Case of the Week #523

Ho F (1); Godefroy C (1); Cuillier F (2); Alessandri JL (3)

Affiliations
(1) Radiologist, private sector, 97400 Saint-Denis, Reunion Island, France.
(2) Department of Obstetrics, Felix Guyon Hospital, Reunion Island, France.
(3) Department of Paediatrics, Felix Guyon Hospital, 97400 Saint-Denis, Reunion Island, France.

Posting Dates: August 27, 2020 - September 17, 2020

Case Report: A patient without previous personal, family and obstetric history presented for evaluation. Previous ultrasound examinations at 12, 22 and 28 weeks gestation were normal. Ultrasound at 31 weeks gestation revealed the following anomalies:

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We performed an MRI at 31 weeks gestation.

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Image 6: Coronal T2 image
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Image 7: Coronal T2 image
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Image 8: Coronal T2 image
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Image 9: Coronal T2 image
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Image 10: Coronal T2 image
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Image 11: Axial T2 image
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Image 12: Axial T2 image
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Image 13: Axial T2* image
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Image 14: Axial T2* image
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Image 15: Axial and coronal T1 image
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Image 16: Axial and coronal T1 image

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Answer

We present a case of dural sinus malformation with thrombosis of the right lateral sinus and venous infarction of the adjacent brain parenchyma.

Based on ultrasound, our first hypothesis was intracranial teratoma, with major mass effect and hydrocephaly. There was cardiomegaly secondary to vascular steal syndrome with mild pleural effusion and mild ascites.

MRI was performed and revealed:

  • A thin line of cerebrospinal fluid best seen in coronal view next to the frontal right lobe and parietal right lobe, separating the mass from the brain parenchyma itself : most likely an extra axial mass.
  • Intra axial brain damage as well on the adjacent cortex.
  • The mass mainly showed hemorraghic signal, with characteristic black hypointense signal on T2* images. The mixed signal in T1 images, and also fluid-fluid levels on all sequences suggested continuous hemorrhage since the blood signal varies according to its age.

Our final diagnosis was dural sinus malformation with thrombosis, of the right lateral sinus in this case, with venous infarction of the adjacent brain parenchyma. The major mass effect, brain damage, and associated signs of cardiac failure bore a poor prognosis. There was therefore very little hope for spontaneous regression without sequelae.

The parents opted for normal delivery at 38 weeks gestation. The newborn passed away 2 days after delivery and autopsy was denied.

References
[1] Andreeva E. "Dural sinus thrombosis." TheFetus.net. https://thefetus.net/content/dural-sinus-thrombosis. Publish Date 7/2017.
[2] Ha N. "Dural sinus thrombosis." TheFetus.net. https://thefetus.net/content/dural-sinus-thrombosis-1. Publish Date 8/2017.
[3] Laurichesse Delmas H, Winer N, Gallot D, et al. Prenatal diagnosis of thrombosis of the dural sinuses: report of six cases, review of the literature and suggested management. Ultrasound Obstet Gynecol. 2008 Aug;32(2):188-98.

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