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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

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.

Keywords

Sturge–Weber syndrome Cavernous sinus Venous abnormalities Brain MRI 

Abbreviations

CS

Cavernous sinus

CSAI

Cavernous sinus asymmetry index

PWS

Port-wine stain

SWS

Sturge–Weber syndrome

VM

Vascular malformation

Notes

Funding

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)
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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)

References

  1. 1.
    Comi AM (2015) Sturge–Weber syndrome. Handb Clin Neurol:157–168Google Scholar
  2. 2.
    Waelchli R, Aylett SE, Robinson K, Chong WK, Martinez AE, Kinsler VA (2014) New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol 171:861–867.  https://doi.org/10.1111/bjd.13203 CrossRefGoogle Scholar
  3. 3.
    Goyal P, Mangla R, Gupta S, Malhotra A, Almast J, Sapire J, Kolar B (2018) Pediatric congenital cerebrovascular anomalies. J Neuroimaging.  https://doi.org/10.1111/jon.12575
  4. 4.
    Dymerska M, Kirkorian AY, Offermann EA, Lin DD, Comi AM, Cohen BA (2017) Size of facial port-wine birthmark may predict neurologic outcome in Sturge-Weber syndrome. J Pediatr 188:205–209.e1.  https://doi.org/10.1016/j.jpeds.2017.05.053 CrossRefGoogle Scholar
  5. 5.
    Roach ES (1992) Neurocutaneous syndromes. Pediatr Clin N Am 39:591–620CrossRefGoogle Scholar
  6. 6.
    Comi AM (2011) Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. Neurologist 17:179–184.  https://doi.org/10.1097/NRL.0b013e318220c5b6 CrossRefGoogle Scholar
  7. 7.
    Benedikt RA, Brown DC, Walker R et al Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol 14:409–415Google Scholar
  8. 8.
    Adams ME, Aylett SE, Squier W, Chong W (2009) A spectrum of unusual neuroimaging findings in patients with suspected Sturge-Weber syndrome. Am J Neuroradiol 30:276–281.  https://doi.org/10.3174/ajnr.A1350 CrossRefGoogle Scholar
  9. 9.
    Warne RR, Carney OM, Wang G et al (2018) The bone does not predict the brain in Sturge-Weber syndrome. Am J Neuroradiol.  https://doi.org/10.3174/ajnr.A5722
  10. 10.
    Griffiths PD, Blaser S, Boodram MB, Armstrong D, Harwood-Nash D (1996) Choroid plexus size in young children with Sturge-Weber syndrome. AJNR Am J Neuroradiol 17:175–180Google Scholar
  11. 11.
    Griffiths PD, Boodram MB, Blaser S, Altomare F, Buncic JR, Levin AV, Jay V, Armstrong D, Harwood-Nash D (1996) Abnormal ocular enhancement in Sturge-Weber syndrome: correlation of ocular MR and CT findings with clinical and intracranial imaging findings. AJNR Am J Neuroradiol 17:749–754Google Scholar
  12. 12.
    Miao Y, Juhász C, Wu J, Tarabishy B, Lang Z, Behen ME, Kou Z, Ye Y, Chugani HT, Hu J (2011) Clinical correlates of white matter blood flow perfusion changes in Sturge-Weber syndrome: a dynamic MR perfusion-weighted imaging study. Am J Neuroradiol 32:1280–1285.  https://doi.org/10.3174/ajnr.A2540 CrossRefGoogle Scholar
  13. 13.
    Sari S, Sari E, Akgun V, Ozcan E, Ince S, Saldir M, Babacan O, Acikel C, Basbozkurt G, Ozenc S, Yesilkaya S, Kilic C, Kara K, Vurucu S, Kocaoglu M, Yesilkaya E (2014) Measures of pituitary gland and stalk: from neonate to adolescence. J Pediatr Endocrinol Metab 0:1071–1076.  https://doi.org/10.1515/jpem-2014-0054 CrossRefGoogle Scholar
  14. 14.
    Whitehead MT, Vezina G (2015) Osseous intramedullary signal alteration and enhancement in Sturge-Weber syndrome: an early diagnostic clue. Neuroradiology 57:395–400.  https://doi.org/10.1007/s00234-015-1488-6 CrossRefGoogle Scholar
  15. 15.
    Razek AAKA, Castillo M (2009) Imaging lesions of the cavernous sinus. Am J Neuroradiol 30:444–452.  https://doi.org/10.3174/ajnr.A1398 CrossRefGoogle Scholar
  16. 16.
    Goyal P, Lee S, Gupta N, Kumar Y, Mangla M, Hooda K, Li S, Mangla R (2018) Orbital apex disorders: imaging findings and management. Neuroradiol J 31:104–125.  https://doi.org/10.1177/1971400917740361 CrossRefGoogle Scholar
  17. 17.
    Shirley MD, Tang H, Gallione CJ, Baugher JD, Frelin LP, Cohen B, North PE, Marchuk DA, Comi AM, Pevsner J (2013) Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med 368:1971–1979.  https://doi.org/10.1056/NEJMoa1213507 CrossRefGoogle Scholar
  18. 18.
    Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HPW, Bischoff J, Greene AK, Warman ML (2016) Somatic activating mutations in GNAQ and GNA11 are associated with congenital hemangioma. Am J Hum Genet 98:789–795.  https://doi.org/10.1016/j.ajhg.2016.03.009 CrossRefGoogle Scholar
  19. 19.
    Van Raamsdonk CD, Griewank KG, Crosby MB et al (2010) Mutations in GNA11 in uveal melanoma. N Engl J Med 363:2191–2199.  https://doi.org/10.1056/NEJMoa1000584 CrossRefGoogle Scholar
  20. 20.
    Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, Yale Center for Mendelian Genomics, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA (2016) GNA14 somatic mutation causes congenital and sporadic vascular tumors by MAPK activation. Am J Hum Genet 99:443–450.  https://doi.org/10.1016/j.ajhg.2016.06.010 CrossRefGoogle Scholar
  21. 21.
    Bentson JR, Wilson GH, Newton TH (1971) Cerebral venous drainage pattern of the Sturge-Weber syndrome. Radiology 101:111–118.  https://doi.org/10.1148/101.1.111 CrossRefGoogle Scholar
  22. 22.
    Raybaud C (2010) Normal and abnormal embryology and development of the intracranial vascular system. Neurosurg Clin N Am 21:399–426.  https://doi.org/10.1016/j.nec.2010.03.011 CrossRefGoogle Scholar
  23. 23.
    Mitsuhashi Y, Hayasaki K, Kawakami T et al (2016) Dural venous system in the cavernous sinus: a literature review and embryological, functional, and endovascular clinical considerations. Neurol Med Chir (Tokyo) 56:326–339.  https://doi.org/10.2176/nmc.ra.2015-0346 CrossRefGoogle Scholar
  24. 24.
    Thavikulwat AT, Edward DP, AlDarrab A, Vajaranant TS (2019) Pathophysiology and management of glaucoma associated with phakomatoses. J Neurosci Res 97:57–69.  https://doi.org/10.1002/jnr.24241 CrossRefGoogle Scholar
  25. 25.
    Bayoumi NHL, Elsayed EN (2018) Glaucoma in children with facial port wine stain. Eur J Ophthalmol:112067211881966.  https://doi.org/10.1177/1120672118819668

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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