retina

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Evaluation of 2D FLAIR Hyperintensity of the Optic Nerve and Optic Nerve Head and Visual Parameters in Idiopathic Intracranial Hypertension

Journal of Neuroradiology ◽

10.1016/j.neurad.2021.10.002 ◽

2021 ◽

Author(s):

i ◽

Aseem Sharma ◽

Fatima Alvi ◽

Jyoti Arora ◽

Matthew S. Parsons ◽

...

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension

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Anterior Lamina Cribrosa Surface Position in Idiopathic Intracranial Hypertension and Glaucoma

European Journal of Ophthalmology ◽

10.5301/ejo.5000806 ◽

2016 ◽

Vol 27 (1) ◽

pp. 55-61 ◽

Cited By ~ 13

Author(s):

Jenni M. Villarruel ◽

Xiao Q. Li ◽

Daniella Bach-Holm ◽

Steffen Hamann

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension ◽

Open Angle Glaucoma ◽

Fluid Pressure ◽

Lamina Cribrosa ◽

Enhanced Depth Imaging ◽

Mean Deviation ◽

Control Subjects

Purpose To compare the anterior lamina cribrosa (LC) surface position in patients with idiopathic intracranial hypertension (IIH), primary open-angle glaucoma (high-tension glaucoma [HTG] and normal-tension glaucoma [NTG]), and healthy controls using enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT). Methods This was a retrospective, cross-sectional observational study of 11 eyes with IIH, 20 eyes with HTG, 20 eyes with NTG, and 37 control eyes. Serial horizontal B-scans of the optic nerve head were obtained using EDI-OCT. The LC depth, defined as the distance from the Bruch membrane opening plane to the anterior LC surface, was manually measured on selected B-scans covering the central three-quarters of the optic nerve head in each eye. Results Mean LC depth in patients with IIH (325.2 ± 92.1 µm) was significantly (p<0.01) decreased compared to control subjects (387.8 ± 53.9 µm). In HTG, the mean LC depth (493.0 ± 115.2 µm) was significantly increased compared to NTG (376.6 ± 27.1 µm, p<0.05) and control subjects (332.7 ± 53.1 µm, p<0.001). The trans-LC pressure difference (TLPD) (intraocular pressure − cerebrospinal fluid pressure) was positively correlated with the LC depth (r = 0.96, p<0.001) and inversely correlated with visual field perimetric mean deviation in patients with IIH (r = -0.88, p<0.001). Conclusions The LC is positioned anteriorly in patients with IIH and posteriorly in glaucomatous eyes compared to normal controls. The positional changes of the LC could be a result of the TLPD between the intraocular and intracranial pressure compartments.

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OCT Based Interpretation of the Optic Nerve Head Anatomy and Prevalence of Optic Disc Drusen in Patients with Idiopathic Intracranial Hypertension (IIH)

Life ◽

10.3390/life11060584 ◽

2021 ◽

Vol 11 (6) ◽

pp. 584

Author(s):

Elisabeth Arnberg Wibroe ◽

Lasse Malmqvist ◽

Steffen Hamann

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Disc ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension ◽

Ganglion Cell Layer ◽

Cell Layer ◽

Fiber Layer ◽

Nerve Fiber Layer Thickness ◽

Optic Disc Drusen

We aimed to systematically examine the optic nerve head anatomy in patients with idiopathic intracranial hypertension (IIH) using a standardized optical coherence tomography (OCT) protocol. The study retrospectively included 32 patients diagnosed from 2014 to 2021 with IIH. Using OCT, in accordance with a standardized scanning protocol for patients with optic disc drusen, the presence of optic disc drusen, prelaminar hyperreflective lines, peripapillary hyperreflective ovoid mass-like structures, the retinal nerve fiber layer thickness, and macular ganglion cell layer volume was obtained. Optic disc drusen were found in 3.1%, hyperreflective lines in 31.3%, and peripapillary hyperreflective ovoid mass-like structures in 81.3% of all IIH patients at least three months after the time of diagnosis. We found no significant differences in retinal nerve fiber layer thickness or macular ganglion cell layer volume in patients with hyperreflective lines or PHOMS respectively compared to patients without hyperreflective lines (p = 0.1285 and p = 0.1835). In conclusion, the prevalence of optic disc drusen in IIH patients is similar to the reported prevalence in the general population. The high prevalence of hyperreflective lines and peripapillary hyperreflective ovoid mass-like structures in IIH patients suggest these structures be a result of crowding in the optic nerve head caused by papilledema.

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