Abstract
Purpose
Enlargement of deep cerebral veins and choroid plexus engorgement are frequently reported in Sturge–Weber syndrome. We aim to describe cavernous sinus involvement in patients with this syndrome and to identify possible clinical-neuroimaging correlations.
Methods
Sixty patients with Sturge–Weber syndrome (31 females, mean age 4.5 years) and 120 age/sex-matched controls were included in this retrospective study. We performed a visual analysis to identify patients with asymmetric cavernous sinus enlargement. Then, we measured on axial T2WI the left (A), right (B), and bilateral (LL) transverse diameters of the cavernous sinus. We calculated the module of the difference |A-B| and the cavernous sinus asymmetry index as the ratio |A-B|/LL. Differences among groups were assessed by Mann–Whitney U and Kruskal–Wallis tests. Clinicoradiological associations were evaluated by Fisher exact test.
Results
We found seven subjects (11.6%) with asymmetric CS enlargement. The |A-B| and cavernous sinus asymmetry index were higher in patients with asymmetric CS enlargement compared with controls and patients without visible CS abnormalities (pB < 0.05). Asymmetric CS enlargement was always ipsilateral to facial port-wine stains (7/7), and, when present, to leptomeningeal vascular malformations (4/7). It was significantly associated with ipsilateral bone marrow changes (p = 0.013) and dilated veins (p = 0.002). Together with brain atrophy and deep venous dilatation, this sign was associated with neurological deficits (p < 0.05).
Conclusions
We expanded the spectrum of venous abnormalities in SWS, showing the presence of asymmetric cavernous sinus enlargement in more than one tenth of patients, likely related to increased venous drainage.
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Abbreviations
- CS:
-
Cavernous sinus
- CSAI:
-
Cavernous sinus asymmetry index
- PWS:
-
Port-wine stain
- SWS:
-
Sturge–Weber syndrome
- VM:
-
Vascular malformation
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Online Fig. 1.
Cavernous sinus measurements. A) Axial T2-weighted image at the level of pituitary gland shows the transverse diameter of the left sinus (A), measured from the left lateral margin of the pituitary gland to the left lateral border of the cavernous sinus dura mater, and the transverse diameter of the right sinus (B), measured from the right lateral margin of the pituitary gland to the right lateral border of the cavernous sinus dura mater. B) The same axial T2-weighted image demonstrates the bilateral transverse diameter (LL), measured between the left lateral margin of the left cavernous sinus and the right lateral margin of the right cavernous sinus. (PNG 983 kb)
Online Fig. 2.
Bland Altman plot displaying the agreement between measurements performed by two readers in patients and controls for the transverse diameter of the left sinus (A), the transverse diameter of the right sinus (B), and the bilateral transverse diameter (C). The mean difference between measurements is marked by the continuous line, and almost all subjects lie within the 95% confidence intervals (marked with the dashed lines). (PNG 1312 kb)
Online Fig. 3.
Bar graphs showing the differences in the transverse diameters of the left and right sinuses, the bilateral transverse diameters, the |A-B|, and the CSAI between patients and controls. Note that despite differences in the median of A, B, LL diameters, |A-B| and CSAI are small, the spread of values in the SWS group is greater than for controls. (PNG 7939 kb)
ESM 4.
Box plots demonstrating the differences of |A-B| (A) and CSAI (B) between SWS patients with normal cavernous sinus (group 1a), SWS with asymmetric enlargement of the cavernous sinus (group 1b) and controls (group 2). (PNG 619 kb)
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Pasquini, L., Tortora, D., Manunza, F. et al. Asymmetric cavernous sinus enlargement: a novel finding in Sturge–Weber syndrome. Neuroradiology 61, 595–602 (2019). https://doi.org/10.1007/s00234-019-02182-4
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DOI: https://doi.org/10.1007/s00234-019-02182-4