scholarly journals Vein of Galen Malformation with Cerebral Calcification: A Reversible Cause of Neurodegenerative Disease

Author(s):  
Stephen J. Phillips ◽  
Joseph M. Dooley ◽  
Peter R. Camfield

ABSTRACT:This paper reports two children who had mental retardation as the predominant manifestation of vein of Galen malformations. C.T. scans demonstrated extensive intracerebral calcification. Surgery reversed the progressive downhill course in one patient and may have arrested further deterioration in the other. The pathophysiological mechanisms underlying the clinical and radiographic features are discussed.

Perfusion ◽  
2021 ◽  
pp. 026765912110015
Author(s):  
Alex Robertson ◽  
Nagarajan Muthialu ◽  
Mike Broadhead

We present a dissection of the patent ductus arteriosus and pulmonary artery for surgical repair utilising cardiopulmonary bypass in the setting of vein of Galen malformation. Several strategies were employed to attenuate the cerebral shunt including pH-stat, high cardiac index, restrictive venous drainage, continuous ventilation and deep hypothermic circulatory arrest. The patient recovered from surgery with no apparent neurological sequelae.


Neurosurgery ◽  
1988 ◽  
Vol 22 (5) ◽  
pp. 908-910 ◽  
Author(s):  
Jane Matjasko ◽  
Walker Robinson ◽  
Daniel Eudaily

Abstract A 12-day-old infant in intractable cardiac failure due to a vein of Galen malformation was treated successfully with serial ligation of the majority of the vessels feeding the malformation. Despite some residual vascular supply to the malformation, the congestive heart failure has disappeared and growth and development have been normal over a 3-year follow-up period.


2018 ◽  
Vol 51 (3) ◽  
pp. 421-423 ◽  
Author(s):  
A. Yukhayev ◽  
N. Meirowitz ◽  
R. Madankumar ◽  
I. E. Timor-Tritsch ◽  
A. Monteagudo

PEDIATRICS ◽  
1967 ◽  
Vol 40 (4) ◽  
pp. 586-589
Author(s):  
George Link Spaeth ◽  
G. Winston Barber

The prevalence of homocystinuria in patients with mental retardation institutionalized in the United States is about 0.02%; this is lower than a previous estimation from Northern Ireland (0.3%). On the other hand, about 5% of patients with dislocated lenses may be expected to have the disease. A silver-nitroprusside test which is almost completely specific for homocystine has been evaluated. It should be useful for screening.


2018 ◽  
Vol 35 (11) ◽  
pp. 1868-1871 ◽  
Author(s):  
Jay Relan ◽  
Saurabh K. Gupta ◽  
Anita Saxena

1986 ◽  
Vol 22 (2) ◽  
pp. 195
Author(s):  
T K Eun ◽  
S S Cha ◽  
S S Han ◽  
D H Chung

2016 ◽  
Vol 9 (9) ◽  
pp. 880-886
Author(s):  
Dan Meila ◽  
Katharina Melber ◽  
Dominik Grieb ◽  
Collin Jacobs ◽  
Heinrich Lanfermann ◽  
...  

IntroductionVein of Galen malformation (VGM), a high-flow intracranial arteriovenous shunt, is among the most severe neurovascular diseases in childhood. In many cases untreated children die or survive only severely disabled. Endovascular embolization is the preferred treatment.ObjectiveTo develop a simple fistulous-type VGM phantom model for teaching and training of different endovascular treatment methods and to investigate new treatment options and devices.MethodsAn experimental in vitro pulsatile phantom model was developed imitating a high-flow fistulous-type VGM, which is typical, especially in the neonatal phase. Pressure measurements at different arterial sites were performed before and after closure of the VGM. Closure of the VGM was achieved by coiling using a combined microcatheter-based transvenous and transarterial approach called ‘kissing microcatheter technique’.ResultsThe behaviour of the phantom model in vitro under fluoroscopy and under angiographic runs was extremely similar to that in in vivo conditions in children. The results showed that intra-arterial pressures changed and increased statistically significantly at all measurement sites after embolization, as in human arteriovenous malformation. We also demonstrated different and complementary visualizations of hemodynamics and angioarchitecture by antegrade and retrograde microcatheter injections.ConclusionsOur phantom model behaves like a typical fistulous-type VGM and can be used in vitro for teaching and training and for further research. It offers a new and better understanding of hemodynamics and angioarchitecture in the endovascular management of VGM.


PEDIATRICS ◽  
2014 ◽  
Vol 134 (1) ◽  
pp. e284-e288 ◽  
Author(s):  
D. Grieb ◽  
A. Feldkamp ◽  
T. Lang ◽  
M. Melter ◽  
C. Stroszczynski ◽  
...  

1974 ◽  
Vol 23 (S1) ◽  
pp. 253-258 ◽  
Author(s):  
B. Dallapiccola ◽  
P. E. Gallenga ◽  
A. Pinca ◽  
L. Capra

A case of 8 trisomy syndrome, identified on the basis of the heat denaturation technique, has been detected in a patient with mental retardation, congenital malformations, and peculiar ocular anomalies. This observation confirms that, even without evidence of mosaicism, this chromosome does not seriously upset the viability of cells and could be less deleterious to the individual than trisomy 13, 18, or 21.The mental development is not too much impaired and the skeletal abnormalities are mild, as compared to those found in previously reported cases. On the other hand, the cardiovascular system is involved and the ocular lesions are complex. They include prominent eyes, epicanthus, hypertelorism, antimongoloid slant of palpebral fissures, ectopia of lacrimal point, megalocornea, enlarged iridocorneal angle, subatrophic papilla, tortuosities of retinal vessels, absence of deep perception.


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