Nadhem KAMMOUN, Dora KHOMSI, Wièm DOUIRA-KHOMSI
Department of Paediatric Radiology, Béchir Hamza Children’s Hospital, Tunis, Tunisia *
Correspondences: Nadhem.kammoun@medecinesfax.org; dora.khomsi@gmail.com; khomsiwiem@yahoo.fr
A 22-year-old patient in her first pregnancy was referred to our department for prenatal ultrasound at 26 weeks of gestation.
Ultrasound revealed an anechoic, cystic structure above the cerebellum and below the thalamus with blood flow on Colour Doppler
(images 1 and 2).
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CORRECT ANSWER EXPLAINED BELOW | |
Correct answer to Q1 is: Vein of galen aneurysmal malformation. |
CORRECT ANSWER EXPLAINED BELOW | |
Correct answer to Q1 is: Vein of galen aneurysmal malformation. |
Ultrasound revealed an anechoic, interhemispheric structure posterior to the 3rd ventricle, with vascular flow on color Doppler. There were no other cerebral abnormalities. The diagnosis of aneurysm of the vein of Galen was retained.
Classically, it has a “racket” or “keyhole” shape, as the oval lumen of the malformation communicates posteriorly with a tubular structure corresponding to a venous drainage sinus.
Color Doppler confirms the vascular nature of the lesion, demonstrating bidirectional turbulent flow (1,2,3). It enables differential diagnosis with other midline fluid lesions of the brain with different prognoses (4).
Image 3: B-mode ultrasound shows increased cardiothoracic ratio and dilatation of the right heart cavities (red arrows).
Image 4: Colour Doppler shows tricuspid regurgitation and dilatation of the jugular vessels
Image 5: B-mode ultrasound (left: transverse; right: sagittal) shows a peritoneal effusion (white arrow). VB: gallbladder
CORRECT ANSWER EXPLAINED BELOW | |
Correct answer to Q2 is: Foetal heart failure caused by the cerebral vascular malformationAdditional discussionPatients with aneurysmal malformation of the vein of Galen have an underlying arteriovenous shunting of blood in the cerebral circulation. Ultrasound assessment therefore also includes evaluation of the hemodynamic impact of the intracranial shunt. The cardiac outcome is characterized by dilatation of the right cavities (image 3). Colour Doppler may reveal tricuspid regurgitation and dilatation of the jugular vessels (image 4). These signs can occur early, sometimes as early as the second trimester, and are pathognomonic of intracranial arteriovenous fistula (5). Other signs of right heart failure, such as hepatomegaly, serous intraperitoneal effusion (image 5) and hydramnios, are more rare and appear later. The final stage is fetoplacental hydrops (6). ConclusionVein of Galen aneurysm is a rare, intracranial, vascular, congenital malformation. We report an antenatal diagnosis by ultrasound at 26 weeks gestation. The neonatal prognosis is poor if fetal cardiac insufficiency or cerebral lesions (hydrocephaly, porencephaly, intracerebral hemorrhage) are present antenatally. Conflicts of Interest:“The authors declare no conflict of interest.” References
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CORRECT ANSWER EXPLAINED BELOW | |
Correct answer to Q2 is: Foetal heart failure caused by the cerebral vascular malformationAdditional discussionPatients with aneurysmal malformation of the vein of Galen have an underlying arteriovenous shunting of blood in the cerebral circulation. Ultrasound assessment therefore also includes evaluation of the hemodynamic impact of the intracranial shunt. The cardiac outcome is characterized by dilatation of the right cavities (image 3). Colour Doppler may reveal tricuspid regurgitation and dilatation of the jugular vessels (image 4). These signs can occur early, sometimes as early as the second trimester, and are pathognomonic of intracranial arteriovenous fistula (5). Other signs of right heart failure, such as hepatomegaly, serous intraperitoneal effusion (image 5) and hydramnios, are more rare and appear later. The final stage is fetoplacental hydrops (6). ConclusionVein of Galen aneurysm is a rare, intracranial, vascular, congenital malformation. We report an antenatal diagnosis by ultrasound at 26 weeks gestation. The neonatal prognosis is poor if fetal cardiac insufficiency or cerebral lesions (hydrocephaly, porencephaly, intracerebral hemorrhage) are present antenatally. Conflicts of Interest:“The authors declare no conflict of interest.” References
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