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To test the value of currently proposed angiographic categorizations of vein of Galen malformations and a hypothesis regarding the causes of vein of Galen malformations and of accompanying hydrocephalus, as well as to assess the relative utility of MR imaging and CT in clinical evaluation, we reviewed the clinical and radiologic records of 34 patients with vein of Galen malformations. Patients were divided into two groups on the basis of the angiographic demonstration of either an arteriovenous malformation nidus or a direct arteriovenous fistula to the wall of the vein of Galen or one of its tributaries. Patients with such a nidus (n = 17) could be distinguished from those with arteriovenous fistulas alone (n = 17) on the basis of age at presentation (p less than .01) and presenting symptoms. Venous constraints, thought to be etiologically important, were identified in 31 of 34 patients. The presence or absence of hydrocephalus was explainable by mass effect in only 24 of 32 patients. In seven of 32 cases, no obvious mass effect was seen in the presence of hydrocephalus, but arteriographic evidence of venous hypertension was present in all patients with hydrocephalus. MR provided improved depiction of both arterial and venous anatomy as compared with CT. Parenchymal abnormalities were uncommon. No patients had subarachnoid hemorrhages. We conclude that MR is superior to CT in the clinical evaluation of vein of Galen malformations, that the angiographic finding of a nidus separates patients with vein of Galen malformations into clinical and therapeutically relevant groups, and that simple mass effect on the aqueduct is not an adequate explanation for all cases of hydrocephalus in patients with this disease.
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PMID:Vein of Galen malformation: correlation of clinical presentation, arteriography, and MR imaging. 190 42

Seven cases of vein of Galen aneurysms treated by percutaneous transvenous endovascular occlusion of the aneurysmal vein are presented. In one case, the approach was via the femoral vein, and in the other six cases, by the jugular vein. All of the malformations were multipedicular and, additionally, in six of the seven there was an intervening arterial-arterial network between the posterior thalamoperforating arteries and the wall of the venous aneurysm. This fistulous network was interpreted as purely arterial and not as an associated arteriovenous malformation. For this reason, the transvenous approach was considered justified, and was performed without risk of hemorrhage caused by retrograde venous hypertension. Measurement of intra-aneurysmal pressure during the course of treatment allowed better understanding of the hemodynamics of the lesions, guided the amount of occlusion to be accomplished during each treatment session, and thus may have prevented the phenomenon of normal perfusion pressure breakthrough. The percutaneous transvenous approach offers all the advantages of the transtorcular approach but avoids surgery. Because of our excellent angiographic and clinical results--five complete and two partial occlusions, with favorable outcomes and no major complications--we believe that this technique is better for the treatment of multipedicular vein of Galen aneurysms than transarterial embolization or surgery.
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PMID:Percutaneous transvenous catheterization and embolization of vein of galen aneurysms. 199 95

Severe hypertension developed secondary to renal artery stenosis in 11 of 229 children who received a renal allograft. Renal artery stenosis was suspected because of de novo development of hypertension or exacerbation of pre-existing hypertension, which was detected 1 to 24 months after transplantation. Selective renal angiography was performed 2 to 74 months after transplantation (mean 13 months). Follow-up was 1 to 8 years (mean 2.5 years). The stenosis involved the anastomosis in 5 patients and was distal to the anastomosis in 6. One graft had an arteriovenous malformation. Seven grafts were suitable for vessel dilation; percutaneous transluminal angioplasty was partially successful in 4 cases in which the stenosis occurred at the anastomosis. The remaining patients were treated with medical therapy alone and the grafts were not lost. Our findings suggest that strictures distal to the anastomosis rarely are amenable to percutaneous transluminal angioplasty and should be treated medically whenever possible. Strictures at the anastomosis respond to vessel dilation but antihypertensive medication also often is required. An operation should be reserved for patients who do not respond to these measures.
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PMID:Percutaneous transluminal angioplasty for transplant renal artery stenosis in children. 213 41

The range of disorders affecting the cerebral veins and sinuses is increasing and now includes blood disorders, abnormalities in the patterns of blood flow, and infiltrative or inflammatory conditions, all of which may promote thrombosis. We describe 10 patients with cerebral venous thrombosis: two had protein S deficiency, one had protein C deficiency, one was in early pregnancy, and there was a single case of each of the following: dural arteriovenous malformation, intracerebral arteriovenous malformation, bilateral glomus tumours, systemic lupus erythematosus, Wegener's granulomatosis, non-Hodgkin's lymphoma. The recognition of such diverse aetiology may be importance since clinical features are non-specific, and may consist only of raised intracranial pressure, allowing confusion with 'benign intracranial hypertension'. The existence of effective treatment both for the thrombosis and for many of the underlying disorders makes early diagnosis essential. The prognosis of treated patients may be favourable.
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PMID:Cerebral venous thrombosis: new causes for an old syndrome? 214 67

Only 6 patients with intracranial hypertension associated with unruptured cerebral arteriovenous malformations have been reported. We report 6 additional patients seen at the Cleveland Clinic during the past 10 years. The average age was 28 years (range, 19-44 years); 4 were women. Symptoms and signs included papilledema (6 patients), headache (6), transient nonepileptic focal symptoms (4), visual obscurations (3), ipsilateral carotid or ocular bruits (3), abnormal visual fields (3), focal seizures (2), and progressive visual loss (1). Enhanced computed tomography (CT) or magnetic resonance imaging (MRI) demonstrated the malformations in all 6 patients. The malformations were large, supplied by the branches of the middle and anterior cerebral arteries, with the posterior cerebral artery contributing in 3 patients, and all drained into the superior sagittal sinus. Associated venous obstruction was seen in 2 patients. Four patients underwent excision of the arteriovenous malformation, with resolution of papilledema in all 4. Measurements of cortical arterial and venous pressures during surgery in 3 patients showed decreased feeding artery pressures and elevated draining vein pressures, which normalized after removal of the malformation. Treatment in the 2 remaining patients consisted of medical therapy (acetazolamide, furosemide, steroids) alone in 1 patient, and in conjunction with proton beam radiation in the other. Papilledema resolved in the former patient, but the patient receiving proton beam radiation still had papilledema 2 years later. Intracranial hypertension associated with unruptured cerebral arteriovenous malformations occurs in young patients with high flow malformations that drain into the superior sagittal sinus, and is likely the result of increased cortical venous and superior sagittal sinus pressure. Excision of the malformation effectively reduces the intracranial pressure.
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PMID:Intracranial hypertension associated with unruptured cerebral arteriovenous malformations. 219 10

We report the case of a 69 year-old female, with cardiac disease, hypertension, and chronic renal failure treated by hemodialysis; she had several episodes of gastrointestinal hemorrhage, which could not be diagnosed by conventional methods. Digital angiographic study demonstrated the exact cause: it allowed to localize an arterio-venous malformation. A right hemicolectomy was performed and the pathologic study showed the presence of an arteriovenous fistula. Three years later the patient is free of symptoms. We recommend digital angiography in cases of gastrointestinal hemorrhage of unknown or unclear etiology, particularly in patients in chronic renal failure, on the basis of the association of hemodialysis and arteriovenous malformation.
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PMID:[Arteriovenous fistula of the cecum diagnosed by digital angiography]. 225 91

Intracranial hemorrhage (ICH) from an intracranial aneurysm or arteriovenous malformation is a grave complication of pregnancy and is responsible for 5 to 12% of all maternal deaths. We critically analyzed 154 cases of verified ICH during pregnancy from an identified intracranial lesion, including 2 patients treated at our institution and 152 cases previously reported in the literature in English. Aneurysms were responsible for ICH in 77% of patients, and arteriovenous malformations in 23%. Hemorrhage occurred antepartum in 92% of patients and postpartum in 8%. Women with angiomatous hemorrhage were younger than those with aneurysmal hemorrhage; however, in contrast to previous reports, we found no differences between angiomatous and aneurysmal hemorrhage with respect to parity or gestational age at the time of the initial hemorrhage. Hypertension and/or albuminuria were present at some time during the pregnancy in 34% of patients with documentation, which sometimes made it difficult to differentiate angiomatous or aneurysmal ICH from that associated with eclampsia. In a logistic regression analysis, surgical management of aneurysms, but not arteriovenous malformations, was associated with significantly lower maternal and fetal mortality, independent of other covariants. For those patients with a lesion not operated on, cesarean delivery afforded no better maternal or fetal outcome than did vaginal delivery. We conclude that the decision to operate after ICH during pregnancy should be based upon neurosurgical principles, whereas the method of delivery should be based upon obstetrical considerations. The perioperative and anesthetic management of the pregnant patient with a neurosurgical complication is discussed.
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PMID:Intracranial hemorrhage from aneurysms and arteriovenous malformations during pregnancy and the puerperium. 227 25

Congenital bronchopulmonary malformations are uncommon but potentially life-threatening anomalies of infants and children. Between 1970 and 1988, 45 patients from birth to 13 years of age (23 boys and 22 girls) underwent evaluation and treatment for bronchopulmonary malformations. Thirty-seven had solitary lesions: bronchogenic cyst (n = 13), cystic adenomatoid malformation (n = 9), congenital lobar emphysema (n = 6), pulmonary sequestration (n = 6), arteriovenous malformation (n = 2), and bronchial atresia (n = 1). Eight additional patients had two simultaneous abnormalities and three patients had congenital diaphragmatic hernias. Twenty-one patients had respiratory symptoms, which were severe in seven. Twelve had pulmonary infection and 10 patients were completely free of symptoms. Plain chest roentgenogram was the only diagnostic imaging performed in 11 patients. Thirteen patients underwent computed tomographic scan, but in only four was it essential for diagnosis. Prenatal ultrasonography in three patients demonstrated cystic adenomatoid malformation in two, with one false negative study. Postnatally, ultrasonography was also useful in establishing the diagnoses of cystic adenomatoid malformation and pulmonary sequestration. Thoracotomy with excision of the lesion by lobectomy or pneumonectomy resulted in survival of 42 patients (93%). Three deaths in neonates were due to pulmonary hypoplasia and hypertension. Two of them had concomitant diaphragmatic hernia; the other had a cystic adenomatoid malformation and died despite the use of postoperative extracorporeal membrane oxygenation. These data demonstrate that congenital bronchopulmonary malformations usually can be diagnosed by plain chest x-ray films. Ancillary studies such as ultrasonography or computed tomography may occasionally be necessary. Combinations of the different types of bronchopulmonary malformations occurred frequently. All lesions, including symptomatic lesions in neonates, can be managed surgically soon after diagnosis.
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PMID:Congenital bronchopulmonary malformations. Diagnostic and therapeutic considerations. 231 79

Eighteen patients with subcortical lobar hematomas were reviewed. Arterial hypertension was the leading cause and three had arteriovenous malformation and were treated surgically. More than half of cases had hematomas of either the temporooccipital or occipital lobes. Common neurologic findings were headaches, vomiting, alertness, dysarthria, hemiparesis and hemianopsia. All patients survived and had better resolution of neurological deficits, suggesting that surgical intervention is not necessary.
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PMID:Subcortical lobar hematomas: clinico-computed tomographic correlations. 270 3

Surgical specimens taken from 14 patients with lobar intracerebral haemorrhage or cerebellar haemorrhage without vascular abnormalities on angiograms were examined histologically. In seven of the 14 patients, arteriovenous malformation or amyloid angiopathy were found by ordinary pathological examinations. Among the remaining seven patients, definite microaneurysms were verified in five and possible ones in two patients by using the technique of serial sectioning of the solid nodular tissues removed from the presumed bleeding site, where an arterial connection between the tissues and the surrounding brain was noted. Four of these seven patients had no history of hypertension and showed normal blood pressure before and after surgery. To verify microaneurysms in surgical specimens, it seems important to search the presumed bleeding site properly by a meticulous microsurgical technique and to section the tissues serially for the histological examination.
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PMID:Histological verification of microaneurysms as a cause of cerebral haemorrhage in surgical specimens. 273 28


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