J. Neurol. Neurosurg. Psychiat., 1969, 32, 308-312

Fistula between three main cerebral arteries anda large occipital vein

J. BRET AND Z. KUNC

From the Neurosurgical Clinic of the Charles University, Prague, Czechoslovakia

The literature contains few references to directcommunications between cerebral arteries and veins.Dandy (1945) was the first to describe the passageof a branch from the middle cerebral artery into avein leading into the Sylvian group. Jaeger andForbes (1946) reported direct communicationsbetween the posterior cerebral and posteriorchoroid arteries and the basilar vein, together withmultiple anastomoses with the great vein of Galen.Haberland (1950) published an account of theaccidental finding of a communication between thebranches of the posterior cerebral artery andthe dilated vein of Rozenthal and proved it micro-scopically. Bergstrand, Olivecrona, and Tonnis(1936), Rottgen (1937), Sorgo (1938), and Verbiest(1951) reported observations of intradural directarteriovenous communications. Some cases ofvascular malformations of the great vein of Galenmay also belong to this category, although they aregenerally regarded as a separate entity.The following report describes an abnormality

which is unique in our experience, and we havenot encountered a similar one in our survey of theliterature.

CASE REPORT

A 26-year-old man was first seen in September 1961.From childhood he had suffered from right-sidedheadaches, mainly in the temporal region and below theeyebrow, the pain being treated as migraine. As timewent on, the headache ceased to trouble him, but hethen began to complain of attacks of vertigo, associatedwith nausea or vomiting, usually lasting about one hourand being so severe that they forced him to go to bedfor fear of falling. It was for these attacks that he soughtadvice.The neurological examination and funduscopy at

that time were normal. Skull films and tomogramsrevealed a hyperostosis in the left fronto-parietal region,growing from the outer table, with a circular defect,limited sharply by sclerotic bone. In the tangential viewthere was thickening of the inner table in the base of thehyperostosis. These appearances were suggestive of ameningioma, and angiography was undertaken with thispossibility in mind.

LEFT CAROTID ANGIOGRAM (Fig. 1) This showed adilatation of the internal carotid artery at its siphon andof the anterior cerebral artery in its first part. Themajority of the contrast medium was drawn throughthe anterior communicating artery to the right anteriorcerebral artery, which was dilated to a width of 5 mm inthe early part of its course and 7 mm further posteriorly.It terminated directly into an enormous occipital veinrunning to the sagittal sinus. There was poor visualizationof the territory supplied by the left anterior and middlecerebral arteries, indicating that most of the blood wasgoing to the fistula.

RIGHT CAROTID ANGIOGRAM (Fig. 2) This also showeddilatation of the internal carotid artery. The beginning ofthe anterior cerebral artery was not seen. The middlecerebral artery was greatly enlarged, elongated, meander-ing, and terminated in the same anomalous occipitalvein as had been seen in the previous angiogram. Thevein wound in large loops to the sagittal sinus. Thesagittal and both transverse sinuses were also dilated.As on the left side, the blood supply of the righthemisphere appeared to be deficient, because most of thecirculation was going to the fistula.

ANGIOGRAM OF THE LEFT VERTEBRAI. ARTERY (Fig. 3a, b)This showed that the vertebral and basilar arteries wereenlarged, elongated, and winding. Almost all the contrastmaterial passed into the right posterior cerebral artery,which was dilated and ran in big curves into theanomalous occipital vein. The cerebellar vessels werepoorly visualized.

Total serial angiography confirmed these circulatoryanomalies. The patient refused surgical treatment, as hedid not consider that his complaints were troublesomeenough at this time. He was readmitted on 2 February1964 because the headaches had recurred, and becausehe had had several attacks of transient weakness of theright hand, and on two occasions loss of consciousness.The neurological state remained normal.At operation on 25 February 1964 a right occipito-

parietal craniotomy was performed. On the surface ofthe brain an anomalous vein, as large as a thumb, ranfrom the occipital to the parietal lobe, forming a largevarix just before its termination in the sagittal sinus.The arachnoid was thickened over the whole of thisregion. From all directions large arteries entered the veinso that a thrill could be felt on palpation. When allthese arteries had been ligated, the tension in the vein

308

Protected by copyright.

on June 6, 2022 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 A

ugust 1969. Dow

nloaded from

Fistula between three main cerebral arteries and a large occipital vein 309

FIG. 1. Before operation.

FIG. 2. Before operation.

Protected by copyright.

on June 6, 2022 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 A

ugust 1969. Dow

nloaded from

J. Bret and Z. Kunc

FIG. 3b

FIG. 3 Before operation.

greatly decreased and the thrill disappeared. Then thegigantic vein with a part of the brain was removed.The procedure was extremely difficult, as the walls of thevessels were very fragile and, in spite of careful dissection,often tore with violent bleeding, serious blood loss, anddrop of the arterial pressure. The patient received ablood transfusion of 4,100 ml. The procedure took fourhours.For some days after the operation the patient was

somnolent and confused and had a left-sided hemiparesis.This complication cleared up rapidly, but a superficialwound infection required antibiotic therapy for severaldays. He was discharged in good mental and physicalcondition. Control angiography was carried out on1 April 1964 (Fig. 4a, b, c, d). The cerebral circulation inboth hemispheres now appeared to bevirtually normal. Allof the main cerebral arteries on the right side haddiminished in size and the arteriovenous communicationswere no longer visible. Both of the carotid arteries weresmaller and irregularly narrowed, but there was ananeurysmal dilatation of the origin of the posteriorcommunicating artery on the left side.

COMMENT The vascular anomaly described above wouldexplain the chronic headache of which the patient hadcomplained since his childhood. All three main cerebralarteries on the right side terminated in an occipito-parietal vein which emptied into the sagittal sinus. Allthese vessels were enormously dilated, and the fistulouscommunication between them led to serious deprivationof the blood supply to the whole brain. The radicalremoval of the malformation led to a complete relief ofsymptoms.

DISCUSSION

This vascular anomaly is unique in its form, andin the literature no similar observation could befound. It is the nearest to the one described byDandy (1945), but in comparison is more com-plicated and larger. In Haberland's (1950) case thearteriovenous communication was microscopic. Inother cases described in the literature the lesion hasbeen situated in the dura mater and the arterialcommunications were mostly from the meningealarteries. The malformation which we have describedis more primitive than in the typical arteriovenousmalformations. Between the feeding arteries anddraining vein there was no system of transit vascularchannels, and so the arterial blood stream randirectly into the enormously dilated cerebral vein.

This arteriovenous fistula is the result of adevelopmental defect of the capillary system in theearly stage of embryonic development of the cerebralvascular system, as in arteriovenous malformations.It is possible that embryonic vessels, destined to bearteries and veins, may cross in such a way that theyare separated only by two layers of endothelial cellsand that this barrier gradually disappears under theinfluence of the arterial pulse.

It is probable that the abnormality, present atbirth and enlarging steadily thereafter, was respon-sible for the long-standing headaches. Progressiveenlargement of the contributing vessels increased

310

FIG. 3a

T. M:

Protected by copyright.

on June 6, 2022 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 A

ugust 1969. Dow

nloaded from

Fistula between three main cerebral arteries and a large occipital vein

the power of the shunt, and led to a deficit in thecerebral and cerebellar blood supply, witnessed byvertigo, transient hemianopia, and attacks of lossof consciousness. Haberland's findings suggest thatarteriovenous communications, at first only micro-scopic, open successively during life. In our case,the normal development of the brain, as indicatedby intact mentality and the absence of neurologicalsigns, suggests that the shunt, as seen at the time of

angiography and operation, may have been presentat birth, but enlarged progressively throughoutlife.The indication for surgery was clear, not only

because of severe headache but also because of theprospect of the cerebral circulation becoming moreembarrassed, leading to the grave mental changeswhich are known to occur in large arteriovenousmalformations.

FIG. 4a FIG. 4b

FIG. 4c

FIG. 4d

FIG. 4 After operation.

...::;:;:::y ::::

a| |- t.| I e: w ... 9s: : ,.4, .... F*:.: -: ... . . 1....... .. w }.

::: |

..

.. .::.: ::: ... : :'.:::*: :: . ; . ... : |. :: . .: .... : :: .: . . :: ^: ': 4

p

311

Protected by copyright.

on June 6, 2022 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 A

ugust 1969. Dow

nloaded from

J. Bret and Z. Kunc

SUMMARY

A case of unique multiple arteriovenous fistulaebetween all three main cerebral arteries-anterior,middle, and posterior arteries-and a corticaloccipito-parietal vein, is reported. The malformationdisturbed the cerebral circulation and caused severe

headaches, vertigo, transient hemianopia, andattacks of loss of consciousness. Surgical ablation ofthe fistula relieved these symptoms and restored thecirculation to normal. A possible explanation ofthe abnormality is offered.

REFERENCES

Bergstrand, H., Olivecrona, H., and Tdsnnis, W. (1936). Gefassmiss-bildangen and Gefdssgeschwulste des Gehirns. Thieme: Leipzig.

Dandy, W. E. (1945). Surgery of the brain, in Lewis' Practice of

Surgery, Vol. 12. Edited by W. Walters. W. F. Prior: Hagers-

town, Maryland.Haberland, C. (1950). Arteriovenous anastomosis on the base of the

brain. Mschr. Psychiat. Neurol., 119, 199-206.Jaeger, R., and Forbes, R. P. (1946). Bilateral congenital arterio-

venous communications of the cerebral vessels. Arch. Neurol.Psychiat. (Chic.), 55, 591-599.

Rottgen, P. (1937). Weitere Erfarungen an kongenitalen arterio-venosen Aneurysmen des Schadelinnern. Zbl. Neurochir.,2, 18-34.

Sorgo, W. (1938). Weitere Mitteilungen uber Klinik und Histologiedes kongenitalen arteriovenosen Aneurysmas des Gehirns.

Zbl. Neurochir., 3, 64-87.Verbiest, M. H. (1951). L'an&vrisme art6rioveineux intradural. Rev.

Neurol., 85, 189-199.

312

Protected by copyright.

on June 6, 2022 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.32.4.308 on 1 A

ugust 1969. Dow

nloaded from