Article Figures & Data
Figures
- Fig 1.
A 15-year-old boy with double vision and reduced acuity has bilateral papilledema. A, On axial T2WI, ventriculomegaly with periventricular CSF flow (black arrows) due to an obstructing tumor within the fourth ventricle is found. Optic nerve sheath hydrops, elongation of the optic nerve (white arrows in A), and optic papilla protrusion are seen (black arrowhead). B, Anteroposterior view of MIP of MRV depicts bilateral narrowings of the lateral RTS and LTS and the sigmoid sinus (arrows). C, On primary axial sections of MRV, the SOVs are enlarged (arrows, right SOV, 2.8-mm width). Medulloblastoma was found at surgery.
- Fig 2.
A 30-year-old woman with arterial hypertension and visual disturbances has bilateral papilledema. A, MR imaging of the orbit displays a flattened posterior sclera (arrowheads). B, ONS hydrops is present, and there is edema of the optic nerve as seen on the coronal STIR sequence measured 20 mm behind the globe (white arrows, ONS width of 5.4 mm). C, Slightly oblique MIP of MRV shows lengthy narrowings of the intracranial venous sinuses, especially of the superior sagittal sinus (arrows). Vision returns to normal with successful treatment of hypertension. On follow-up MR imaging 3 months later, ONS width is reduced to 4.8 mm, and the posterior sclera appears normal (not shown). D, The intracranial venous sinuses regain normal caliber, as shown by MRV.
- Fig 3.
A 28-year-old woman with venous sinus thrombosis secondary to in vitro fertilization. A, Signal intensity typical for thrombosed blood is seen in the SSS and the RTS on axial T2w (black arrow) but not in the LTS (white arrow). B, There is corresponding signal-intensity loss on MIP of MRV in the RTS and SSS (left oblique view). There is a stenosis in the LTS (white arrow) without evidence of a thrombus. C, ONS hydrops is present (not shown), and the height of the pituitary gland is reduced to 2.5 mm (coronal STIR). D and E, CSF pressure is 40 cm H2O. Six months later, partial recanalization of the RTS and SSS occurs following therapy with low-molecular heparin seen on T2WI (black arrow in D) and on MIPs of MRV (E). The stenosis in the LTS is believed to be the result of IH vanishing (white arrow in E). F, The pituitary height-weight returns to normal (4.5 mm).
- Fig 4.
Cranial MR imaging of a 7-year-old boy with congenital posthemorrhagic hydrocephalus before (A–D) and 7 days later after (E–H) correction of the distal part of an insufficient ventriculoperitoneal shunt (arrows in A and E). Standard imaging (axial T2WI in A and E; sagittal T2WI and FLAIR in B and F) does not reflect IH, and does not change after therapy. ONS hydrops (arrows in C, width of 6.8 mm measured in a plane 3 mm dorsal to the optic globe; upper normal limit is 6.3 mm) normalizes after therapy (arrows in G, width of 5.9 mm). Oblique views of MIP of MRV (D and H) display signal-intensity losses in the LTS and RTS and the SSS (arrows in D), also normalizing on follow-up (H). Bilateral papilledema resolves.
Tables
Patient Groupa Opening CSF Pressure on LP in cm H2O (mean) (range) Demographics Diagnosis, Clinical Signs, and Symptoms Group Size, Sex Patient Age (mean) (range) Diagnosis Papilledema Mild VI Severe VI Headache Nausea/Vomiting Other Symptoms All patients with IH 39.8 (24–70) n = 36 F (n = 23)M (n = 13) 40.9 yr 2–76 yr Meningeal disease (n = 10), thrombosis or occlusion of venous sinus or the dominant IJV (n = 10), intracranial tumor (n = 8), hydrocephalus (other than caused by tumor, n = 5), other (n = 5), see below, multiple (n = 3) 23/35 66% 20/36 56% 12/36 33% 30/36 83% 9/36 25% See below Patient Subgroup I 29.5 (22–38) n = 12 F (n = 7)M (n = 5) 41.6 yr 32–66 yr Occlusion of a venous sinus or IJV by meningioma or postoperatively (n = 3), viral meningitis (n= 3), bacterial meningitis (n = 1), venous sinus thrombosis (n = 1), excessive arterial hypertension (n = 1), head trauma with hygromas (n = 1), hydrocephalus (n = 1), minocycline medication (n = 1) 7/12 58% 7/12 58% 3/12 25% 12/12 100% 4/123 3% Fever, meningism (n = 4), transient aphasia (n = 3), vertigo (n = 2), transient apraxia (n = 1), cough (n = 1), hypacusis (n = 1), tinnitus (n = 1), reduced consciousness (n= 1), paraesthesia (n = 1) Patient Subgroup II 52 40–70 n = 10 F (n = 6)M (n = 4) 34.5 yr 2–62 yr Thrombosis of venous sinus or IJV (n = 4), hydrocephalus (n = 3), meningiosis (n = 2), neurosarcoid meningitis (n = 1) 7/97 8% 4/10 40% 5/10 50% 8/10 80% 3/10 30% Vertigo, dysaesthesia (n = 1), tinnitus (n = 1), double vision (n = 1), head enlargement (n = 1), unspecific feeling of illness (n = 1) Patient Subgroup III Not testedb n = 14 F (n = 10)M (n = 4) 44.8 yr 9–76 yr Meningioma alone or combined with stenosis or occlusion of a venous sinus (n = 4), venous sinus thrombosis alone or combined with brain abscess or dural metastasis (n = 3), brain tumor with occlusive hydrocephalus (n = 2), meningeosis (n = 2), arterial hypertension, PRES (n = 1), hydrocephalus (n = 1) 9/14 64% 9/14 64% 4/14 29% 10/14 71% 2/14 14% Double vision (n = 4), paresis (n = 4), reduced consciousness (n = 3), nystagmus (n = 1). vertigo (n = 1), fever (n = 1) Controls Not tested. n = 36 F (n = 23) M (n = 13) 39 yr 2–80 yr Mostly psychiatric Not tested 0/36 0% 0/36 0% 0/36 0% 0/36 0% a Patients with IH (n = 36) were subgrouped into those with CSF pressure <40 cm H2O (n = 12, group I), those with >40 cm H2O (n = 10, group II), and those without direct pressure measurements (n = 14, group III).
b In subgroup III, 3 patients were reported to have elevated CSF pressure intraoperatively.
Diameter of the ONS (mm)b Pituitary Height (mm) SOV Diameter (mm) Position 1 Position 2 Position 3 Position 4 Patients with IH (all, n = 36) Mean 7.0 6.1 5.4 5.1 3.1 2.3 SD 0.9 0.9 1.0 0.7 1.6 0.7 95% CI 6.7–7.3 5.8–6.4 5.1–5.7 4.9–5.3 2.6–3.6 2.0–2.5 ICC 0.832 0.84 0.866 0.84 0.863 0.389 Patient subgroup I (n = 12) Mean 7.2 6.2 5.3 5.1 3.2 2.2 CSF pressure 22–38 cm H2O SD 0.6 0.7 1.1 0.5 1.0 0.8 Patient subgroup II (n = 10) Mean 6.7 5.9 5.2 5.1 2.8 2.4 CSF pressure 40–70 cm H2O SD 0.6 0.7 0.8 0.7 1.3 0.5 Patient subgroup III (n = 14) Mean 7.0 6.2 5.7 5.0 3.2 2.2 No CSF pressure measurements SD 1.2 1.1 1.0 0.8 2.0 0.7 Controls (n = 36) Mean 5.8 4.9 4.4 4.1 5.2 1.7 SD 0.6 0.6 0.5 0.3 1.5 0.5 95% CI 5.6–6.0 4.7–5.1 4.3–4.6 4.0–4.3 4.7–5.6 1.5–1.8 ICC 0.76 0.728 0.78 0.732 0.893 0.634 a Results of all measurements differed significantly between all patients and controls (P < .01) and also between the different patient subgroups (I∼III) and controls (P < .05). No significant differences existed among results of patient subgroups I, II, and III.
b ONS diameter was measured 3, 6, 10, and 20 mm behind the globe (positions 1–4).
Patient Group Opening CSF Pressure on LP in cm H2O (mean) (range) CVOO ONS Hydropsb Reduced Pituitary Height (<2.6 mm) Flattened Posterior Sclera Optic Disc Protrusion Dilated SOV (<2.6 mm in diameter) Ventriculomegaly Periventricular CSF Flow Optic Nerve Edema Optic Nerve Elongation No. of Positive MRI Signs (mean) (range) All patients 39.8 Frequency, 34/36 33/36 20/36 23/36 12/36 11/36 7/36 5/36 5/36 2/36 4.3 (2–9) 24–70 Sensitivity, 94% 92% 56% 64% 33% 31% 19% 14% 14% 6% CI 95%, 81%–99% 76%–98% 38%–72% 46%–79% 19%–91% 16%–48% 8%–36% 5%–29% 5%–29% 1%–19% Specificity, 100% 89% 97% 78% 100% 97% 100% 100% 100% 100% 95% CI, 90%–100% 73%–96% 85%–100% 61%–90% 90%–100% 85%–100% 90%–100% 90%–100% 90%–100% 90%–100% OR 1007 88 44 6 37 15 18 13 13 5 95% CI, 47–21,741 18–424 5–355 2–17 2–658 2–127 1–338 1–240 1–240 .3–114 Agreement,c 100% 100% 91.7% 63.9% 77.8% 67% 91.7% 91.7% 69.4% 80.6% κc 1 1 0.888 0.484 0.675 0.338 0.852 0.79 0.47 0.533 Patient 29.5 Frequency 10/12 11/12 4/12 5/12 2/12 4/12 1/12 1/12 0/12 0/12 3.3 (2–6) subgroup I 22–38 83% 92% 33% 42% 17% 33% 8% 8% 0% 0% Patient 52 Frequency 10/10 9/10 7/10 8/10 6/10 3/10 3/10 1/10 3/10 1/10 5.1 (3–7) subgroup II 40–70 100% 90% 70% 80% 60% 30% 30% 10% 30% 10% Patient N.T. Frequency 14/14 13/14 9/14 10/14 4/14 4/14 3/14 3/14 2/14 1/14 4.5 (2–9) subgroup III 100% 93% 64% 71% 29% 29% 21% 21% 14% 7% Controls N.T. Frequency 0/36 4/36 35/36 8/36 0/36 1/36 0/36 0/36 0/36 0/36 0.4 (0–1) 0% 11% 3% 22% 0% 3% 0% 0% 0% 0% Agreementc 100% 78% 100% 69.4% 94.4% 94.4% 94.4% 97.2% 97.2% 94.4% a Frequency of all MRI signs differed between patients and controls (P< .01). Frequency of the MRI signs “optic disc protrusion” and “optic nerve edema” were more often found in patient group II compared with patient group I (P< .05). Frequencies of all MRI signs did not differ between patient subgroup III and the other patient groups.
b ONS hydrops was assumed if the diameter was above normal limits in ≥1 different position. Normal upper limits of ONS diameters were 6.4, 5.8, 5.1, and 4.8 mm in measurement positions 1, 2, 3, and 4 (3, 6, 10, and 20 mm behind the globe).
c Results of 3 readers used to determine the interobserver-reliability.
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