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Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen.

Arteriovenous malformations of the Vein of Galen continue to present diagnostic and therapeutic challenges in the neonatal period. Approximately 40-50% of all malformations of the Vein of Galen present in the neonatal period, usually with congestive heart failure. These neonates represent the most severe cases and are also the most difficult to manage. We report a case of a neonate with a Vein of Galen Malformation who presented with cyanosis, a cardiac murmur, and severe persistent pulmonary hypertension of the newborn. Cardiac failure developed later in the patient's course. The degree of pulmonary hypertension on echocardiography was used to time endovascular embolization of the Vein of Galen Malformation. Following embolization, his pulmonary hypertension subsided dramatically. We speculate that pulmonary hypertension associated with Vein of Galen Malformations has been underestimated in the morbidity and demise of these neonates, and should be more aggressively monitored and treated.

Arteriovenous Malformations↗

Galen and the ventricular system.

This paper examines the anatomy and physiology, together with the pathophysiology, of the ventricular system of the brain, as it was understood by arguably its greatest exponent in Western Antiquity, Galen. According to him, the purpose of the ventricles was to elaborate, store and distribute psychic pneuma, the motive force of Galenic neurology, throughout the nervous system. However, impressive as the delineation of the ventricular system is, the details of this distribution are not forthcoming from Galen. Finally, I discuss the ventricles as the site of intellect, a notion only tentatively advanced by Galen, but cast into dogma by his successors. For all the mistakes Galen made in anatomy and physiology, the study of the ventricular system reveals a mind not dissimilar to our own.

Brain↗

Galen's anecdote of the fallen sophist: on the certainty of science through anatomy.

In this paper the author recounts an anecdote presented by Galen of Pergamum (circa 130-200 A.D.) about a sophist named Pausanias, who fell from his mount and struck his back against a rock. The patient developed a subsequent loss of sensation in the fingers of his left hand with complete sparing of motor function. Numerous medications were applied to his hand but to no avail. Galen stated that he applied the same medications to the original point of dorsal tenderness, resulting in the patient's dramatic and full recovery. Galen attributed the healing to local drug action at the site of a presumed spinal root injury, at the level of C-7. Galen repeated this anecdote elsewhere to illustrate the remote effects of spinal cord and nerve injury and the importance of treating the site of pathology, rather than its somatic manifestations. Galen's observation is interpreted in light of his earlier experiments on spinal cord and nerve transections in live animals and his evolving concepts of functional and correlative neuroanatomy. This anecdote is also discussed as a striking example of the dangers of conjecture and the temptation to confuse association with causation when interpreting the effects of therapy in light of widely accepted paradigms.

Greece, Ancient↗

Microsurgical anatomy of the great cerebral vein of Galen and its tributaries.

OBJECT: The deep cerebral veins may pose a major obstacle in operative approaches to deep-seated lesions, especially in the pineal region where multiple veins converge on the great cerebral vein of Galen. Because undesirable sequelae may occur from such surgery, the number of veins and branches to be sacrificed during these approaches should kept to a minimum. The purpose of this study was to examine venous drainage into the vein of Galen with a view to surgical approaches. If a vein hampering surgical access must be sacrificed, it can therefore be selected according to the smallest draining territory. METHODS: The deep cerebral veins and their surrounding neural structures were examined in 50 cerebral hemispheres from 25 adult cadavers in which the arteries and veins had been perfused with red and blue silicone, respectively. Special consideration was given to the size and location of drainage of the vein of Galen and its tributaries. CONCLUSIONS: When a surgeon approaches the pineal region, several veins may hamper the access route. From posterior to anterior, these include the following: the superior vermian and the precentral or superior cerebellar veins, which drain into the posteroinferior aspect of the vein of Galen; and the tectal and pineal veins, which drain into its anterosuperior aspect. The internal occipital vein is the main vessel draining into the lateral aspect of the vein of Galen. It may be joined by the posterior pericallosal vein, and in that case has an extensive territory. To avoid intraoperative venous infarction, it is important to use angiography to determine the venous organization before surgery and to estimate the permeability and size of the branches of the deep venous system.

Adult↗

[Galen on the topic of abnormalities].

Galen on deformities--Galen's treatment of problems of deformities has its primary source in his twofold teleological conviction that nature acts purposefully and that the human organism and his parts serve their purposes in an optimal way. Thus he defines deformities as functionally deviant body parts. Operative therapy therefore is indicated only if an abnormal part of the body hinders its normal function. On the basis of scientific physiological concepts of his time Galen blames avoidable diatetic failures of the parents as causes of deformities. Galen's engagement on this point reveals the influence of his own experiences as a physician. Galen's statements on deformities show that his teleological concept of nature, his practical medical experience and scientific theories of his time enable him to draw fruitful theoretical and practical conclusions for matters of definition, indication for treatment and prophylaxis of deformities.

Congenital Abnormalities↗

[Choroidal type aneurysmal malformation of the vein of Galen associated with Dandy-Walker malformation in an adult].

Lasjaunias differentiated true vein of Galen aneurysmal malformations (MAVG), which drain to the median prosencephalic vein of Markowski, the embryonic precursor of the vein of Galen, from the aneurysmal dilatations of vein of Galen (VGDA), which reveal venous drainage into a dilated vein of Galen, but already formed. In angioarchitectural terms, MAVG can still be divided in two subtypes: the mural form and the choroidal form, the most common. The authors describe the clinical case of a 18 years-old female, without symptoms until July 2001, who presented an episode of impaired equilibrium associated with vomits and non-specific vision disturbances. This episode had an approximate duration of 6-8 hours with complete recovery. Neuroimaging studies were performed including CT, MRI and digital angiography, disclosed an aneurysmal vein of Galen malformation of choroidal type and Dandy-Walker malformation, association that the authors couldn't found so far described in the literature.

Adolescent↗

[Galen's "On the anatomy of veins and arteries" translation from the Greek text and discussion].

Galen's treatise "On the anatomy of veins and arteries" was translated literally from the Greek text (Kühn's edition, vol. 2, pp. 779-830) into Japanese, utilizing a knowledge of anatomy. The previous Latin and English translations, as well as the descriptions of the vascular system in the other Galenic texts such as "Anatomical Procedures" were utilized as references for the present translation. The present study has identified many branches of veins and arteries, and discussed the influence on anatomy until Vesalius. For example, Galen considered that the main trunk of the portal vein sent off 7 branches, and Avicenna's "Canon" and Mondino's "Anatomia" identified a trunk and 8 branches, whereas Vesalius found bifurcation of the portal vein as in the modern anatomy. As to the vein of the forearm, Galen found 3 superficial veins including the central largest one and Vesalius' "Tabulae anatomicae" depicted this arrangement of veins, whereas Vesalius' 'Fabrica" corrected this erroneous description, based on anatomical findings. The precise understanding of Galenic anatomy provides essential information to understand the developing process of Vesalius' anatomy.

Arteries↗

[Galen's intermittent fever].

In his comprehensive Oeuvre, Galen of Pergamon, who interpreted and perfected Hippocratic medicine, made many times and in various contexts mention of the intermittent fevers, among which malaria undoubtedly held a prominent position. The following article gives an outline of Galen's theoretical concept of this infectious disease, which was of utmost importance for the history of Italy. Galen describes three different types of intermittent fevers, of which, according to his theory of humoural pathology, each one is caused by a special humour with its respective qualities. Thus the quotidian fever is caused by phlegm, the tertian fever by the yellow bile and the quartan fever by the black bile. Apart from the three basic types of fever Galen describes a number of other mixed forms which can either be developed out of identical types or out of different ones. A mixture of a special kind is febris semitertiana: a continuous quotidian is accompanied by an intermittent tertian. It is the worst and most dangerous of these fevers. On the whole Galen's theory of malaria is a paradigm for his forming the reality of diseases on the basis of his pre-knowledge of humoural pathology into a closed system of theory.

Greece, Ancient↗

Arden syntax and GALEN terminology support: a powerful combination to represent medical knowledge.

Arden syntax is an ASTM standard defined a few years ago to represent rule-based knowledge in the medical domain in an application- and implementation-independent way. Knowledge is expressed as Medical Logic Modules (MLMs). So far, it has mainly been used for providing critique and reminders in data driven, medical decision support systems, where typically rules are evoked by certain database operations (e.g., "store systolic blood pressure"). The syntax is an important step towards a general medical knowledge representation, with version control, maintenance information, arithmetic operations, logic rule syntax, and temporal operations, but it is not yet complete. The standard does not define how medical concepts (e.g., "systolic blood pressure") are to be referenced. Existing coding schemes such as SNOMED and ICD10 may be used for this, but they have generally failed to provide the required level of detail. The use of Arden syntax to transfer knowledge between centers in the form of MLMs has been shown to be non-trivial [1,2]. GALEN (Generalized Architecture for Languages, Encyclopedias, and Nomenclatures in Medicine) is an EC AIM project aiming at a machine-readable system to represent medical concepts in a semantic network. The network allows for the combination and specialization of concepts to the required level of detail. The project has resulted in a notation (GRAIL: GALEN Representation and Integration Language) and several tools, such as browsers for manipulating medical concepts. In a GALEN subproject, we are exploring the potential utility of combining Arden syntax with semantics from the GALEN terminology, and we are developing tools to facilitate the creation of MLMs with terminology support. Our impression is that the combination is very useful: GALEN tools allow a domain expert to express her knowledge in purely medical terms, independent of an actual or intended patient database structure and data representation. They can also help a knowledge engineer or quite possibly an automatic interpreter to translate a well defined medical concept (expressed in GRAIL) to actual database queries (in SQL or other notation). This translation can be facilitated by a Data Dictionary describing the local patient database.

Expert Systems↗

[The angiographic classification and endovascular therapy of the vein of Galen aneurysmal malformation].

We treated 11 cases of the vein of Galen aneurysmal malformation, one of which was diagnosed by MRI only, and 10 underwent CAG diagnostic procedure. Among the 10 CAG diagnosed cases, 5 were classified as the vein of Galen aneurysmal malformation (VGAM) with the AV shunt directing to the vein of Galen. The other 5 were classified as the vein of Galen aneurysmal dilitation (VGAD) secondary to parenchymal AVM or dural AVF. 8 cases underwent endovascular treatment. For VGAM, the shunts in the wall of the vein were embolized. For VGAD, the primary AVM or AVF were embolized. The pathphysiology, angiographic characteristics, classification and the principle of endovascular therapy of the vein of Galen aneurysmal malformation were discussed.

Adolescent↗

The GALEN project.

The GALEN project is developing language independent concept representation systems as the foundations for the next generation of multilingual coding systems. It aims to support the flexibility required to cope with the diversity amongst medical applications, while ensuring the coherence necessary for integration and re-use of terminologies. GALEN is developing a fully compositional and generative formal system for modelling concepts: the GALEN Representation and Integration Language (GRAIL) Kernel. Its goal is to overcome many of the problems with traditional coding and classification systems, in particular the combinatorial explosion of terms in enumerative systems and the generation of nonsensical terms in partially compositional systems. It will also provide a clean separation between the concept model and linguistic mechanisms which interpret that model (i.e., the words in a specific language, syntax, alternative phrasings, etc.) in order to allow the development of multilingual systems. GRAIL aims to be formally sound and produce models that are verifiable and contain no contradictions or ambiguities, with realistic human effort. A Coding Reference (CORE) Model of medical terminology covering is being developed which aims to represent the core concepts in for example pathology, anatomy and therapeutics, that have widespread applicability in medical applications. It should also provide the basis for specialist extensions according to the formal principles of GRAIL. The main results of GALEN will be delivered as a Terminology Server (TeS) which encapsulates and coordinates the functionality of the concept module, multilingual module, and code conversion module, and also provides a uniform applications programming interface and network services for use by external applications.

Databases, Bibliographic↗

Rapid normalization of marked dilatation of the cerebral duro-venous system in a newborn infant mimicking a great vein of Galen varix.

A newborn infant with a marked dilatation of the cerebral duro-venous system is presented. The patient was diagnosed as having a vein of Galen aneurysmal varix by a cranial ultrasound examination immediately following delivery. Computed tomographic angiography on the following day, however, showed a marked dilatation of the cerebral duro-venous system, including the great vein of Galen, superior sagittal sinus, torcular herophili and transverse sinuses. There were no arteriovenous fistulas at the vein of Galen. Dilatation of the duro-venous system and concomitant heart failure subsided rapidly after intravenous administration of indomethacin for the treatment of the patent ductus arteriosus on the fourth day of life. Dilatation of the duro-venous system in a newborn infant should be differentiated from any form of vein of Galen aneurysm.

Anti-Inflammatory Agents, Non-Steroidal↗

Temperament profiles of children with vein of Galen malformations.

Unlike many brain injured children who are often rather difficult, toddlers and school-aged children with congenital vein of Galen malformations (n = 20; age range, 4 months to 12 years with a mean of 4 years) evidence a relatively positive temperament profile (based on the Temperament Scales of Carey, McDevitt, Fullard, Hegvik, Medoff-Cooper). They do not, however, differ from normal children with regard to the frequency of easy, difficult, slow to warm up, and intermediate temperament clusters. The temperament profile of children with vein of Galen malformations appears unaffected by additional neurologic abnormalities (hydrocephalus, abnormal developmental quotient, seizure disorder, focal cortical lesions) or other factors including age at testing, sex, or socioeconomic status. Contrary to the findings in adults of right hemisphere dominance for attention, presence of additional right-sided focal cortical pathology was not associated with greater activity level, distractibility, or impersistence. In contrast to the findings in adults and children of right hemisphere dominance for emotions in general, children with vein of Galen malformations and additional right hemisphere cortical damage are not more difficult than those with additional left cortical pathology or no additional focal cortical pathology. The fact that the pathology in vein of Galen malformations is subcortical may explain their relatively easier temperament and the absence of lateralization effects.

Analysis of Variance↗

Treatment of vein of Galen aneurysmal malformation.

The authors report the case of a vein of Galen aneurysmal malformation in a 1-year-old baby presenting with an enlarging head. The lesion was a direct arteriovenous fistula in a dilated vein of Galen and was treated by complete embolization in one session. Four months after occlusion of the shunt, the ectatic vein of Galen and torcular were normal, and the head circumference had stabilized. This case serves as an opportunity to emphasize the quality of results that can be obtained with endovascular techniques. Proper analysis of the vein of Galen angioarchitecture allows planning for appropriate treatment with the lowest possible risk of morbidity and mortality.

Cerebral Angiography↗

[Galen and the refounding of medicine].

The writings of Galen discussed in this article present an outline of the project he devoted his life to. Firstly, the reconstruction of a higher model of medical knowledge, in terms of its epistemological legitimization and its cultural and social role. Galen's refoundation of the epistemological statute of medicine involved increased emphasis on axiomatization, albeit not without disregard to data obtained through experience as a final guarantee. Secondly, the construction of a lay morality, independent of the ties to power or religious constraints. Through anatomy and physiology, Galen was attempting to comprehend the providential notion that governed nature: a powerful ideology set against the uncertainty of philosophers and the propagation of irrationalist religions. From this extreme position, the galenic physician demanded that medicine be not only a general task of ideological and cultural orientation, but also a source of real, particular power over the control of moral behavior and social deviation.

History, Ancient↗

[Giant arteriovenous malformation of the vein of Galen in a 50 year old man].

True malformations of the vein of Galen are usually diagnosed within the first weeks of life. They represent less than 1 p. 100 of the cerebral arteriovenous malformations. These true malformations are exceptional in adults but should be known as an endovascular treatment could be performed. The most frequent clinical presentation is a severe cardiac failure leading to death. We report a giant arteriovenous malformation of the vein of Galen in a right-handed, 50 year-old man. During childhood, he suffered from a compensated cardiac failure which remained of unknown cause. Neurological examination showed kinetic and static cerebellar syndrome and a Parinaud syndrome. A cerebral MRI scan revealed a giant vascular malformation of the vein of Galen with a normal posterior fossa. The angiography enabled the diagnosis of a true malformation of the vein of Galen in its choroidian form. Its high blood flow entails cardiac failure because of a steal phenomenon. An endovascular treatment was declined because of numerous arterial afferences and the potential risks of peroperative haemorrhage.

Cardiac Output, Low↗

[Galen (121-201) and the first experimental test of ureteral function].

Galen and Hippocrates are the two leading representatives of Ancient Greek and Western medicine. Galenic medicine retained an indisputable authority for fifteen centuries. Urology occupies a considerable place in Galen's work. Galen was the first to provide experimental proof of ureteric function.

History, Ancient↗

[The concept of disease in Galen].

Galen was, with no doubt, a great authority in ancient medicine rivalled only with "the father of medicine" Hippocrates. His medicine inherited not only Hippocratic tradition, which is characterized by dynamic pathology, but also Alexandrian medicine, which made a great contribution to anatomy. He did not generalize all the pathological phenomena according to one dogmatic theory. His medicine was open to all kinds of medicine, but he was quite selective in receiving different medical traditions.Galen defined disease as impairment of bodily activities. Whatever that impairs the bodily activities is the cause of disease. Galen' s pathology is built upon two heterogeneous medical traditions: 1) Dynamic pathology of the Hippocratic medicine, but which ignored anatomy 2) Anatomical pathology of Alexandrian medicine. Galen integrated these two different traditions by his concept of disease. His definition of disease, impairment of bodily activities, made it possible to harmorize these two different traditions otherwise which would have been conflictual. It is Galen' s great contribution to Western medicine to have laid a foundation of pathology by combining physiological and anatomical point of view .

Disease↗