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Published in: Neuroradiology 5/2019

01-05-2019 | Cranial MRI | Paediatric Neuroradiology

Asymmetric cavernous sinus enlargement: a novel finding in Sturge–Weber syndrome

Authors: Luca Pasquini, Domenico Tortora, Francesca Manunza, Maria Camilla Rossi Espagnet, Lorenzo Figà-Talamanca, Giovanni Morana, Corrado Occella, Andrea Rossi, Mariasavina Severino

Published in: Neuroradiology | Issue 5/2019

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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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Metadata
Title
Asymmetric cavernous sinus enlargement: a novel finding in Sturge–Weber syndrome
Authors
Luca Pasquini
Domenico Tortora
Francesca Manunza
Maria Camilla Rossi Espagnet
Lorenzo Figà-Talamanca
Giovanni Morana
Corrado Occella
Andrea Rossi
Mariasavina Severino
Publication date
01-05-2019
Publisher
Springer Berlin Heidelberg
Published in
Neuroradiology / Issue 5/2019
Print ISSN: 0028-3940
Electronic ISSN: 1432-1920
DOI
https://doi.org/10.1007/s00234-019-02182-4

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