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Operative treatment of cerebral arteriovenous aneurysm of vein of Galen complicated by congestive heart failure.

A rare cause of congestive heart failure in the neonatal period is an intracranial arteriovenous malformation, but this condition should be borne in mind when there is unexplained right-sided congestive heart failure. A case is reported of an aneurysm of the great vein of Galen, complicated by congestive heart failure. Successful surgical treatment was carried out using a two-stage procedure.

Female↗

Medical-concept models and medical records: an approach based on GALEN and PEN&PAD.

OBJECTIVES: To investigate the issues raised in applying a preliminary version of the GALEN compositional concept reference (CORE) model to a series of radiographic reports, and to demonstrate that the same underlying concept model could be used in conjunction with both a detailed, fine-grained model of medical records based on that used in the PEN&PAD project and with other more conventional medical-record models. DESIGN: Following analysis and representation of concepts from a set of reports, a single report was taken as a "case study." This report was analyzed in detail in its entirety and represented using each of the medical-record models. RESULTS: The reports were successfully represented within the limits of the study, but a number of significant issues were raised. CONCLUSION: The compositional approach plus the PEN&PAD medical-record model allowed detailed information in the radiographic report to be represented, including information about the inferences and the clinical process. The resulting representation was large, and more compact representations may be necessary for some systems. Alternative encapsulations of the information as might be used in such systems were successfully prepared. The compositional approach avoided many issues that often cause controversy in the design of traditional coding and classification systems, but it raised other issues, including the handling of ambiguity and underspecification, linkage to information not explicitly present in the report, and questions concerning the focus of individual concepts. All work is preliminary and definitive conclusions await further studies and systematic evaluation.

Decision Making, Computer-Assisted↗

Cerebral damage in neonates resulting from arteriovenous malformation of the vein of Galen.

Seven neonates with arteriovenous malformation of the vein of Galen are described. Six presented with cyanotic heart failure. Two cases had antenatal cerebral infarction, five periventricular leukomalacia, and three recent haemorrhagic infarction. Cerebral damage due to the malformation suggests that surgical correction of the arteriovenous malformation offers little to neonates, though after the neonatal period individuals can be cured by operation.

Cardiac Catheterization↗

How Galen's "sixteen books" came to China in the tenth century AD.

Ibn an-Nadim, the famous 10th century bookseller and bibliographer of Baghdad and author of the "Fihrist" (Catalogue), tells the story of an unnamed Chinese student who found in the library of the famous physician and philosopher ar-Razi the so-called "Sixteen Books," i.e. the Arabic summary of the most influential books written by Galen, and translated them into Chinese. We do not know if this Chinese translation was safely transported to China.

Arab World↗

Gianturco coil embolization of vein of Galen aneurysms: technical aspects.

Galenic A-V fistulas typically result in hydrocephalus and increased cerebral venous pressure, with symptoms of progressive seizure activity, chronic cardiac failure and failure to thrive. Surgery and arterial embolization have been only partially successful in reducing flow through these shunts. The authors present technical details of a procedure for embolizing such lesions via a transtorcular venous approach. Early results in 15 patients are reported: Twelve patients appear to have had significant symptomatic improvement; two have died of persistent heart failure, and one died of a subdural hematoma associated with ventriculostomy.

Arteriovenous Fistula↗

Spontaneous thrombosis of vein of Galen aneurysmal malformation.

Two cases of aneurysmal malformations of the vein of Galen (AVG) with spontaneous thrombosis are reported. Angiogram and MRI before thrombosis demonstrated AVGs with slow arteriovenous shunts and associated stagnation of contrast in the venous sac secondary to severe outflow restriction. Based on these findings, one patient was managed conservatively, and the other underwent placement of a ventriculoperitoneal shunt. Surveillance of the lesions with subsequent MRIs revealed spontaneous thrombosis of the AVGs with excellent clinical outcomes. Proposed mechanisms of spontaneous thrombosis include slow flow shunts, obstruction of the venous outflow or obstruction of the feeding artery. Similar cases in the literature are reviewed with special emphasis on diagnostic tests, symptomatology, mechanisms of thrombosis and therapeutic options.

Central Nervous System Vascular Malformations↗

Successful treatment of a neonate with vein of Galen aneurysmal malformation.

We present a follow-up study on a 5-year-old boy who was born with vein of Galen aneurysmal malformation (VGAM) accompanied by extreme cardiovascular distress. He was successfully treated by the staged endovascular technique during the neonatal stage. Postoperative angiographic study confirmed complete occlusion of the VGAM and the establishment of normal brain perfusion. At 5 years of age, the child is growing normally without neurological deficits or developmental retardation. We suggest that the restoration of normal brain perfusion in neonates with VGAM can cause normal brain development.

Cardiomegaly↗

Prenatal diagnosis of a vein of galen aneurysm.

We present the prenatal diagnosis of a vein of Galen aneurysm in a 34-week fetus. The aneurysm was 8 x 3.5 x 3 cm in dimension, and located posterior to the third ventricle. Color flow examination revealed a turbulent flow in the lesion. There was significant cardiomegaly. Prenatal ultra-fast magnetic resonance imaging was used to view the malformation in three dimensions. Although the pregnancy continued uneventfully, the fetus died of congestive heart failure on the 3rd day postpartum. The value of antenatal sonography and magnetic resonance imaging in the prediction of prognosis is discussed.

Adult↗

Neuroanatomical study of Galen's anastomosis (nervus laryngeus) in the dog.

To further knowledge of the laryngeal nerves, the nerve fibers of Galen's anastomosis were studied using two neuroanatomical methods, namely nerve degeneration and horseradish peroxidase labeling. It is demonstrated that the superior laryngeal nerve forms part of the tracheal and esophageal nervous system. The value of the results in relation to physiological laryngeal studies and to human laryngeal diseases is discussed.

Animals↗

Abnormal fetal cerebral blood flow velocity waveforms as a sign of an aneurysm of the vein of Galen.

A cerebral midline cystic lesion was detected by real-time ultrasound in utero in the absence of other congenital abnormalities. Pulsed Doppler ultrasound assessment of fetal circulation demonstrated normal peripheral and intracardiac hemodynamics associated with decreased cerebral vascular resistance. Furthermore, a markedly turbulent flow pattern was evidenced within the cerebral lesion. The presence of a cerebral arteriovenous malformation with an aneurysm of the vein of Galen was suggested and the provisional diagnosis was later confirmed by computed tomography and carotid angiography performed after birth.

Adult↗

The "sceptical crisis" reconsidered: Galen, rational medicine and the libertas philosophandi.

This paper reassesses the role of sceptical thinking in the emergence of the new science of the seventeenth century, in the context of the seminal but contestable History of Scepticism by Richard Popkin. It investigates the anti-sceptical essay by Galen De optimo modo docendi (on the best method of teaching), which was retranslated in the sixteenth century by Erasmus and later published as an adjunct to the works of Sextus Empiricus, in order to highlight the currency of ideas about hyperbolic doubt, and links this to the long tradition of free enquiry (libertas philosophandi) in which doubting authority is seen as a profitable exercise closely associated with the independence of philosophy from theological domination; and it argues that this long tradition (along with a number of other factors) played an important role in the emergence of the new science.

History, 15th Century↗

Neonatal vein of Galen malformations: experience in developing a multidisciplinary approach using an embolization treatment protocol.

A multidisciplinary team approach using a staged transcatheter embolization and neurosurgical protocol was applied to 22 patients with neonatal presentation of vein of Galen malformations over a 12 year period. Aggressive medical therapy was combined with interventions including: ventricular shunting, transcatheter embolization, retrograde transtorcular embolization, and neurosurgical obliteration. There was a high frequency of high output cardiac failure, multiple organ system dysfunction, seizures, hydrocephalus, visual, developmental and neurological disability. Of the first 11 patients, five survived; four with seizures and three with marked retardation. Of the last 11 patients, six survived; five with seizures but only one with retardation. Despite persistently high morbidity and mortality, our continuously evolving protocol offers these otherwise hopeless patients some chance of survival.

Cerebral Veins↗

Arteriovenous malformation of the vein of Galen.

Vein of Galen aneurysm may be caused by a spectrum of abnormal arteriovenous connections ranging from extensive malformations involving the tentorial dura and upper brain stem vessels to the rarer condition of a simple end to end fistulous connection. The diagnosis of the latter condition of a simple arteriovenous fistula is important because it may be effectively managed by surgical interruption of the feeding fistulous vessels. Two cases with the direct fistulous connections are presented.

Cerebral Angiography↗

The father of sports medicine (Galen).

Although there were many physicians who treated athletes before Galen, I believe that he was the first to devote a major portion of his time to this field of endeavor. Furthermore, his systematic observations, his aggressive pursuit of newer and better ways of treatment, his teaching, and his publishing of his observations make him a kindred soul to the team physician and practitioners of sports medicine of today. It is for these reasons that I believe that he can justly be called the "Father of Sports Medicine."

History, Ancient↗

Vein of Galen malformation and infantile spasms.

Recently, there has been increasing interest in the study, characterization, and management of infantile spasms. With technological advances, it is now possible to determine a specific diagnosis on many patients with infantile spasms, thus broadening the spectrum of conditions associated with this electroclinical syndrome. We report a child with infantile spasms who also had a vascular malformation of the vein of Galen. Both clinical and electroencephalographic findings improved rapidly after endovascular treatment of the vascular anomaly, suggesting a possible association.

Cerebral Veins↗

High melatonin concentrations in third ventricular cerebrospinal fluid are not due to Galen vein blood recirculating through the choroid plexus.

Melatonin has been implicated in several neurotropic effects, but few studies have investigated the bioavailability of melatonin in the brain. The discovery of periventricular sites of action adjacent to the third ventricle forced us to investigate the dynamics of cerebrospinal fluid (CSF) melatonin release and the source of this melatonin. Our first study demonstrated unequivocally that third ventricle CSF melatonin, like jugular plasma melatonin, accurately reflects the duration of the night and is rapidly suppressed by light. However, third ventricle CSF melatonin levels are 20-fold higher than nocturnal plasma concentrations. A further study showed that melatonin increased in plasma before third ventricle CSF, raising the possibility that melatonin is taken up from the blood after recirculation through the Galen vein. However, a final experiment suggested strongly that CSF melatonin is released directly into the third ventricle, as melatonin levels in the lateral ventricle were 7-fold lower than those in the third ventricle. Our study raises the possibility that there may be two compartments of melatonin affecting physiological functioning: the first in plasma acting on peripheral organs, and the second in the CSF affecting neurally mediated functions at a much higher concentration of this pineal indoleamine.

Animals↗

Neurodevelopmental outcome after endovascular treatment of vein of Galen malformations.

OBJECTIVE: To assess neurodevelopmental outcome after endovascular treatment of vein of Galen malformations (VOGM). METHODS: Outcome of patients who underwent endovascular treatment for VOGM between 1983 and 2002 was assessed by chart review and parental questionnaires. Development was classified as normal, minor delay (slow initial acquisition of milestones but no permanent disability), or significant delay (slow or incomplete acquisition of milestones with some permanent disability) using an adaptation of the Denver Developmental Questionnaire. RESULTS: Twenty-seven patients were identified: five presented prenatally (by ultrasound), 16 as neonates, and 6 after the neonatal period. The most common presenting features were congestive heart failure (CHF; 16/27) and hydrocephalus (8/27). The 16 patients with CHF all presented either prenatally or neonatally; 4 died acutely, 6 had significant delay, and 6 had no or minor developmental delay. Of those presenting in the perinatal period without CHF, all survived, two of five were significantly delayed, and three of five had no delay. Of those presenting after the neonatal period, all survived and only one of six had delay. By angiographic classification, outcome was worse for those with choroidal VOGM (3/13 died; 5/13 had significant delay) than for those with mural VOGM (2/10 had significant delay; none died). For the entire series, 52% of all cases (61% of survivors) had no or minor delay. CONCLUSIONS: Fourteen of 27 children who received treatment for VOGM had a favorable outcome. Features associated with worse outcome were perinatal presentation, presence of CHF, and choroidal angioarchitecture.

Cardiovascular Abnormalities↗