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Aneurysm of the vein of Galen: prenatal diagnosis and perinatal management.

An aneurysm of the vein of Galen is a rare arteriovenous malformation of the central nervous system. Fetal manifestations have included nonimmune hydrops, hydrocephalus, and intracranial hemorrhage. This anomaly may be diagnosed prenatally by several imaging modalities. A cystic cranial mass was identified by ultrasound in a fetus at 30 weeks gestation. Both pulsed-wave Doppler and color-velocity imaging studies suggested aneurysm of the vein of Galen was the most likely diagnosis. The fetus demonstrated no evidence of hydrops on serial ultrasound examinations. A 2430 g female infant was delivered vaginally at 35 weeks gestation. Postnatal management included transarterial embolization of the vessels feeding the aneurysm with craniectomy, an intra-aneurysmal balloon, and vascular microcoils. Hydrocephalus developed and a ventriculo-peritoneal shunt was placed. The infant has grown appropriately in the first year of life. An aneurysm of the vein of Galen may be diagnosed prenatally by real-time ultrasound, pulsed-wave Doppler, color-velocity imaging, or magnetic resonance imaging. The presence of this malformation should prompt close follow-up for the remainder of the pregnancy. Careful obstetric management and early postnatal intervention may lead to a favorable outcome.

Adolescent↗

Schizophrenia-like psychosis associated with vein of Galen malformation: a case report.

A 34 year old patient presented with a schizophrenia-like clinical picture, and was later found to have a large vein of Galen Arteriovenous Malformation (AVM). There are reports in the literature suggesting that vein of Galen AVMs are associated with psychiatric symptoms. However, the clinical pictures tend to be non specific. We discuss the relevance of the association of vein of Galen AVMs with schizophrenia-like symptomatology.

Adult↗

Return to Galen.

AT THE BEGINNING of the previous century, the English editor of a minor work by Galen called for a return to that ancient scholar after centuries of neglect. The appeal did not meet with success. Galen's reputation is currently based on renown arising from tradition, rather than an awareness of his actual contributions to medical science. His methodological approach to experimental medicine seems to have fallen into obscurity, although it anticipated modern principles. This report reviews the rise and fall in popularity of Galen's medical works, in an attempt to highlight the circumstances that still prevent a full appreciation of those writings.

History, Ancient↗

The management of vein of Galen aneurysmal malformations.

OBJECTIVE: The vein of Galen aneurysmal malformation (VGAM) is a choroidal type of arteriovenous malformation involving the vein of Galen forerunner. This is distinct from an arteriovenous malformation with venous drainage into a dilated, but already formed, vein of Galen. Reports of endovascular treatment of VGAM in the literature approach the disease from a purely technical viewpoint and often fail to provide satisfactory midterm results. To focus the therapeutic challenge to a strictly morphological goal overlooks the fundamental aspects of neonatal and infant anatomy and fluid physiology. During the past 20 years, our approach to VGAM has remained the same. Our experience, based on 317 patients with VGAM who were studied in Hospital Bicêtre between October 1981 and October 2002, allows us to describe the angioarchitecture, natural history, and management of VGAM in neonates, infants, and children. METHODS: Of our cohort of 317 patients, 233 patients were treated with endovascular embolization; of these, 216 patients were treated in our hospital. The treatment method of choice was a transfemoral arterial approach to deliver glue at the fistulous zone. RESULTS: Of 216 patients, 23 died despite or because of the embolization (10.6%). Twenty out of the 193 (10.4%) surviving patients were severely retarded, 30 (15.6%) were moderately retarded, and 143 (74%) were neurologically normal on follow-up. CONCLUSION: Our data demonstrate that most treated children survive and undergo normal neurological development; an understanding of the clinical, anatomical, and pathophysiological features of VGAM has, therefore, reversed the former poor prognosis. Our level of understanding about the lesion allows us to predict most situations and remedy them by applying a strict evaluation protocol and working within an optimal therapeutic window. Patient selection and timing remain the keys in the management of this condition. It is more important to restore normal growth conditions than a normal morphological appearance, with the primary therapeutic objective being normal development in a child without neurological deficit.

Cerebral Veins↗

Occlusion of the great vein of Galen associated with a huge meningioma in the pineal region. Case report.

The authors report a case in which the great vein of Galen was occluded during removal of a huge meningioma in the pineal region. The patient recovered satisfactorily without any serious neurological deficits after the operation. Preoperative angiography had shown marked stenosis of the great vein of Galen and anastomoses between the deep and the superficial venous systems. Occlusion of the great vein of Galen may be well tolerated in particular cases if this vein is already compromised.

Adult↗

Therapeutic alternatives for vein of Galen vascular malformations.

From September, 1986, to March, 1990, the authors treated 28 children harboring a vein of Galen vascular malformation. Eleven (39.3%) of the patients were neonates, 13 (46.4%) were 1 to 2 years old, and four (14.3%) were more than 2 years old. Fifteen patients (53.6%) presented with severe congestive heart failure, six (21.4%) had seizures, four (14.3%) had hydrocephalus, and three (10.7%) presented with intraventricular hemorrhage. Based on the Yaşargil classification of malformations, 10 lesions (35.7%) were Type I, seven (25%) were Type II, eight (28.6%) were Type III, and three (10.7%) were Type IV. In 11 patients (39.3%), a combined transfemoral, transarterial, and transvenous embolization of the vein of Galen malformation was performed. A pure transtorcular approach was utilized in eight patients (28.6%), and postembolization surgical clipping of arterial feeders was performed in two cases with intractable congestive heart failure. Complete anatomical occlusion of the galenic malformation was achieved in 13 patients (46.4%). An immediate postembolization improvement in the patient's clinical status was obtained in 23 (82.1%) of 28 patients and a good long-term clinical outcome was seen in 17 patients (60.7%). Five deaths (17.9%) occurred in this series of 28 patients; three (10.7%) were related to a transtorcular embolization and two (7.1%) to the unchanged natural history of the disease.

Cerebral Veins↗

A case of aneurysm of the vein of Galen.

Aneurysm of the vein of Galen is a very rare disease. The authors present a case of secondary aneurysm of the vein of Galen which was confirmed by characteristic clinical symptoms, brain CT and angiographic findings. The patient was a 14-year-old right handed girl with intermittent headache, nausea, vomiting, dysphasia and gait disturbance. Neurologic examination revealed dysarthria, nasal voice, blurring of both margins of optic discs, truncal ataxia and dysdiadochokinesia. Sensory function was normal but right hemiparesis was seen. Roentgenogram of the skull revealed diffuse thinning of the calvarium, widening of sella turcica and erosion of clinoid processes. Computed tomogram of the brain showed dilatation of all ventricles and round hyperdense mass behind the third ventricle in the midline. The lesion was enhanced markedly and homogeneously. Left and right internal carotid angiograms showed arteriovenousmal-formation with drainage to the aneurysm of the vein of Galen.

Adolescent↗

Perinatal three-dimensional color power Doppler ultrasonography of vein of Galen aneurysms.

OBJECTIVE: To present the prenatal and postnatal three-dimensional color power Doppler ultrasonographic features in cases of vein of Galen aneurysmal malformation. METHODS: Prenatal three-dimensional color power Doppler ultrasonography was used to image the neurovascular malformations in 3 fetuses with vein of Galen aneurysms and in unaffected control fetuses at the same gestational age. Postnatal transfontanel three-dimensional power Doppler ultrasonography was also performed in the third affected case and in an unaffected control neonate. The feeding and drainage vessels were analyzed in the three-dimensional rendering mode. RESULTS: The first case was complicated by porencephaly and fetal heart failure, and the pregnancy was terminated. The other 2 cases were uncomplicated prenatally, but 1 of the infants died after aneurysmal embolization due to acute cerebral ischemia. The angioarchitecture of the arteriovenous fistula was characterized in greater detail by three-dimensional color power Doppler ultrasonography than by two-dimensional ultrasonography, especially regarding the anatomic features of the feeding and drainage vessels and their connections with the dilated vein of Galen. CONCLUSIONS: Three-dimensional color power Doppler ultrasonography provided detailed images of the aneurysmal malformation and its vascular connections. Further studies are needed to correlate three-dimensional vascular imaging patterns with outcome.

Female↗

[Galen on the seat of the mind: medical experiment and philosophical tradition].

In books I-VI of his On the doctrines of Hippocrates and Plato, the medical scientist and philosopher Galen of Pergamon (c. 129-210 CE) defends the platonic tripartition and trilocation of the soul against the cardiocentric theory endorsed by the Aristotelians, Stoics and many physicians. This article concentrates on Galen's anatomical experiments (vivisections) concerned with the nervous and arterial systems. These experiments are embedded in a theoretical framework derived in large part from Aristotelian theory of science. Though Galen is to some extent aware of certain theoretical problems involved in experimentation, notably that of experimental error, he remains in many respects a captive of a traditional debate, which is marked by a limited set of available options. Thus his experiments serve the purpose of confirming the platonic position, even though they are sometimes open to alternative interpretations.

Brain↗

Angiographic findings in two cases of aneurysmal malformation of vein of Galen prior to spontaneous thrombosis: therapeutic implications.

Two cases of aneurysmal malformations of the vein of Galen with later spontaneous thrombosis are reported. Angiograms before thrombosis in both cases showed mural type aneurysmal malformations of the vein of Galen with slow arteriovenous shunts and associated stagnation of contrast in the venous sac secondary to severe outflow restriction. Based on these angiographic findings, the patients were managed conservatively and the arteriovenous malformations of the vein of Galen thrombosed with good clinical outcomes. Twenty cases of spontaneous thrombosis previously reported in the literature are reviewed.

Cerebral Angiography↗

[Galen of Pergamum (130-200)--his views on ophthalmology (part I)].

The development of medicine owes a lot to Galen of Pergamum (Asia Minor) who lived around 130-200 AD. He was of Greek origin and he wrote his works only in Greek. However, he lived and worked mostly in Italy. He studied both medicine and philosophy. When he came to Rome in 162 he was quickly recognised as a talented physician due to his unusual medical skills. He even became a physician at the Emperor's court. Apart from being an outstanding physician, he had a great knowledge of medicines and ways of their production. Being familiar with various philosophical doctrines, in particular with Hippocrates' thoughts, as well as the views of Plato, Aristotle and the Stoics, Galen developed his general theory of body functioning. It was only after his death that his works attracted more general attention. Both theoretical developments and his works on various ailments were first recognised in the Arabic world. It was only this fact that resulted in his works being gradually translated from Arabic into Latin and published in Europe where they grew important among European university circles. The article outlines ophthalmologic aspects included in Galen's works. The accuracy of descriptions of eye diseases and elements of descriptions of particular cases are underlined. It is concluded that the knowledge of symptoms of certain diseases followed from clinical observations, as well as from experiments on animals.

Attitude to Health↗

History of surgery for cerebrovascular disease in children. Part II. Vein of Galen malformations.

Vein of Galen aneurysmal malformations are a mixed group of lesions characterized by an abnormal fistula between abnormal distal branches of the choroidal and/or posterior cerebral arteries and the great vein of Galen. In this paper the authors trace the historical evolution of the current approach to diagnosis and treatment, and the literature is reviewed comprehensively. During the historical era, vein of Galen malformations were described in individual case reports and an early classification system was developed. In the early era of treatment, open surgery was the preferred approach, although morbidity and mortality rates were high. The development of neurointerventional techniques allowed the introduction of occlusive materials into the fistula, with pronounced improvements in clinical outcome.

Cerebral Veins↗

The kidney from Galen to Vesalius -- a first approach.

In 1543, Andreas Vesalius (1514 - ca. 1564) published de Humani corporis fabrica in Basel (Basel, 1543). In current literature, the work is interpreted as either an imitation or a refutation of previous anatomical knowledge, particularly the treatises by the classical Greek physician Galen (129 -- after 216 A.D. [?]). In this paper we focus on Vesalius' study of the kidneys (Book V, chapter 10). On the basis of a comparison of Vesalius' and Galen's Greek text, we reach the conclusion that Vesalius closely reproduced Galen's model and text, thus re-enacting the ancient practice of medicine and dissection.

Greece↗

Vein of Galen aneurysm complicated with pulmonary hypertension: report of one newborn case.

The vein of Galen aneurysm is a rarely seen congenital intracranial vascular malformation with abnormal aneurysmal dilation of the vein of Galen in neonatal stage. We report a full- term female newborn presented with intractable heart failure, pulmonary artery hypertension, and respiratory distress soon after birth, in whom persistent pulmonary hypertension of newborn was suspected initially. Further study by ultrasound revealed turbulent blood flow in the cerebral vascular lesion in the region of vein of Galen; therefore, secondary pulmonary artery hypertension complicated with 'steal' phenomenon was impressed. With the advancement of diagnostic technique, ultrasound provides a rapid and noninvasive method for diagnosing the condition.

Cerebral Veins↗

[Aneurysms of the vein of Galen. Angiographic study and morphogenetic considerations].

The angiographic and/or anatomic study of a series of 30 cases of Vein of Galen aneurysm (which is defined as an arterio-venous fistula within the wall of the vein of Galen itself, therefore as a purely extra cerebral lesion) permits the analysis of the afferent arteries, of the efferent veins, and of the aneurysmal sac itself. The examination of the afferent arteries confirms the data from the literature, indicating that most often it consists of the abnormal hypertrophy of otherwise normally organized arteries, including the dural arteries. In particular, the involvement of distal subcallosal branches of the anterior cerebral arteries as well as the arterio-arterial maze extending above the collicular plate, fit well the normal anatomy. The venous side of the malformation nevertheless presents with more unusual features; the most striking is the non-opacification of the straight sinus and/or part of the transverse sinuses in half of the cases; also, an aberrant falcine sinus is frequently observed, which actually represents the persistence of a normal channel in the fetal anatomy. A more complex pattern was observed in two cases in which the aneurysmal sac drained into the straight sinus via a loop made of a falcine sinus, the superior sagittal sinus and then backward through a second falcine sinus. Finally, from the systematization of the afferents arteries, the aneurysmal sac appears to belong both to the (prosencephalic) velum interpositum and to the ambient cistern. Such a vessel cannot be either the true vein of Galen or an internal cerebral vein; therefore it has to be a persistent fetal vein, the median prosencephalic vein, which drains the choroid plexuses of the lateral and third ventricles between the 7th and 12th weeks, and disappears normally to be replaced by the internal cerebral veins, when the intrinsic vascularization of the neural tube develops. The adult pattern of the brain artery being attained during the 8th week, the event that led to the arterio venous fistula should have occurred some time during the 3rd month. Unfortunately, no clue was found to orient to a specific cause for the anomaly. The frequently observed venous occlusions are actually not seen in neonates but rather in older children. This, and the fact that spontaneous thromboses within the malformative veins have been observed in vivo, make likely the conclusion that venous occlusions are a secondary phenomenon only.

Adolescent↗

[Microsurgical anatomy of the ampulla of Galen].

Anatomic study of 20 injected specimens dissected under operating microscope allowed evaluation of anatomic site of Galen's ampulla, venous confluents of Galen's ampulla, and internal cerebral veins' terminations and their arterial connections in this region. Findings demonstrated that even the wall of the great vein of Galen was supplied by arterioles, as though it was vascularized in the same way as durameter.

Cerebral Arteries↗

Cardiac failure in a case of aneurysm of the vein of Galen associated with diffuse meningeal angiectasia.

A case of aneurysm of the vein of Galen associated with diffuse meningeal angiectasia is described and compared with a similar reported case. The current literature dealing with arteriovenous malformation of the vein of Galen in young children is reviewed. The need for proper clinical and angiographic examination of all newborn infants with congestive cardiac failure due to extracardiac causes is stressed. Attention is drawn to the association of meningeal angiectasia with arteriovenous malformation of the vein of Galen.

Dilatation, Pathologic↗

Galen and his patients.

A prosopography of Galen's patients can contribute to our knowledge of the social and cultural history of Rome in the second century AD. In his voluminous works Galen now and then mentions the patients he actually treated, observed, or whom he saw treated by others. Sometimes he mentions their names, so that we can recognize them from other sources, in the corpus Galenicum or elsewhere. Even if they remain anonymous, or if their data are only partially known, often other relevant information is given e.g. concerning their age, sex, social status etc. This inventory permits some answers to questions concerning Galen's daily routine, his patients, his therapy and his bedside manners.

Complementary Therapies↗