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NEURORADIOLOGY Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 753-756
Prenatal evaluation of vein of galen malformation with three dimensional doppler angiography - a case report


Super Scans, 31-A, Kaliappa Pillai Street, Tuticorin - 628 001, India

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Date of Submission15-Jun-2006
Date of Acceptance10-Aug-2006
 

Keywords: Vein of Galen malformation, Color Doppler, prenatal evaluation

How to cite this article:
Shanmugam S, Bhagavati A. Prenatal evaluation of vein of galen malformation with three dimensional doppler angiography - a case report. Indian J Radiol Imaging 2006;16:753-6

How to cite this URL:
Shanmugam S, Bhagavati A. Prenatal evaluation of vein of galen malformation with three dimensional doppler angiography - a case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:753-6. Available from: http://www.ijri.org/text.asp?2006/16/4/753/32340

   Introduction Top


Vein of Galen malformation, also known as vein of Galen aneurysm is a rare congenital anomaly of intracranial circulation that constitute 1% of all intra cranial vascular malformation. The widespread use of routine antenatal ultrasound examination has enabled antenatal detection of Vein of Galen Malformation. Prenatal diagnosis of this vascular anomaly has been greatly facilitated by the use of color Doppler sonography, which is crucial for differentiating this lesion from other cystic lesions of the fetal brain, because vein of Galen malformation is the only lesion that clearly displays blood flow within it. Three Dimensional (3D) Doppler angiography adds useful information in the prenatal evaluation of vein of Galen malformation because it provides images similar to those produced by conventional neonatal angiography.


   Case History Top


32 Yr. old primigravida with 36 weeks of amenorrhoea was referred for antenatal ultrasonography as the size of uterus was bigger than the period of amenorrhoea. Two dimensional gray scale ultrasound examination revealed single live intrauterine pregnancy of 36-37 weeks (corresponding to the period of amenorrhoea) in breech presentation. There was evidence of polyhydramnios (AFI = 22.9). Fetal anatomical survey revealed oval anechoic structure in fetal brain superior to the level of thalamus in midline (Fig 1).

Color Doppler study of the above structure revealed turbulent blood flow within the above anechoic structure (Fig 2).

Color Power Doppler examination revealed the above lesion communicating with the straight sinus and also visualization of color flow inside transverse sinuses in both sides (Fig 3).

3D Color Doppler angiography in marked contrast to 2D color Doppler revealed a mesh of vessels inside the lesion depicting the true nature of the lesion. The dilated vein of Galen draining the above lesion into straight sinus is identified. Color flow inside both transverse sinuses is also demonstrated (Fig 4).

3D angiography on Power Doppler mode further delineated the feeding vessels from internal carotid system entering the above vascular region. The entire angio architecture of the lesion with its communications is clearly shown by 3D power Doppler angiography. (Fig 5).

The above findings were diagnostic of vein of Galen malformation. Fetal heart was normal and there was no evidence of fetal hydrops. No other fetal anatomical defect was noted. Placenta was normal in thickness and echo texture. On the third day after the EDD from LMP, the baby was delivered by cesarean section. The birth weight of the baby was 3.5 Kg. 2D and color Doppler Ultrasonography of brain through the anterior fontonelle of the baby on day 2 after birth confirmed the presence of vein of Galen malformation (Fig 6).

The baby developed breathlessness after birth and features of cardiac failure subsequently. In spite of intensive treatment, the baby died on day 6 after birth.


   Discussion Top


Vein of Galen (great cerebral vein) formed by the union of two internal cerebral veins, is a short median trunk below the splenium of corpus callosum. It passes caudally and distally to merge with the inferior sagittal sinus forming the straight sinus. Vein of Galen malformation was first described by Jager in1937. It is a complex arterio venous fistula caused by multiple abnormal communications between the system of vein of Galen and the cerebral arteries[1]

Three anatomical types described; arterio venous fistula, arterio venous malformation with ectasia of vein of Galen and varix of the vein of Galen [2] The Arteriovenous fistula frequently manifests in the neonatal period with cardiac failure. Both the ectasia and the varix appear to present later in life with intra cranial bleeding and do not present in the neonate with cardiac failure [3].

Though vein of Galen malformation is a developmental anomaly, it is often visible only in third- trimester sonography. Many cases are detected only in the neonatal period when the child presents with cardiac failure [4].

The most striking ultrasound feature is the detection of a cerebral midline tubular anechoic structure superior to the thalamus, which is contiguous with the dilated sagittal sinus (the "comet tail" or "keyhole" sign). [5] Color Doppler is crucial for differentiating this lesion from other cystic lesions of fetal brain because the vein of Galen malformation is the only lesion that clearly displays blood flow within it [6]. 3D ultrasound clearly localizes the lesion antero superior to the third ventricle in sagittal and coronal planes. 3D

3D Doppler angiography further characterizes the vascular anomaly in the fetal brain. 3D Doppler angiography gives high quality images comparable to those generated by conventional neonatal angiography for recognizing the dilated vein of Galen, the straight sinus and abnormal arterial vessels feeding the malformation. This method allows a three dimensional reconstruction of spatial vessels shown only partly on two dimensional color Doppler imaging.[4] Superimposed gray scale sonography (glass body mode) can also assist in the diagnosis because it allows exact localization of vessels in relation to fetal tissue structures [7].

In fetuses with vein of Galen malformation, associated high output cardiac failure is seen in 95% leading to hydrops. Hydrocephalus, sub arachnoid or intra ventricular hemorrhage may be seen in 5% [8]. One or more of the other sonographically detectable findings such as cardiomegaly, pericardial effusion, hepato splenomegaly, ascitis and poly hydramnios may also be present.

Fetuses with mild degrees of cardiac insufficiency are prone to have decompensation in the early neonatal period. This could be mediated by the unique features of fetal and neonatal circulations. In the fetus, the placenta is the organ with the lowest resistance to blood flow and more blood from aorta is directed to placenta than to fetal brain. When the cord if clamped after delivery, there is abrupt circulatory changes characterized by redistribution of blood flow, resulting in low resistance in the fetal lungs and brain, which increases the blood flow through the vein of Galen malformation considerably.

This particular hemo dynamic phenomenon could explain the rapid deterioration in neonates with borderline cardiac insufficiency or could lead to neonatal death in those already having cardiac failure.[5] as seen in our case. Vein of Galen malformation should be strongly considered in the differential diagnosis of cardiac insufficiency in the first week of life [9]

The prognosis of the Vein of Galen malformation depends essentially on fetal cardiac function and associated brain ischemic injuries. Even in the absence of these complications, the postnatal outcomes of children with this malformation are not uniformly good.[10]. The size of arterio venous fistula may indicate the severity of the disease and possibly a better indicator of the prognosis. As the shape of the lesion is irregular, diameter of the lesion may not truly reflect the size of the lesion. In 3D ultrasound we are able to trace the outline of the lesion in all planes and reconstruct the cast of the lesion and the computer in the Scanner accurately calculates the volume of the lesion by VOCAL. (Fig 7). The volume of the lesion in our case was 7.67cm 3 .

Thus, 3D Doppler Angiography adds useful information in the antenatal evaluation of vein of Galen malformation by clearly depicting the angio architecture of the lesion. 3D ultrasound is also useful to calculate accurately the volume of the lesion. Further studies are required to correlate above anatomic features of the shunts with the post natal outcome.

 
   References Top

1.Brunelle F. Arteriovenous malformation of the vein of Galen in children Pediatr Radiol 1997: 27:501-513.  Back to cited text no. 1    
2.Lasjaunias P, Manelfe C, Terbrugge K et al: Endovascular treatment of cerebral Arteriovenous malformations. Neurosurgery 1986;9::265-275  Back to cited text no. 2    
3.Rayboud CA, Hold JK, Strother CM, Aneurysm of the vein of Galen. Angiographic study and morphologic considerations. Neurochirugie 1987;33:302-308  Back to cited text no. 3    
4.Chaoui R, Kalache KD, Hartung J. Application of three dimensional; power Doppler ultrasound in prenatal diagnosis. Ultrasound Obstet Gynecol. 2001; 17:22-29  Back to cited text no. 4    
5.Sepulveda W, Platt CC, Fisk NM. Prenatal diagnosis of cerebral venous Arteriovenous malformation using color Doppler ultrasonography: case report and review of the literature. Ultrasound Obster Gynecol 1995; 6:282-286  Back to cited text no. 5    
6.Pilu G, Falco P, Perolo A, et al. Differential diagnosis and outcome of fetal intracranial hypoechoic lesions: report of 21 cases. Ultrasound Obstet Gynecol 1997; 9:229-236.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Waldo S, Tina V, Jayshree P, et al. Vein of Galen malformation prenatal evaluation with three dimensional power Doppler angiography. J Ultrasound Med 2003; 22: 1395-1398.  Back to cited text no. 7    
8.Volpe JJ. Brain tumors and vein of Galen malformation. Neurology of the newborn; Neuronal Proliferation, Migration, Organisation and Myelination. 3rd ed. Philadelphia: WB Saunders, 1995: 80-2-806  Back to cited text no. 8    
9.Mitchell PJ, Rosenfield JV, Dargaville P, et al. Endovascular management of vein of Galen malformations presenting in the neonatal period. AJNR Am J Neuroradiol 2001;22: 1403-1409  Back to cited text no. 9    
10.Rodrigo R, Alexandra B, Maries CA, et al. Perinatal three dimensional color power Doppler ultrasonography of vein of Galen aneurysms. J Ultrasound Med 2003;22:1357-1362  Back to cited text no. 10    

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Correspondence Address:
S Shanmugam
Super Scans, Ss Centre, 31-A, Kaliappa Pillai Street, Tuticorin - 628 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32340

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]

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