Summary
The authors report a series of 36 vein of Galen aneurysmal malformations (VGM) diagnosed in the paediatric (78%) and adult (22%) populations that were referred to them for therapeutic management between 1982 and 1988. The clinical signs leading to the diagnosis were variable: 36% of systemic manifestations, 22% of neurological symptoms, 17% of hydrocephaly and 11% of intracranial haemorrhage.
30 angioarchitectural analyses could be obtained and allowed to classify these VGMs into 5 different types: 44% parenchymatous AVMs, 20% mural AVFs, 30% choroidal arteriovenous fistulas, 3% dural AVFs, 7% vein of Galen varices. This series demonstrates that the paediatric population is most sensitive to shunt effect whatever its type. Systemic manifestations and hydrocephaly are the most common signs encoutered in the newborn and infants; whereas neurological signs and symptoms and haemorrhage belong mostly to the adult symptomatology. Because of the poor outcome of VGMs, all authors believe that these malformations have to be treated aggressively. However, we found contra-indications to be represented by pretherapeutic demonstration of cerebral tissue damage, or uncontrolable systemic failure, thus treatment is indicated to compensate for cardiac failure previously responding (even partially) to medical treatment. Secondly, appearance of sub-cortical calcifications, resistance to medication or clinical deterioration will also lead to urgent treatment.
The endovascular method represents at present the best treatment with an overall low mortality (13%) and a 0% technical morbidity in children compared to the surgical one of (91% mortality in newborns and 38% in infants). The results achieved by embolization in this series were as follows: 27% satisfactory results with complete or almost complete occlusion of AV Shunt, 53% significant clinical improvement, 7% of patients were unchanged. The authors believe fundamentally that these patients (specially those belonging to the paediatric population) have to be treated in a centre where a paediatric intensive care unit, neurological, neurosurgical and surgical neuro-angiographic departments coexist, in order to assure the best possible management of these children.
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References
Berenstein A, Lasjaunias P: Surgical neuroangiography, Vol IV. Endovascular treatment of brain, spine, and spinal cord lesions. Springer, Heidelberg Berlin New York London Paris Tokyo (under press)
Clarisse J, Dobbelaere P, Rey Cet al (1978) Aneurysms of the great vein of galen. Radiological study of 22 cases. J Neuroradiol 5: 91–102
Diebler C, Dulac O, Renier Det al (1981) Aneurysms of the great vein of galen in infants aged 2 to 15 months. Diagnosis and natural evolution. Neuroradiology 21: 185–197
Glatt BS, Rowe RD (1960) Cerebral arteriovenous fistula associated with congestive heart failure in the newborn: report of 2 cases. Pediatrics 9: 596–603
Gold AP, Ransohoff J, Carter S (1964) Vein of Galen malformation. Acta Neurol Scand [Suppl] II, 40: 5–31
Gomez MR, Whitten CF, Nolke Aet al (1963) Aneurysmal dilatation of the vein of Galen, causing heart failure in the early infancy. Report of 5 cases. Pediatrics 31: 400–411
Grossmann RI, Bruce DA, Zimmermann RAet al (1984) Vascular steal associated with vein of Galen aneurysm. Neuroradiology 26: 381–386
Hirano A, Solomon S (1960) Arteriovenous aneurysm of the vein of Galen. Arch Neurol 5: 589–593
Hoffmann HJ, Chuang S, Hendrick EBet al (1982) Aneurysm of the vein of Galen. Experience at the Hospital for Sick Children, Toronto. J Neurosurg 57: 316–322
Johnston I, Whittle I, Besser Met al (1987) Vein of Galen malformations. Diagnosis and management. Neurosurg 20–5: 747–758
Lasjaunias P, Terbrugge K, Chiu M (1986) Coaxial balloon-catheter device for treatment of neonates and infants. Radiology 159: 269–271
Lasjaunias P, Terbrugge K, Choi IS (1988) Transmesencephalic arteries and veins. Angiographic aspects in tectal vascular lesions. Acta Neurochir (Wien) 92: 138–143
Lasjaunias P, Terbrugge K, Lopez Ibor Let al (1987) The role of dural anomalies in vein of Galen aneurysms: report of 6 cases and review of the literature. AJNR 8: 185–192
Litvak J, Yahr MD, Ransohoff J (1960) Aneurysm of the vein of Galen and midline cerebral arteriovenous anomalies. J Neurosurg 17: 945–987
Long DM, Seljeskog EL, Chou SNet al (1974) Giant arteriovenous malformation in infancy and childhood. J Neurosurg 40: 304–312
Maheut J, Santini JJ, Barthez MAet al (1987) Symptomatologie clinique de l'anévrysme de l'ampoule de Galien. Résultats d'une enquête nationale. Neurochirurgie 33: 285–290
Maheut J, Santini JJ, Barthez MAet al (1987) Anévrysmes de l'ampoule de Galien. Résultats thérapeutiques de l'étude multicentrique nationale. Neurochirurgie 33: 337–340
Massey CE, Carson LV, Beveridge MDet al (1982) Aneurysms of the great vein of Galen: report of 2 cases and review of the literature. In: Smith RR, Haerer A, Russel WF (eds) Vascular malformations and fistulas of the brain. Raven Press, New York
Mickle JP, Quisling RG (1986) The trans-torcular embolization of vein of Galen aneurysms. J Neurosurg 64: 731–735
Raybaud Ch, Hald JK, Strother Ch Met al (1987) Les anévrysmes de la veine de Galien. Etude angiographique et considérations morphogénétiques. Neurochirurgie 33: 302–314
Rodesch G, Lasjaunias P, Terbrugge Ket al (1988) Lesions vasculaires artérioveineuses de l'enfant. Place des techniques endovasculaires à propos de 44 cas. Neurochirurgie 34: 293–303
Velut S (1987) Embryologie des veines cérébrales. Neurochirurgie 33: 258–263
Willinsky R, Lasjaunias P, Terbrugge Ket al (1988) Brain arterio-venous malformations (BAVMs) analysis of the angioarchitecture in relationship to hemorrhage. J Neuroradiol 15:225–237
Yaşargil MG (1988) AVM of the brain. In: Microneurosurgery, Vol IIIb, Thieme, Stuttgart New York
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Lasjaunias, P., Rodesch, G., Terbrugge, K. et al. Vein of Galen aneurysmal malformations. Acta neurochir 99, 26–37 (1989). https://doi.org/10.1007/BF01407773
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DOI: https://doi.org/10.1007/BF01407773