Vein of Galen Malformations: Epidemiology, Clinical Presentations, Management

Recinos, Pablo F.; Rahmathulla, Gazanfar; Pearl, Monica S.; Recinos, Violette Renard; Jallo, George I.; Gailloud, Philippe; Ahn, Edward S.

doi:10.1016/j.nec.2011.09.006

Cited by 73 publications

(63 citation statements)

References 44 publications

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“…Spinal actinomycosis is unusual and spread occurs by direct invasion or hematological dissemination. [1] Direct spinal cord involvement has not yet been described in the literature and hence this report.…”

Section: Vein Of Galen Aneurysmal Malformation: Strategy Transarteriamentioning

confidence: 85%

“…They are characterized by multiple arteriovenous shunts draining into a dilated median prosencephalic vein (MProsV) of Markowski, and not the vein of Galen. [1][2][3] A 15-month-old male child presented with seizures disorder, abnormally dilatation of the scalp and facial veins, and a mild increase in head circumference [ Figure 1a]. Three-dimensional time-of-flight (3D TOF) magnetic resonance angiography (MRA) done at the age of 9 weeks revealed the VGAM [ Figure 1b].…”

Section: Vein Of Galen Aneurysmal Malformation: Strategy Transarteriamentioning

confidence: 99%

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Vein of Galen aneurysmal malformation

Cai

Yang

et al. 2012

Neurology India

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Section: Vein Of Galen Aneurysmal Malformation: Strategy Transarteriamentioning

confidence: 85%

Section: Vein Of Galen Aneurysmal Malformation: Strategy Transarteriamentioning

confidence: 99%

Vein of Galen aneurysmal malformation

Cai

Yang

et al. 2012

Neurology India

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“…By decreasing flow to the VGM, perfusion to the kidneys and heart improves, venous hypertension in the pulmonary circulation can be decreased, and persistent right-to-left shunts can close. Management options to decrease flow include diuretics, inotropic agents and vasodilators 4. Their use offers stabilisation before intervention and contributes in reducing neurological injury.…”

Section: Discussionmentioning

confidence: 99%

Necrotising enterocolitis in a full-term infant with reversed diastolic flow in the descending aorta: what is the diagnosis?

et al. 2012

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SUMMARYVein of Galen malformations (VGM) are rare intracranial vascular anomalies that constitute 1% of all intracranial vascular malformations. Untreated VGM have a very poor prognosis. A high proportion of patients who present in the neonatal period rapidly deteriorate and succumb to congestive cardiac failure. The prenatal diagnosis and possible in utero referral to highly specialised centre of postnatal care have resulted in considerable improvement in prognosis. The authors present a case of a postnatally diagnosed VGM in a fullterm infant presenting with progressive cardiac failure and necrotising enterocolitis secondary to gut hypoperfusion. They emphasise the importance of prenatal diagnosis by pulsed wave Doppler and colourvelocity imaging for subsequent referral to a centre of excellence in a multidisciplinary approach involving neonatologists, paediatric cardiologists and interventional radiologists in order to optimise the management and limit the neurological sequelae in children with this type of malformation. BACKGROUND

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“…from 10 to 20%, and developmental delay or more serious neurological sequelae in at least 26% of survivors [5][6][7][8] . One recent report described intraprocedural complications in 43% of infants undergoing endovascular treatment, and postprocedural hydrocephalus requiring intervention in 24% [9] .…”

mentioning

confidence: 99%

“…One recent report described intraprocedural complications in 43% of infants undergoing endovascular treatment, and postprocedural hydrocephalus requiring intervention in 24% [9] . CAVMs are a known cause of high cardiac output heart failure in the neonate [10,11] , with cardiac dilation [12] , as well as pulmonary hypertension and, in extreme cases, multisystem organ failure [6] . Pulmonary hypertension is presumed to be due to increased pulmonary blood flow [13,14] , and along with heart failure is predictive of worse prognosis [14,15] .…”

mentioning

confidence: 99%

Cardiac Findings in the Fetus with Cerebral Arteriovenous Malformation Are Associated with Adverse Outcome

et al. 2016

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Objectives: To assess cardiac sequelae of fetal cerebral arteriovenous malformations (CAVMs) and evaluate any association with outcomes. Methods: We retrospectively analyzed cardiac structure and function in fetuses with CAVMs who underwent fetal echocardiography (October 1999 to August 2015, n = 11), and compared them with normal controls. Results: The median gestational age was 36 weeks (range 18-38). Common abnormal findings included dilated superior vena cava (100%) and right atrium (82%), reduced middle cerebral artery pulsatility index (86%), tricuspid regurgitation (82%), and right ventricular (RV) dysfunction (64%). Hydrops was present in 1 fetus, who did not survive. The median cardiothoracic ratio (CTR) was 0.36 (0.29-0.45, n = 10); the median combined cardiac output indexed to estimated fetal weight (iCCO) was 565 ml/kg/min (379-1,565, n = 7). Of the 11 fetuses, 1 patient elected for termination, and 5 suffered neonatal demise. Comparing survivors (n = 5) and nonsurvivors (n = 6), a larger tricuspid valve (TV) z-score (p = 0.009) and RV dysfunction (p = 0.015) were associated with nonsurvival, and nonsurvivors had a higher iCCO than controls (990 vs. 550 ml/kg/min, p = 0.035). A larger difference between the TV and mitral valve z-scores (surrogate for RV dilation, p = 0.052), and CTR >0.38 (p = 0.0762) tended towards nonsurvival. Conclusion: CAVMs impose volume load on the fetal circulation, mainly affecting right heart structures. Increased right heart dilation and dysfunction are associated with nonsurvival.

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Vein of Galen Malformations: Epidemiology, Clinical Presentations, Management

Cited by 73 publications

References 44 publications

Vein of Galen aneurysmal malformation

Vein of Galen aneurysmal malformation

Necrotising enterocolitis in a full-term infant with reversed diastolic flow in the descending aorta: what is the diagnosis?

Cardiac Findings in the Fetus with Cerebral Arteriovenous Malformation Are Associated with Adverse Outcome

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