参考文献
Johnston IH, Whittle IR, Besser M, Morgan MK.
Vein of Galen malformation: diagnosis and management. Neurosurgery. 1987 May;20(5):747-58.
The vein of Galen malformation is a midline arteriovenous fistula with aneurysmal dilatation of the vein of Galen. The clinical details of diagnosis and treatment in 13 patients with such lesions together with a review of 232 cases collected from the literature are presented in this report. There were 132 males, 77 females, and 36 cases in which the sex was not stated. Eighty patients presented as neonates, 82 were 1 to 12 months old, 39 were 1 to 5 years old, 22 were 6 to 20 years old, and 22 were over the age of 20. The most common presenting symptoms were congestive cardiac failure (110 cases), raised intracranial pressure secondary to hydrocephalus (94 cases), cranial bruit (57 cases), focal neurological deficit (37), seizures (26 cases), and hemorrhage (25 cases). The most characteristic vascular supply to the midline fistula involved multiple bilateral vessels, although bilateral posterior cerebral and unilateral posterior cerebral supply was relatively common. The overall figures for treatment and outcome showed that 91 patients (37.1%) were treated by direct operation and 29 patients (11.3%) were treated by other forms of operation, predominantly shunting or remote vessel ligation. Forty-six patients (18.8%) were treated by medical means (digoxin, diuretics, and ventilatory support). In 79 patients (22.2%), there was no treatment or no details of treatment were available. There was an overall series mortality of 55.6% (no details were available in 33 cases) and a 37.4% mortality for surgically treated cases. After operation, there was a 46.3% incidence of significant morbidity in surviving patients. Neonatal patients fared worst, with an overall mortality of 64 of 70 cases (91.4%) where details were available. The outcome was equally bad for surgically and conservatively treated cases. Operation in the 1- to 12-month age group was more successful, but still carried a mortality of 31.7%, with a significant morbidity in approximately half of the surviving patients. Over the age of 1 year, the surgically treated patients had a 25.6% mortality and a 42.3% major morbidity in survivors. Consideration is given to some of the ways in which these figures may be improved, in particular a staged approach during the neonatal period, with the use of selective embolization or occlusion of vessels to reduce the volume of the arteriovenous shunt until the patient is older and better able to tolerate major operation.
Komiyama M, Nishikawa M, Yasui T, Kitano S, Sakamoto H, Inoue T.
Vein of Galen aneurysmal malformation in a neonate treated by endovascular surgery--case report. Neurol Med Chir (Tokyo). 1996 Dec;36(12):893-900.
A male neonate with vein of Galen aneurysmal malformation developed fetal heart failure after a gestation period of 37 weeks. Labor was induced, but a cesarean section had to be performed because of fetal distress. His Apgar scores were 2/8 at birth. The boy required controlled ventilation due to generalized seizure and cyanosis. Neuroimaging revealed vein of Galen aneurysmal malformation with enormous arteriovenous shunt flows. Transarterial embolization was performed on days 4 and 6 to reduce the progressive high-output congestive heart failure. Three large feeders were occluded with interlocking detachable coils, markedly reducing the shunt flow. Although heart failure improved markedly, he died of pulmonary hemorrhage on day 12. Endovascular surgery can improve the prognosis of heart failure in the neonatal period less invasively than other treatment modalities, and may help to minimize ischemic brain damage and thus increase the possibility of normal brain maturation.
Komiyama M, Nishikawa M, Kitano S, Sakamoto H, Miyagi N, Kusuda S, Sugimoto H.
Transumbilical embolization of a congenital dural arteriovenous fistula at the torcular herophili in a neonate. Case report. J Neurosurg. 1999 May;90(5):964-9.
A neonate, in whom a congenital cerebral vascular anomaly had been diagnosed prenatally, exhibited progressive high-output congestive heart failure soon after birth. Cerebral angiography revealed a congenital dural arteriovenous fistula (AVF) with a huge dural lake located at the torcular herophili. In addition to the meningeal blood supply, an unusual pial blood supply from all cerebellar arteries was observed to feed the fistula. The patient was treated by repeated transarterial and transvenous embolization through the umbilical venous route. To the authors' knowledge, neither the existence of a congenital dural AVF at the torcular herophili presenting with an enormous pial blood supply or the technique of trans-umbilical venous intervention has been reported in the literature.
Komiyama M, Ishiguro T, Kitano S, Sakamoto H, Nakamura H.
Serial antenatal sonographic observation of cerebral dural sinus malformation. AJNR Am J Neuroradiol. 2004 Sep;25(8):1446-8.
Dural sinus malformation is an extremely rare congenital cerebrovascular malformation. We report serial antenatal sonographic findings in two patients with dural sinus malformation. Sonography can reveal dural sinus malformation at 24 weeks' gestation. Correct and early diagnosis may help determine the appropriate place, timing, and mode of delivery, which may result in a better therapeutic course and patient outcome.
Lasjaunias P, Rodesch G, Terbrugge K, Pruvost P, Devictor D, Comoy J, Landrieu P:
Vein of Galen aneurysmal malformations. Report of 36 cases managed between 1982 and 1988. Acta Neurochir (Wien). 1989;99(1-2):26-37.
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 encountered 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 uncontrollable 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.