Luca Pasquini1, Domenico Tortora2, Francesca Manunza3, Maria Camilla Rossi Espagnet4, Lorenzo Figà-Talamanca4, Giovanni Morana2, Corrado Occella3, Andrea Rossi5, Mariasavina Severino2. 1. Neuroradiology Unit, NESMOS Department, Sant'Andrea Hospital, La Sapienza University, Rome, Italy. 2. Neuroradiology Unit, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16147, Genoa, Italy. 3. Dermatology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 4. Neuroradiology Unit, Imaging Department, Bambino Gesu' Children's Hospital, Rome, Italy. 5. Neuroradiology Unit, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16147, Genoa, Italy. andrearossi@gaslini.org.
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.
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.
Authors: Matthew D Shirley; Hao Tang; Carol J Gallione; Joseph D Baugher; Laurence P Frelin; Bernard Cohen; Paula E North; Douglas A Marchuk; Anne M Comi; Jonathan Pevsner Journal: N Engl J Med Date: 2013-05-08 Impact factor: 91.245
Authors: Young H Lim; Antonella Bacchiocchi; Jingyao Qiu; Robert Straub; Anna Bruckner; Lionel Bercovitch; Deepak Narayan; Jennifer McNiff; Christine Ko; Leslie Robinson-Bostom; Richard Antaya; Ruth Halaban; Keith A Choate Journal: Am J Hum Genet Date: 2016-07-28 Impact factor: 11.025
Authors: P D Griffiths; M B Boodram; S Blaser; F Altomare; J R Buncic; A V Levin; V Jay; D Armstrong; D Harwood-Nash Journal: AJNR Am J Neuroradiol Date: 1996-04 Impact factor: 3.825