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A Large Occipital Vein J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 August 1969. Downloaded from J. Neurol. Neurosurg. Psychiat., 1969, 32, 308-312 Fistula between three main cerebral arteries and a large occipital vein J. BRET AND Z. KUNC From the Neurosurgical Clinic of the Charles University, Prague, Czechoslovakia The literature contains few references to direct LEFT CAROTID ANGIOGRAM (Fig. 1) This showed a communications between cerebral arteries and veins. dilatation of the internal carotid artery at its siphon and Dandy (1945) was the first to describe the passage of the anterior cerebral artery in its first part. The the middle cerebral artery into a majority of the contrast medium was drawn through of a branch from the anterior communicating artery to the right anterior vein leading into the Sylvian group. Jaeger and cerebral artery, which was dilated to a width of 5 mm in Forbes (1946) reported direct communications the early part of its course and 7 mm further posteriorly. between the posterior cerebral and posterior It terminated directly into an enormous occipital vein choroid arteries and the basilar vein, together with running to the sagittal sinus. There was poor visualization multiple anastomoses with the great vein of Galen. of the territory supplied by the left anterior and middle Haberland (1950) published an account of the cerebral arteries, indicating that most of the blood wasguest. Protected by copyright. accidental finding of a communication between the going to the fistula. branches of the posterior cerebral artery and it micro- RIGHT CAROTID ANGIOGRAM (Fig. 2) This also showed the dilated vein of Rozenthal and proved dilatation of the internal carotid artery. The beginning of scopically. Bergstrand, Olivecrona, and Tonnis the anterior cerebral artery was not seen. The middle (1936), Rottgen (1937), Sorgo (1938), and Verbiest cerebral artery was greatly enlarged, elongated, meander- (1951) reported observations of intradural direct ing, and terminated in the same anomalous occipital arteriovenous communications. Some cases of vein as had been seen in the previous angiogram. The vascular malformations of the great vein of Galen vein wound in large loops to the sagittal sinus. The may also belong to this category, although they are sagittal and both transverse sinuses were also dilated. generally regarded as a separate entity. As on the left side, the blood supply of the right The following report describes an abnormality hemisphere appeared to be deficient, because most of the which is unique in our experience, and we have circulation was going to the fistula. not encountered a similar one in our survey of the ANGIOGRAM OF THE LEFT VERTEBRAI. ARTERY (Fig. 3a, b) literature. This showed that the vertebral and basilar arteries were enlarged, elongated, and winding. Almost all the contrast CASE REPORT material passed into the right posterior cerebral artery, which was dilated and ran in big curves into the was in September 1961. anomalous occipital vein. The cerebellar vessels were A 26-year-old man first seen http://jnnp.bmj.com/ From childhood he had suffered from right-sided poorly visualized. headaches, mainly in the temporal region and below the Total serial angiography confirmed these circulatory eyebrow, the pain being treated as migraine. As time anomalies. The patient refused surgical treatment, as he went on, the headache ceased to trouble him, but he did not consider that his complaints were troublesome then began to complain of attacks of vertigo, associated enough at this time. He was readmitted on 2 February with nausea or vomiting, usually lasting about one hour 1964 because the headaches had recurred, and because and being so severe that they forced him to go to bed he had had several attacks of transient weakness of the for fear of falling. It was for these attacks that he sought right hand, and on two occasions loss of consciousness. state remained normal. advice. The neurological on September 23, 2021 by The neurological examination and funduscopy at At operation on 25 February 1964 a right occipito- that time were normal. Skull films and tomograms parietal craniotomy was performed. On the surface of revealed a hyperostosis in the left fronto-parietal region, the brain an anomalous vein, as large as a thumb, ran growing from the outer table, with a circular defect, from the occipital to the parietal lobe, forming a large limited sharply by sclerotic bone. In the tangential view varix just before its termination in the sagittal sinus. there was thickening of the inner table in the base of the The arachnoid was thickened over the whole of this hyperostosis. These appearances were suggestive of a region. From all directions large arteries entered the vein meningioma, and angiography was undertaken with this so that a thrill could be felt on palpation. When all possibility in mind. these arteries had been ligated, the tension in the vein 308 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 August 1969. Downloaded from Fistula between three main cerebral arteries and a large occipital vein 309 guest. Protected by copyright. FIG. 1. Before operation. http://jnnp.bmj.com/ on September 23, 2021 by FIG. 2. Before operation. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 August 1969. Downloaded from 310 J. Bret and Z. Kunc T. M: FIG. 3a FIG. 3b guest. Protected by copyright. FIG. 3 Before operation. greatly decreased and the thrill disappeared. Then the DISCUSSION gigantic vein with a part of the brain was removed. The procedure was extremely difficult, as the walls of the This vascular anomaly is unique in its form, and vessels were very fragile and, in spite of careful dissection, in the literature no similar observation could be often tore with violent bleeding, serious blood loss, and found. It is the nearest to the one described drop of the arterial pressure. The patient received a by blood transfusion of 4,100 ml. The procedure took four Dandy (1945), but in comparison is more com- hours. plicated and larger. In Haberland's (1950) case the For some days after the operation the patient was arteriovenous communication was microscopic. In somnolent and confused and had a left-sided hemiparesis. other cases described in the literature the lesion has This complication cleared up rapidly, but a superficial been situated in the dura mater and the arterial wound infection required antibiotic therapy for several communications were mostly from the meningeal days. He was discharged in good mental and physical arteries. The malformation which we have described condition. Control angiography was carried out on is more primitive than in the typical arteriovenous 1 April 1964 (Fig. 4a, b, c, d). The cerebral circulation in both hemispheres now appeared to bevirtuallynormal. All malformations. Between the feeding arteries and http://jnnp.bmj.com/ of the main cerebral arteries on the right side had draining vein there was no system of transit vascular diminished in size and the arteriovenous communications channels, and so the arterial blood stream ran were no longer visible. Both of the carotid arteries were directly into the enormously dilated cerebral vein. smaller and irregularly narrowed, but there was an This arteriovenous fistula is the result of a aneurysmal dilatation of the origin of the posterior developmental defect of the capillary system in the communicating artery on the left side. early stage of embryonic development of the cerebral vascular system, as in arteriovenous malformations. COMMENT The vascular anomaly described above would It is possible that embryonic vessels, destined to be on September 23, 2021 by explain the chronic headache of which the patient had arteries and veins, may cross in such a way that they complained since his childhood. All three main cerebral are separated only by two layers of endothelial cells arteries on the right side terminated in an occipito- and that this barrier under the parietal vein which emptied into the sagittal sinus. All gradually disappears these vessels were enormously dilated, and the fistulous influence of the arterial pulse. communication between them led to serious deprivation It is probable that the abnormality, present at of the blood supply to the whole brain. The radical birth and enlarging steadily thereafter, was respon- removal of the malformation led to a complete relief of sible for the long-standing headaches. Progressive symptoms. enlargement of the contributing vessels increased J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 August 1969. Downloaded from Fistula between three main cerebral arteries and a large occipital vein 311 the power of the shunt, and led to a deficit in the angiography and operation, may have been present cerebral and cerebellar blood supply, witnessed by at birth, but enlarged progressively throughout vertigo, transient hemianopia, and attacks of loss life. of consciousness. Haberland's findings suggest that The indication for surgery was clear, not only arteriovenous communications, at first only micro- because of severe headache but also because of the scopic, open successively during life. In our case, prospect of the cerebral circulation becoming more the normal development of the brain, as indicated embarrassed, leading to the grave mental changes by intact mentality and the absence of neurological which are known to occur in large arteriovenous signs, suggests that the shunt, as seen at the time of malformations. guest. Protected by copyright. FIG. 4a FIG. 4b ...::;:;::: y :::: |a|- t. 4 ....... .. w }. ::: | .. p .. .: '.::: *:.:s:| I : e: ,.4, -: w ........... F 9 . 1 http://jnnp.bmj.com/ *::.:. :: . :: .: ::: .... ;: ::.....: ... .: :: :^: :': | on September 23, 2021 by FIG. 4c FIG. 4d FIG. 4 After operation. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 August 1969. Downloaded from 312 J.
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