, Volume 61, Issue 5, pp 595–602 | Cite as

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

  • Luca Pasquini
  • Domenico Tortora
  • Francesca Manunza
  • Maria Camilla Rossi Espagnet
  • Lorenzo Figà-Talamanca
  • Giovanni Morana
  • Corrado Occella
  • Andrea RossiEmail author
  • Mariasavina Severino
Paediatric Neuroradiology



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.


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.


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).


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.


Sturge–Weber syndrome Cavernous sinus Venous abnormalities Brain MRI 



Cavernous sinus


Cavernous sinus asymmetry index


Port-wine stain


Sturge–Weber syndrome


Vascular malformation



No funding was received for this study.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

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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)

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High resolution image (TIFF 11136 kb)
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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)

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High resolution image (TIFF 443 kb)
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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)

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High resolution image (TIFF 23429 kb)
234_2019_2182_Fig7_ESM.png (620 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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High resolution image (TIFF 244 kb)
234_2019_2182_MOESM5_ESM.docx (92 kb)
ESM 1 (DOCX 92 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Luca Pasquini
    • 1
  • Domenico Tortora
    • 2
  • Francesca Manunza
    • 3
  • Maria Camilla Rossi Espagnet
    • 4
  • Lorenzo Figà-Talamanca
    • 4
  • Giovanni Morana
    • 2
  • Corrado Occella
    • 3
  • Andrea Rossi
    • 2
    Email author
  • Mariasavina Severino
    • 2
  1. 1.Neuroradiology Unit, NESMOS Department, Sant’Andrea HospitalLa Sapienza UniversityRomeItaly
  2. 2.Neuroradiology Unit, IRCCS Istituto Giannina GasliniGenoaItaly
  3. 3.Dermatology Unit, IRCCS Istituto Giannina GasliniGenoaItaly
  4. 4.Neuroradiology Unit, Imaging DepartmentBambino Gesu’ Children’s HospitalRomeItaly

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