Clinical syndromes ofarteriovenous malformations …Mentaldisorders 4 4 Brain-stemsyndrome 1 origin...

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Journal of Neurology, Neurosurgery, and Psychiatry, 1975, 38, 436-451 Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus' S. OBRADOR, M. SOTO, AND J. SILVELA From the Department of Neurosurgery of the Spanish Social Security (C.S. La Paz) and the Faculty of Medicine of the Autonomous University, Madrid, Spain SYNOPSIS Arteriovenous malformations or fistulae shunting arterial blood from branches of the external and internal carotid and vertebral arteries into the transverse-sigmoid sinus may produce different clinical syndromes. The literature is reviewed with 96 patients including six personal cases. Usually these malformations have a congenital origin and only in 40o of the series was there a previous history of a severe head injury. Clinical groups are defined and the role of angiography assessed. Direct surgical approach with occlusion or removal of the vascular malformation is the treatment of choice. Possible methods of treatment by selective embolization are discussed. During the past 20 years an interesting condi- tion named intradural arteriovenous malforma- tion (AVM), arteriovenous fistula (AVF), or arteriovenous aneurysm (AVA) of the region of the transverse-sigmoid sinus has been recognized. According to some reviews (Laine et al., 1963; Verbiest, 1968a, b; Aminoff, 1973) there were a few early observations in the 1930s of such dural vascular malformations sometimes also involving the petrous bone and extracranial soft tissue. The two cases of Tonnis (1936) from Olivecrona's series and the patient of Rottgen (1937) demon- strated by angiography are examples of these intradural aneurysms in adults. Verbiest (1951) published a case that developed an intracranial bruit after a head injury and at operation found an AVA of the tentorium and sigmoid sinus but the angiography did not show the vascular malformation. The same year Obrador and Urquiza (1951) reported the first case of a large AVM of the transverse-sigmoid sinus demonstrated at angiography. It was accompanied by a severe bruit and produced an infantile hydrocephalus. Since then other cases have been recorded in the literature under different names: intradural 1 Paper presented at the Joint Meeting of the Dutch and Spanish- Portuguese Societies of Neurosurgery, Utrecht, May 1974. (Accepted 12 September 1974.) 436 AVA or angiomas; dural AVM of the tentorium, posterior fossa or sigmoid sinus; tentorial AVM, AVF or communications between middle meningeal artery, occipital artery, or external carotid artery with the transverse sinus. As has been properly remarked by Houser et al. (1972), these lesions were rarely identified before 1960 and are emerging now as distinct entities due to the great advances of angio- graphy. These authors also estimate that the various types of dural AVA represent about 10 to 15% of all the intracranial AVA, though they found only 65 cases reported in the literature at the time of their review (Houser et al., 1972). A careful revision of the literature has been carried out and 96 cases collected, including six personal observations. (see Appendix Table). Four of these have been reported earlier especially from the angiographic point of view (Fernaindez Urdanibia et al., 1974). In most of the reported cases of dural or intradural AVM or AVF there is not enough information for an adequate grouping in separate clinical entities. Thus, only some of the collected observations can be considered in the evaluation of certain features. These AVM or AVF have been related by Aminoff (1973) to the main drainage sinuses. The cases with an anterior and inferior drainage Protected by copyright. on August 7, 2020 by guest. http://jnnp.bmj.com/ J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.38.5.436 on 1 May 1975. Downloaded from

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Page 1: Clinical syndromes ofarteriovenous malformations …Mentaldisorders 4 4 Brain-stemsyndrome 1 origin as in the cases ofObrador and Urquiza (1951), vanderWerf(1964), andothers. Arteriovenous

Journal ofNeurology, Neurosurgery, and Psychiatry, 1975, 38, 436-451

Clinical syndromes of arteriovenous malformationsof the transverse-sigmoid sinus'

S. OBRADOR, M. SOTO, AND J. SILVELA

From the Department of Neurosurgery of the Spanish Social Security (C.S. La Paz)and the Faculty of Medicine of the Autonomous University, Madrid, Spain

SYNOPSIS Arteriovenous malformations or fistulae shunting arterial blood from branches of theexternal and internal carotid and vertebral arteries into the transverse-sigmoid sinus may producedifferent clinical syndromes. The literature is reviewed with 96 patients including six personal cases.

Usually these malformations have a congenital origin and only in 40o of the series was there a

previous history of a severe head injury. Clinical groups are defined and the role of angiographyassessed. Direct surgical approach with occlusion or removal of the vascular malformation is thetreatment of choice. Possible methods of treatment by selective embolization are discussed.

During the past 20 years an interesting condi-tion named intradural arteriovenous malforma-tion (AVM), arteriovenous fistula (AVF), orarteriovenous aneurysm (AVA) of the region ofthe transverse-sigmoid sinus has been recognized.

According to some reviews (Laine et al., 1963;Verbiest, 1968a, b; Aminoff, 1973) there were afew early observations in the 1930s of such duralvascular malformations sometimes also involvingthe petrous bone and extracranial soft tissue.The two cases ofTonnis (1936) from Olivecrona'sseries and the patient of Rottgen (1937) demon-strated by angiography are examples of theseintradural aneurysms in adults.

Verbiest (1951) published a case that developedan intracranial bruit after a head injury and atoperation found an AVA of the tentorium andsigmoid sinus but the angiography did not showthe vascular malformation. The same yearObrador and Urquiza (1951) reported the firstcase of a large AVM of the transverse-sigmoidsinus demonstrated at angiography. It wasaccompanied by a severe bruit and produced aninfantile hydrocephalus.

Since then other cases have been recorded inthe literature under different names: intradural

1 Paper presented at the Joint Meeting of the Dutch and Spanish-Portuguese Societies of Neurosurgery, Utrecht, May 1974.(Accepted 12 September 1974.)

436

AVA or angiomas; dural AVM of the tentorium,posterior fossa or sigmoid sinus; tentorialAVM, AVF or communications between middlemeningeal artery, occipital artery, or externalcarotid artery with the transverse sinus.As has been properly remarked by Houser et

al. (1972), these lesions were rarely identifiedbefore 1960 and are emerging now as distinctentities due to the great advances of angio-graphy. These authors also estimate that thevarious types of dural AVA represent about 10 to15% of all the intracranial AVA, though theyfound only 65 cases reported in the literature atthe time of their review (Houser et al., 1972).A careful revision of the literature has been

carried out and 96 cases collected, including sixpersonal observations. (see Appendix Table).Four of these have been reported earlierespecially from the angiographic point of view(Fernaindez Urdanibia et al., 1974).

In most of the reported cases of dural orintradural AVM or AVF there is not enoughinformation for an adequate grouping in separateclinical entities. Thus, only some of the collectedobservations can be considered in the evaluationof certain features.These AVM or AVF have been related by

Aminoff (1973) to the main drainage sinuses.The cases with an anterior and inferior drainage

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Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus 437

TABLE 1CLINICAL FINDINGS

Case no. Sex Age of Length of Symptonisonset history(yr) First Other

Neurological examination

M 57 3 m Headaches,mentaldeterioration

M 43 Hours Headaches,L hemiparesis

3 F 48 12 yr(afterheadtrauma)

Headaches

4 r t6 15 d Headaches,vomiting, lossof vision, bruit(SAH)

5 F 59 8 m Loss of memory, Fitsdisorientation

Fits, hyper- Stuporous, papilloedema,somnia, gait L hemiparesis, bilateralunstable tremor

- Disorientation, papilloedema,L hemiparesis, hemi-hypaesthesia

Bruit, loss of Bruit, papilloedema,vision, nystagmustinnitus, gaitunstable

Bruit, meningeal irritation, IL homonymous hemi-anopsia, hemiparesis,hemihypaesthesia, nystagmus

Mental deterioration, papil- ]loedema, R hemiparesis

6 F 54 9 m Tinnitus Headaches, Papilloedema, VI nervediplopia, loss paresisof vision

Normal R frontotemporalfocus,centrencephalicdysfunction

Increased R frontotemporalvascular focuschannels

Increased L temporal focusvascularchannels

Increasedvascularchannels

L temporal focus

Normal Diffuse corticaland centren-cephalic dys-function

Normal Normal

TABLE 2ANGIOGRAPHIC FINDINGS

Case no.and site

1 R TS Meningeal branciPosterior meniartery

2 R TS Meningeal branc]arteries, R mai

3 R and L Meningeal brancdTSS arteries. Poster

Arterial supply Primary Anterograde Retrograde venous Sinusvenous venous flow drainage obstructiondrainage (along usual

channels)hes of R occipital artery. R TS - Cortical veins. Straight R SSingeal branch of R vertebral sinus-internal cerebral

veins-basal veinshses of R and L occipital R TS - Cortical veins. Straight R SSrginal tentorial artery sinus-internal cerebralveins-basal veinshes of R and L occipital R TSS L jugular veinior branches of R and L L TS

middle meningeal arteries. Lateral tentorialarteries of R and L internal carotid arteries.Deep cervical artery of the R subclavianartery

4 R TS Meningeal branches of R and L occipitalarteries. Posterior branches of R middlemeningeal artery

5 R TSS Meningeal branches of R and L occipitalarteries. Posterior branch of L middlemeningeal artery

6 R TSS Meningeal branches of R occipital artery.Lateral tentorial artery of R internalcarotid artery

Cortical veins R jugular veinR TS

L TSS

R TSS

- L vein of Labbe-super-ficial middle cerebralvein-cavernous sinus

Distal portionof SS

Distal portionof R SS

T: transverse. S: sinus. SS: sigmoid sinus.

receive the blood from both carotid arteries intothe cavernous, intercavernous, sphenoparietal,and superior or inferior petrosal sinuses. Thereare several observations of such arteriovenousconnections but much more frequent are theAVF of the superior-posterior group of Aminoff

(1973) draining into the superior and inferiorsagittal sinuses or the straight, transverse, sig-moid, and occipital sinuses.Of these groups of dural vascular malforma-

tions, the more frequent and of greater clinicalinterest are the AVF or AVM that shunt and

Skullradiograph

EEG

II

I

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S. Obrador, M. Soto, and J. Silvela

drain the blood from different branches of theexternal and internal carotid arteries and verte-bral arteries into the transverse-sigmoid sinus.The haemodynamic changes induced by theselesions at the region of the most important out-flow of intradural blood may give rise to agreat variety of symptoms and signs.

PERSONAL MATERIAL

Tables 1, 2, and 3 summarize the clinical andangiographic findings as well as the spontaneousevolution or the course after different thera-peutic measures in the six cases observed inrecent years.

TABLE 3RESULTS OF SURGICAL TREATMENT

Case Surgical treatment Follow-upno.

1 Ligation of branches of external Died 9 d latercarotid arteries

2 - Hemiparesis3 Bilateral external carotid artery Headaches disappeared,

ligation bruit persists4 Occipital craniotomy. Ligation Complete recovery

of arterial supply. Drainageof intracerebral haematoma

5 Ventriculo-peritoneal shunt Died 13 d later6 - Headaches improved,

papilloedema con-tinues

It is interesting that five of these patients(cases 1, 2, 3, 5, and 6) presented a clinical syn-drome with increased intracranial pressure andpapilloedema and that only one of them (case 3)had an audible bruit. Case 4 presented a sub-arachnoid haemorrhage with a bruit and signs ofan intracranial vascular lesion.

AETIOLOGY AND PATHOLOGY

Most authors consider these dural AV fistulae ascongenital malformations between the arterialbranches and the basal emissary veins that drainthe extracranial structures into the dural sinuses(Takekawa and Holman, 1965; Newton andGreitz, 1966). The presence of these lesions insmall children also favours their congenital

TABLE 4MAIN SYMPTOMS (92 CASES RECORDED)

Symptom Total First symptom

(No.) (Y.) (No.) (Y)

Cranial bruit 62 67 43 47Headaches 46 50 17 18

(4 with bruit)Subarachnoid haemorrhages 19 20 12 13Seizures 14 15 4 4Tinnitus 14 15 10 11Visual failure 12 13 2 2Mental deterioration 11 12 4 4Exophthalmos, proptosis,

ocular congestion and pain 9 10 4 4Hemiparesis 5 5 3 3Gait disturbance 4 4 - -Vomiting 5 5 1 1Diplopia 3 3 1 1Speech disturbance 3 3 1 1Transient ischaemic episodes 3 3 3 3Vertigo 2 2 - -

Hydrocephalus 2 2 2 2(with bruit)

Hypacusis 1 1 -

Heart failure 1 1 1 1Dilated scalp veins 1 1 1 1

TABLE 5CLINICAL SIGNS (91 CASES RECORDED)

(No.) (%) (No.) (Y.)Bruit 62 68 Increased scalp veins 3 3Papilloedema 22 24 VII nerve palsy 3 3Meningeal signs 12 13 Palpable pulsatileHemiparesis 11 12 mass 3 3Hemianopsia 5 5 VI nerve palsy 2 2Diplopia 5 5 Nystagmus 2Hydrocephalus 4 4 Ataxia 3 3Exophthalmos 4 4 Tremor 1 1Proptosis 4 4 Heart failure 1Amblyopia 5 5 III nerve palsy 1Cerebellar signs 4 4 Loss of hearing 1Impairment of V nerve palsy I

consciousness 4 4 Optic atrophy 1Mental disorders 4 4 Brain-stem syndrome 1

origin as in the cases of Obrador and Urquiza(1951), van der Werf (1964), and others.

Arteriovenous malformations of the intra-cranial dura mater may also appear in routinenecropsies of patients without any neurologicalcomplaints. McCormick and Boulter (1966)described two examples of this kind with angio-matous masses in the region of the tentoriumand torcular Herophili and mentioned anotherthree cases of such dural angiomas recorded inthe literature. These AVM may also be an inci-

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TABLE 6GROUPING OF CLINICAL SYNDROMES

(76 CASES RECORDED)

Group Signs and symptoms (No.) (%)

I Only subjective complaints due to cranial 27 35bruit and tinnitus secondary to AVshunt (A)

II Neurological signs due to cerebral ischaemia 8 10(B) secondary to AV shunt (A) (with bruit 4)

III Headaches, papilloedema, and visual failure 13 17due to AV shunt (A) and increased venous (with bruit 9)pressure (C)

IV Infantile hydrocephalus with bruit due to 3 4factors A and C

V Subarachnoid haemorrhages due to ruptured 13 17pial vessels (with bruit 7)

(II and III 10 13

VI Combination of previous groupsII and V (with bruit 3)

III and V 1 I

dental and asymptomatic finding during angio-graphies carried out for other cerebral con-ditions (Aminoff, 1973; Aminoff and Kendall,1973).According to Houser et al. (1972) nearly all

these fistulae are direct communications fromdural arteries to dural veins or to both dural andpial veins. Aminoff (1973) also emphasizes thisdirect communication of the arterial branches ofthe external and internal carotid arteries withdural veins and sinuses. Their higher frequencyalong the cranial base and tentorial region mayperhaps be explained by the delay in the embryo-logical development of the external carotidartery and by the existence at this location of agreat number of emissary veins (Takekawa andHolman, 1965; Houser et al., 1972).Angiography also visualizes the direct com-

munication between the arteries and venoussinuses without an interposed capillary system(Debrun and Chartres, 1972). In some casessome interposed and dilated arteriovenous sacsor channels joined the dural arteries to the pialveins (Houser et al., 1972). Histological study ofthese dural AVM reveals masses of multiple andtortous venous channels of various sizes (Debrunand Chartres, 1972; Aminoff, 1973).Although these AVM usually are entirely

dural they may also have a cerebral pial com-ponent (Verbiest, 1968a, b; our case 4) and, ashas been properly emphasized by Debrun andChartres (1972), unless a direct surgical verifica-

TABLE 7NINETY-SIX ARTERIOVENOUS MALFORMATIONS

OF TRANSVERSE-SIGMOID SINUS

Numberof casesF Age (recordedin 96 cases)

X

51-06f0 -S er

o-1

Sex and duration ofsymptoms

Sex (recorded in 95 cases) (No.)

Male 47Fenmale 48

Duration of synmptoms (yr) (Cases)(recorded in 66 cases)

< 1 431-2 52-3 63-4 24-5 25-6 17-8 18-9 1

>10 5

tion has been performed it is difficult to statewhether involvement of the cerebral vesselsthrough corticomeningeal anastomoses is present.In the study of two surgical specimens Houser etal. (1972) demonstrated that the AVM drainedby pial veins formed a dilated vascular sac lyingin the leptomeninges and associated with intra-cranial haemorrhages. In one of these two casesthe haemorrhage extended not only into thesubarachnoid and subdural spaces but also intothe cerebral parenchyma.

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S. Obrador, M. Soto, and J. Silvela

FIG. 1. Case 5. (a) Left common carotid angiogram. Opacification of a short segment of the left sinus (largearrow) from branches of the left occipital artery and the posterior branch of the left middle meningeal artery(long arrows). (b) Retrograde flow into the left vein ofLabbe (arrow) from the above-mentioned segment ofthe left lateral sinus.

These arteriovenous malformations may alsoextend outside the cranium and Verbiest (1968a)separates the purely intradural AVA of theposterior fossa from those more rare ones thatnot only involve the dura mater but also theextracranial soft tissues and the petrosal bone.The association of these dural malformations

with other vascular lesions like intracranialarterial aneurysms has also been recorded byVerbiest (1968b), Houser et al. (1972), andothers.

Other than the congenital origin of the duralAVM aetiological factors like trauma or localinflammatory diseases have also been con-sidered (Aminoff, 1973). In the review of theliterature and personal cases a previous historyof a significant head injury with enough data onthis point appeared in only 400 of 92 cases.

CLINICAL SYNDROMES

It is very important, in our view, to defineproperly and to separate the different clinicalsyndromes of these patients suffering from duralAVM of the transverse-sigmoid sinus. Previousattempts have neither been very systematic norcomplete (Laine et al., 1963; Nicola andNizzoli, 1968; Verbiest, 1968; Debrun and

Chartres, 1972; Houser et al., 1972; Aminoff,1973), mainly because a large number of well-documented cases are necessary for such group-ing of clinical syndromes.

Several factors have to be considered in orderto understand the variety of symptoms andsigns:

1. Subjective disturbances like insomnia are

FIG. 2. Case 6. Right internal carotid angiogram.The markedly enlarged lateral tentorial branch of theinternal carotidartery (long arrow) drains directly intothe right lateral sinus (large arrow).

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Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus

TABLE 8ARTERIAL SUPPLY TO ARTERIOVENOUS MALFORMATIONS

OF TRANSVERSE-SIGMOID SINUS (91 CASES)

Artery Total Only arterial supply

(No.) (%) (No.) (C4)

External carotid 81 89 24 26Internal carotid 47 51 2 2Vertebral 29 31 2 2

caused by the continuous head bruit and tinnitusdue to the arteriovenous shunt.

2. Cerebral hypoxia and ischaemia, alsosecondary to the AV shunt, are responsible forsymptoms and signs of neurological deficit.

3. Increased venous pressure at the torcularregion induces headaches and papilloedema.Infantile hydrocephalus with intracranial bruit isalso due to a tremendous increase of venouspressure as a consequence of the massive arterio-venous shunt at the torcular region.

4. Retrograde drainage through collateralcirculation sometimes produces engorgement ofother basal sinuses, thus secondarily causingdistant symptoms and signs.

5. Obstruction of some of the affected sinuses.6. Involvement of the pial venous system may

produce subarachnoid haemorrhages extendingsometimes into the cerebral tissue leading tohaematomas.The predominance or association of several

of these six main factors will no doubt providethe basis for the clinical syndrome in each par-ticular patient. As a result of the review of theliterature and our personal material we havearrived at the findings summarized in Tables 4and 5 with symptoms and signs listed accordingto their frequency. Correlating the clinical signswith the six main pathophysiological factors,several groups of clinical syndromes accordingto their main features and pathophysiologicalbasis have been outlined (Table 6).

It may be observed that about one-third of allpatients with AVM of the transverse-sigmoidsinus have only subjective symptoms due to thebruit (group I). However, the clinical syn-dromes more frequently observed in this con-dition correspond to groups II and III or acombination of both. Altogether they form 4000

of the whole material. Besides the symptoms andsigns secondary to increased venous pressureand cerebral ischaemia, in those cases there wasa cranial bruit that indicated an AVF or AVMin about half of them. Subarachnoid haemor-rhages represent 17% of these vascular mal-formations, being the first symptom in 13% ofthe patients (Table 4). Finally, a much less fre-quent syndrome is group IV with infantilehydrocephalus and cranial bruit. This waspresent in only 400 of all recorded patients.

Thus, there are various clinical modes ofpresentation of these dural arteriovenous mal-formations that may be confused with otherneurological disorders, especially when there isno cranial bruit as a leading symptom and sign.

There were no sex differences in these lesionsand the duration of the symptoms was very wideas well as the age of presentation, predominantlybetween 41 and 60 years (Table 7).

ANCILLARY EXAMINATIONS

Plain radiographic examination of the skull doesnot show important changes in most cases(Newton et al., 1968). However, Aminoff (1973)found significant abnormal signs in a greatnumber of his patients, such as prominentmeningeal vascular channels, enlarged foramenspinosum, bony lacunae, and erosions.

FIG. 3. Case 6. Right external carotid angiogram.Meningeal branches of lhe right occipital artery (longarrow) are enlarged and drain into the right lateralsinus (large arrow).

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TABLE 9MAIN ANGIOGRAPHIC FEATURES OF AVM

OF TRANSVERSE-SIGMOID SINUS

Cases with Personal Totaldetailed cases cases

angiographic (6) (52)explorationrecorded inliterature

(46)

Arterial supplyBranches of occipital artery 40 6 46Branches of middle meningeal

artery 26 3 29Tentorial branches of internal

carotid artery 28 3 31Other arteries 17 2 19

Primary venous drainageTransverse-sigmoid sinus 33 5 38Cortical veins 1 1Other veins or sinuses 7 7Not specified 6 6

Normal anterograde drainageJugular veins 5 2 7Deep cervical veins 3 3

Retrograde drainage 13 3 16

Sinus obstructionTransverse-sigmoid sinus 6 4 10Other sinuses 3 3

Radioisotopic scanning and measurement ofarterial-venous oxygen saturation have also beencarried out in some of these cases. Phono-cardiography may be helpful for the more pre-

cise localization of the cranial bruit which isusually more intense over the temporomastoidregion. But undoubtedly angiography with therecent techniques of selective arterial catheteriza-tion, substraction, and magnification representsthe only examination that may determine theextent and supply of these malformations with a

greater precision.With regard to the different branches of the

external and internal carotid arteries and of thevertebral arteries which may supply the arterio-venous fistula, the findings in our cases areshown in Table 2, and Table 8 reviews the 91cases reported in the literature, including also thepersonal material. Table 9 summarizes thearterial supply and venous drainage togetherwith the retrograde drainage and sinusesobstructions of 46 cases taken from the litera-ture with adequate angiographic data and thesix personal cases. In our material we have

TABLE 10AV MALFORMATIONS OF TRANSVERSE-SIGMOID SINUS

29 cases untreated Unchanged Death Spontaneous arrest Unknown

9 3 1 16(after angiography)

Cases surgically treated (55; 71 operations) Cured Improvement Unchanged Death Unknown

Ligature of vessels at neck (40)Unilateral external carotid 3 4 10 2 7Bilateral external carotid - 1 2 3 3Vertebral artery - - 4 - -

Cervical artery - - 1 -

Direct approach (27)Afferent arterial ligation and removal 5 4 - 2Dural incision and coagulation 6 2 - -

Transverse sinus ligature - 1 - - -

Unspecified - - 4 2 1

Combined extra-intracranial approach (4)Internal carotid 'trapping' - - 1 - -

With carotid ligation 1 1 - -I

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stressed the alterations in the venous drainage(retrograde drainage and obstruction of sinuses)that have not been fully considered in the litera-ture except by Dichgans et al. (1972).

Several examples of the angiographic featuresof cases 5 and 6 (Figs 1, 2, and 3) are presentedas the other four earlier cases have already beenreported (Fernandez Urdanibia et al., 1974).

TREATMENT

Most authors have observed that occlusion andligation of some of the afferent arteries may notbe sufficient to stop the circulation of thesearteriovenous malformations. The extent andmultiple afferent blood supply through variouschannels are adverse factors for the cure orimprovement of this condition. Some patientsnot treated or submitted only to ligature ofsome vessels in the neck may be improved ordo not show progressive changes in their evolu-tion (Table 10).The direct surgical approach with occlusion

or removal of these malformations has beenconsidered the best form of treatment where thisis possible (Laine et al., 1963; Pecker et al.,1965; Debrun and Chartres, 1972; Kosnik et al.,1974). These direct attempts at removal weremainly carried out in cases with a history of sub-arachnoid haemorrhage and especially withsigns indicating intracranial haematomas (ourcase 4).

Recently, Hugosson and Bergstrom (1974)have treated successfully some of these duralAVM by making a wide exposure of both theoccipital and cerebellar dura mater followed bythree long dural incisions along the occipitalregion to the middle cranial fossa, along thetentorium, and finally along the cerebellar duramater following the inferior border of the trans-verse and sigmoid sinuses. With this technique,coagulation and obliteration of all the vascularchannels and almost complete isolation of thesesinuses may be obtained. Table 10 demonstratesfrom the recorded experience that the directapproach is much more effective. The appro-priate type of treatment naturally depends onthe extent and symptomatology of the vascularlesion but the tendency nowadays is towards amore direct and radical treatment. Perhaps in thefuture, multiple embolization by selective cathe-

terization will provide the possibility for occlu-sion of these dural AVM. Embolization with amixture of wax and iophendylate has also beentried in some of these lesions (P. Albert Lasierra,personal communication).

REFERENCES

Amico, G., Macchi, L., and Parmeggiani, A. (1970). Mal-formazioni vascolari epicraniche drenantisi nei seni duraliposteriori. Minerva Neurochirurgica, 14, 324-329.

Aminoff, M. J. (1973). Vascular anomalies in the intra-cranial dura mater. Brain, 96, 601-612.

Aminoff, M. J., and Kendall, B. E. (1973). Asymptomaticdural vascular anomalies. British Journal of Radiology, 46,662-667.

Billewicz, O., Kamraj-Mazurkiewicz, K., and Pryczkowski,J. (1971). Case of congenital arteriovenous fistula fed bythe middle meningeal artery. Neuroradiology, 2, 234-236.

Ciminello, V. J., and Sachs, E., Jr (1962). Arteriovenous mal-formations of the posterior fossa. Journal of Neurosurgery,19, 602-604.

Debrun, G., and Chartres, A. (1972). Infra and supra-tentorial arteriovenous malformations. A general reviewabout 2 cases of spontaneous supratentorial arteriovenousmalformation of the dura. Neuroradiology, 3, 184-192.

Decker, K., and Backmund, H. (1970). Pddiatrische Neuro-radiologie, pp. 108-109. Thieme: Stuttgart.

Dichgans, J., Gottschaldt, M., and Voigt, K. (1972). Arterio-venose Dura-Angiome am Sinus transversus. KlinischeSymptome, charakteristische arterielle Versorgung undhaufige ven6se Abflusstorungen. Zentralblatt fur Neuro-chirurgie, 33, 1-18.

Epstein, B. S., and Platt, N. (1962). Visualization of anintracranial arteriovenous fistula during angiocardio-graphy in an infant with congestive heart failure. Radiology,79, 625-627.

Fadhli, H. A. (1969). Congenital arteriovenous fistulainvolving the occipital artery and lateral venous sinus. Casereport. Journal of Neurosurgery, 30, 299-300.

Fernandez Urdanibia, J., Silvela, J., and Soto, M. (1974).Occipital dural arteriovenous malformations. Neuro-radiology, 7, 57-64.

Fontaine, R., Dany, A., and Briot, B. (1957). Fistulesarterio-veineuses posttraumatiques de la region parieto-occipitale droite du crane et de la dure-mere sous-jacente.R6le probable des canaux derivatifs du type Sucquet.Neuro-Chirchurgie, 3, 30-39.

Houser, 0. W., Baker, H. L., Jr, Rhoton, A. L., Jr, andOkazaki, H. (1972). Intracranial dural arteriovenous mal-formations. Radiology, 105, 55-64.

Hugosson, R., and Bergstrom, K. (1974). Surgical treatmentof dural arteriovenous malformation in the region of thesigmoid sinus. Journal of Neurology, Neurosurgery, andPsychiatry, 37, 97-101.

Kosnik, E. J., Hunt, W. E., and Miller, C. A. (1974). Duralarteriovenous malformations. Journal of Neurosurgery, 40,322-329.

Kune, Z., and Bret, J. (1969). Congenital arterio-sinusalfistulae. Acta Neurochirurgica, 20, 85-103.

Laine, E., Galibert, P., Lopez, C., Delahousse, J., Delandt-sheer, J. M., and Christiaens, J. L. (1963). Anevrysmesarterio-veineux intra-duraux (developpes dans l'epaisseurde la dure-mere) de la fosse posterieure. Neurochirurgie, 9,147-158.

Legre, J., Boudouresques, J., Daniel, F., Giudicelli, G., and

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Dufour, M. (1969). Angiomatose meningee pure. Interet del'exploration angiographique selective. Neuro-Chirurgie,15, 487-495.

McCormick, W. F., and Boulter, T. R. (1966). Vascular mal-formations ('angiomas') of the dura mater. Journal ofNeurosurgery, 25, 309-311.

Newton, T. H., and Greitz, T. (1966). Arteriovenous com-munication between the occipital artery and transversesinus. Radiology, 87, 824-828.

Newton, T. H., Weidner, W., and Greitz, T. (1968). Duralarteriovenous malformation in the posterior fossa.Radiology, 90, 27-35.

Nicola, G. C., and Nizzoli, V. (1968). Dural arteriovenousmalformations of the posterior fossa. Journal ofNeurology,Neurosurgery, and Psychiatry, 31, 514-519.

Obrador, S., and Urquiza, P. (1951). Angioma arteriovenosode la tienda del cerebelo. Revista Espanola de Oto-Neuro-Ofjalmologia y Neurocirugia, 10, 387-392.

Obrador, S., and Urquiza, P. (1952). Angiome arterio-veineux de la tente du cervelet. Folia Psychiatrica,Neurologica et Neurochirurgica Neerlandica, 55, 385-387.

Pecker, J., Bonnal, J., and Javalet, A. (1965). Deux nouveauxcas d'an6vrysmes arterio-veineux intraduraux de la fossepost6rieure alimentes par la carotide externe. Neuro-Chirurgie, 11, 327-332.

Robinson, J. L., and Sedzimir, C. B. (1970). External carotid-transverse sinus fistula. Case report. Journal of Neuro-surgery, 33, 718-720.

Rottgen, P. (1937). Weitere Erfahrungen an kongenitalenarteriovenosen Aneurysmen des Schiidelinnern. Zentral-blatt fur Neurochirurgie, 2, 18-33.

Senarclens, B. de, Schar, J., and Wiuthrich, R. (1972). Von

der Arteria carotis externa ausgehende arteriovenosaMissbildungen der hinteren Schadelgrube. SchweizerArchiv fur Neurologie, Neurochirurgie und Psychiatrie, 110,69-82.

Storrs, D. G., and King, R. B. (1973). Management of extra-cranial congenital arteriovenous malformations of thehead and neck. Report of five cases. Journal of Neuro-surgery, 38, 584-590.

Takekawa, S. D., and Holman, C. B. (1965). Roentgenologicdiagnosis of anomalous communications between theexternal carotid artery and intracranial veins. AmericanJournal of Roentgenology, 95, 822-825.

Tonnis, W. (1936). In Gefassmissbildungen und Gefassge-schwulste des Gehirns. Edited by H. Bergstrand, H.Olivecrona, and W. Tonnis, Thieme: Leipzig.

Verbiest, H. (1951). L'an6vrysme arterioveineux intradural.Revue Neurologique, 85, 189-199.

Verbiest, H. (1962). Arterial and arteriovenous aneurysms ofthe posterior fossa. Psychiatria, Neurologia, Neurochirurgia,65, 329-369.

Verbiest, H. (1968a). Extracranial and cervical arteriovenousaneurysms of the carotid and vertebral arteries. JohnsHopkins Medical Journal, 122, 350-357.

Verbiest, H. (1968b). Arteriovenous aneurysms of theposterior fossa. Progress in Brain Research, 30, 383-396.

Werf, A. van der (1964). Sur un cas d'anevrisme arterio-veineux intradural bilateral de la fosse posterieure chez unenfant. Neurochirurgie, 10, 140-144.

Wijngaarden, G. K. van, and Vinken, P. J. (1966). A caseof intradural arteriovenous aneurysm of the posteriorfossa. Neurology (Minneap.), 16, 754-756.

ADDENDUMSince this paper was written two articles haveappeared: (1) Padalko, P. I., and Serbinenko, F. A.(1974. Neurosurgical pathology of the cerebralblood vessels. Moscow (in Russian)) present 32cases of AVM of the transverse-sigmoid sinusstudied at the Burdenko Institute; they emphasizethat these patients are often considered to be sufferingfrom brain tumours or vascular lesions. (2) Kosnik,E. J., Hunt, W. E., and Miller, C. A. (1974. Duralarteriovenous malformations. Journal of Neuro-surgery, 40, 322-329,) review this subject and reporttwo cases of transverse-sigmoid AVM.

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445Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus

APPENDIX TABLESUMMARY OF RECORDED CASES

Authors Se'x Age Histsry Main Clinical Angiography Treatment Evolution(yr) duration svmptoms signs

Afferent Venoussupply drainage

1 cases Cited by Verbiest (1951)

54 6 m Bruit, tinnitus Papilloedema, No visualized angiography Combined Died 11 d afterafter communica- tentorial operationhead ting hydro- and cere-

injury cephalus bellar AVA20 m 17 m Exophthalmos, Macrocephaly, Bilat. ext. Bilat. trans. Bilat. ext. Died after 6 m

vomiting, dilated carotid sinus carotidseizures scalp veins, arteries ligatureinstability, bruit, exo-bruit phthalmos

56 6 m Tinnitus, bruit Papilloedema, Ext. and int. Trans. sinus Jugular and Recoveredafter bruit carotid and jugular ext. carotid after 9 mhead arteries vein ligation andinjury division

58 3 h Headache, Meningeal Ext. carotid Galen's vein Suboccipital Recoveredsubarach- signs, and occipital and torcular craniotomy.noid dysphasia arteries Posteriorhaemor- meningealrhages artery

occlusionF 11 w 5 w Cyanosis, Bruit, heart Visualized in angiocardio-

bruit, failure graphydyspnoea

F 40 3 yr Bruit - Ext. carotid Transverse(occipital sinusartery)

M 23 2 yr Mental re- Hemiparesis, Vertebral Cavernoustardation. speech artery sinus3 recurrent disturbancesepisodes ofspeech dis-turbancesand hemi-paresis

M 33 2 yr 2 subarach- Meningeal Int. and ext. Torcular andnoid haemor- signs. III carotid and pterygoidrhages, paresis vertebral veinheadaches, arteryvertigo,visual failure

F 3 - Hydrocephalus Bruit, hydro- Bilat. int. and Transversecephalus, ext. carotid sinus andpapilloedema and vertebral torcular

arteries

- Died fromheart failure

Ext. carotid Bruit improve-and occi- mentpital arteriesligation

No findings in Unchangedposteriorfossa andfronto-temporalcraniotomy

Temporal Recovered aftercraniotomy 2 mand tentorialvesselcoagulation

Bilat. dural Bruit dis-incision appearedfrom tem-poral fossato free edgeof tentor-ium. Bilat.ext. carotidligation

F 56 15 yr Bruit, head- Bruit Ext. carotid Transverse Ext. carotid No changeaches, cer- artery sinus and ligation after 7 yrvical pain, jugular veinvertigo

M 36 Brief 3 subarachnoid Papilloedema Ext. carotid Anomalous Ext. carotid Recoveredperiod haemor- bilateral VI and vertebral vein crossing ligation and

rhages palsy. arteries cisterna suboccipitalEpisodes Meningeal magna craniotomy

syndrome with ligationof venous

drainageF 49 1 yr Headaches Bruit Ext. carotid Transverse Ext. carotid Recovered

and bruit artery sigmoid ligationsinus

M 28 5 m Proptosis Bruit, Ext. carotid Jugular vein Ext. carotid Recurrence ofocular pain proptosis artery ligation chemosis

after 2 m

M

M

F

M

Tonnis (1936)Rottgen (1937)Verbiest (195 1)

Obrador andUrquiza(1951, 1952)

Fontaine et al.(1957)

Ciminello andSachs (1962)

Epstein andPlatt (1962)

Verbiest (1962)

Laine et al.(1963)

van der Werf(1964)

Pecker et al.(1965)

Takekawa andHolman(1965)

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S. Obrador, M. Soto, and J. Silvela

APPENDIX TABLE-continued

Authors Sex Age History Main Clinical Angiography Treatment Evolution(yr) duration symptoms signs

Afferent Venoussupply drainage

van Wijngaarden F 65 5 m Bruit Bruit Ext. carotid Transverse Occipital Bruitartery liga- improvementtion and ext.carotid liga-tion 1 mlater

M 22 - Headaches Bruit, papil- Int. carotid, Galen's vein Bilat. ext.loedema bilat. ext. carotid liga-

carotid, tion andvertebral ventricularartery shunt

M - Macrocephaly Bruit, dilated Int. carotid Torcular Ventricularscalp veins and bilat. shunt and

ext. carotid ligation ofarteries bilat. ext.

carotid andtentorialarteries

M 33 5 yr Bruit, retro- Retroauricular Bilat. int. Transverse Ext. carotidafter auricular mass, bruit carotid, ext. sinus ligation andhead mass carotid and removal ofinjury vertebral extracranial

arteries mass

F 31 Brief Vomiting, gait Hemiparesis, Bilat. ext. Straight sinusperiod disturbance proptosis carotid, int. and dural

carotid and lakevertebralarteries

M 47 2 yr Exophthalmos, Exophthalmos, Int. and ext. Straight sinusseizures, hemianop- carotidarterial sia arterieshypertension

M 51 - Subarachnoid Meningeal Trigeminal Transverse Occipitalhaemorrhage signs artery, int. and superior angioma

and ext. sinus and haema-carotid toma re-

arteries movalM 50 - Hemiparesis VII palsy, and Int. carotid Straight sinus, Common caro-

dysaesthesia artery basal vein, tid ligationand Galen's and ligationvein of meningeal

branchesintracranially

0 A2 tuntiaext,TransverseF 43 - ruit, occipital isruit Jint. anu CAL. Lll4tl:vsial

pain carotid sinusarteries

F 29 Bruit Retroauricular Int. and ext. Transverse

mass. Bruit, carotid sinus anddilated scalp arteries superficialveins veins and

superiorlongitud.sinus

68 Visual failure, Bruit, papil- Int. carotid Transverse

ocular pain loedema, and bilat. sinusvisual ext. carotidfailure arteries

F 49 Visual failure Bruit, papil- Bilat. int. Transverse

bruit, arterial loedema, carotid. sinushypertension blindness in Ext. carotid

R eye artery

M 52 Transient Vertebral Superficialischaemic artery cerebellarepisodes vein

F 58 1 yr Bruit Bruit Ext. carotid Transverseartery sinus

M 52 3 yr Bruit Bruit, loss of Ext. carotid Transversehearing artery sinus

F 66 4 m Bruit, arterial Bruit, nystag- Ext. carotid Transversehypertension mus artery sinus

Occipitalartery liga-tion andoccipitalcraniotomy

446

and Vinken(1966)

Newton et al.(1968)

artery sinus

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Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus 447

APPENDIX TABLE-continued

Authors Sex Age History Main Clinical Angiography Treatment Etcolution(yr) duration symptoms signs

Afferent Venoussupply drainage

Nicola andNizzoli(1968)

F 43 - Subarachnoid Bruit, menin- Ext. carotid Transverse - -haemorrhage geal signs and vertebral sinus

arteriesM 61 2 m Headaches Papilloedema Ext. carotid Torcular, Bilat. occipital Died

artery straight and artery liga-transverse tion and ext.sinus carotid liga-

tionF 52 3 m Headaches, Papilloedema Int. and ext. Torcular and Occipital and Recovered

diplopia, carotid transverse posteriortinnitus arteries sinus fossa

craniotomy.Ligation offeedingarteries

F 60 4 yr Mental de- Mental dis- Int. and ext. Torcular, - Died fromterioration. turbances, carotid straight and myocardialspeech dis- dysphasia, arteries transverse infarctionturbances hemiparesis, sinusand hemi- ataxiaparesis

Fadhli (1969) F 47 months Bruit Bruit Ext. carotid Transverse Ext. carotid Recovered 2 yrartery sinus ligation later

Kune and Bret F 41 7 yr Bruit. Auricu- Bruit, exoph- Int. and ext. Straight sinus, Ext. carotid Improvement(1969) lar mass. thalmos carotid transverse and jugular of bruit,

Exophthal- arteries sinus, and ligation. 3 m exophthalmosmos, visual Galen's vein later verte-failure, head- bral arteryaches ligation. 6 yr

later cranio-tomy, tem-poral veinsligation, fewdays latertransversesinus liga-tion

F 47 - Bruit, head- Papilloedema Bilat. int. Transverse Ext. carotidaches, carotid and sinus and and retro- Bruit increaseddrowsiness vertebral torcular auricular

arteries. Ext. vessels liga-carotid tion. Jugularartery ligation. 6 yr

later trap-ping int.carotid. 2 yrlater occi-pital cranio-tomy, occlu-sion ofdilatedvessels andext. carotidligation. 6 mlater cervicalartery liga-tion. 10 dlater verte-bral and Died 6 d lateroccipitalarteries liga-tion. 3 yrlater muscu-lar vesselsocclusionand 3 yr latertransversesinus directapproachand packingdue tohaemorrhage

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S. Obrador, M. Soto, and J. Silvela

APPENDIX TABLE-continued

Authors Sex Age History Main Clinical Angiography Treatment Evolution(yr) duration symptoms signs

Afferent Venoussupply drainage

F 27 14 yr Seizures, Bruit Bilat. verte- Transverse Occipital and Bruit recur-headaches,bruit

bral artery. and sigmoid ext. carotid rence afterExt. carotid sinus ligation. 3 yr

3 m laterligation ofvertebralarterybranches.3 m latervertebralligation

M 46 8 yr Headaches, Bruit, facial Ext. carotid Transverse Ligation of Bruit improv-bruit hypaesthesia and verte- sinus and occipital and ment

bral arteries jugular vein branches ofvertebralartery

M 59 3 m Transitory Normalaphasia,headaches,subarach-noid hae-morrhages,bruit

F 60 1 yr Bruit Bruit

Ext. and int. Transversecarotid and sinus andvertebral corticalarteries drainage to

sup. longi-tud. sinus

Unchanged

Ext. carotid Transverse Occipital Bruit improve-artery sinus artery liga- ment

tion andexcision ofmalforma-tion

M 3 m Days Subarachnoid Meningeal Ext. carotid Jugular veinhaemorrhage syndrome artery

- Seizures, hemi- Bruit, hemi-paresis- paresissubcutan-eous angioma

3+ 2 m Drowsiness, Bruit, papil-headaches, loedemavomiting, hemiparesis,speech and cerebellargait disturb- syndromeance

Ext. carotid Transverse External caro- Diedand verte- sinus and tid resectionbral arteries venous lake 1 m later

suboccipitalcraniotomy.Later ven-tricularshunt

Ext. carotid Transverse Ext. carotidartery and sigmoid ligation

sinus.Retrogradedrainage tostraightsinus andsup. longitud.

M 6 m - Dilated scalp Bruit, psycho- Bilat. ext. and Bilat. trans- Bilat. ext.veins. Occi- motor re- int. carotid verse sinus carotidpital angi- tardation arteries, and sup. ligationoma, con- vertebral longitud.vulsive artery sinusseizures

F 47 - Subarachnoid Meningeal Int. carotid Bilat. trans- -

haemor- syndrome artery verse sinusrhages, and torcularheadaches

64 m Tinnitus, bruit Bruit Ext. carotid Transverse -

F 72 6 m Bruit, tinnitus Bruit

Partial im-provementafter 2+ yr

Bruit recur-rence 3 mlater

Unchanged

artery sinus, sig-moid sinusoccluded

Ext. and int. Cortical veinscarotid and super-arteries ficial petrous

sinus

448

19 mM

M

Legr6 et al.(1969)

Amico et al.(1970)

Decker andBackmund(1970)

Robinson andSedzimir(1970)

Billewicz et al.(1971)

Debrun andChartres(1972)

Dichgans et al.(1972)

F

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Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus 449

APPENDIX TABLE-continuedAuthors Sex Age History Main Clinical Angiography Treatment Evolution(yr) duration symptoms signs

Afferent Venoussupply drainage

M 41 Hours Subarachnoid Bruit, menin- Ext. and int. Transversehaemorrhage geal signs, carotid sinus and

coma arteries, corticalmiddle cere- veins to thebral artery superior

longitudinalsinus. Trans-verse sinusoccluded

F 55 2 m Headaches, Papilloedema Ext. and int. Sup. longit.tinnitus, carotid and trans-seizures arteries verse sinus

thrombosed.Retrogradecorticalvenousdrainage

M 55 2 yr Seizures, loss Bruit, papil- Ext. carotid Transverseof vision, loedema artery sinus andbruit, loss jugular vein.of hearing Retrograde

drainage tostraight andcavernoussinuses

- 70 1 m Subarachnoid Bruit, papil- Ext. and int. Transversehaemor- loedema, carotid sinus andrhage, left hemianop- arteries retrogradeparietal syn- sia, hemi- cortical veindrome, bruit paresis drainage.

Sigmoidsinusoccluded

Exophthalmosand seizures10 yr later

F: 9 20-29 (1) Bruit (10), Bruit (10), Occipital _ Surgical (5),M: 3 30-39(1) seizures (2), cerebellar artery (9), ext.40-49 (1) < 1 yr headaches syndrome middle men. carotid50-59(1) (7), diplopia (3), propto- artery (9), ligation (5),60-69(1) (1), propto- sis (1), meningeal direct ap-

70(1) sis (1), visual visual fail- hypophyseal proach (3)-iUnchanged (:failure (1), ure (1), artery (5), radio-subarach- ocular vertebral therapy (l)-i-Complicationoid hae- palsy (1), artery (2), (1)morrhages cerebral ascending Non-treated(3), intra- deficit (1), pharyngeal (7)cerebral pontocere- artery (2)hrge (1), bellarsubdural deficit (1)hrge (1)

M 20-29(1) Headaches (1), Bruit (2), - - Surgical (2), Recovered (M 30-39 (1) < 1 yr-2 seizures (1), proptosis, ext. carotid complica-M 40-49 (1)) ocular pain ch:mosis (1) ligation (1), tions (1)12 yr-i (1), propto- direct ap-sis and sub- proach (1),arachnoid untreated (1)haemorrhage(I)

M 52 - Tinnitus, Bruit Ext. carotid Sigmoid sinus - Unchangedbruit, head- artery and jugularaches vein

F 58 - Headaches, Bruit, menin- Bilat. ext. Torcular Bilat. occipital Died after 4bruit, 2 sub- geal signs carotid ext. carotid subarachnarachnoid arteries, artery liga- haemorrhahaemor- vertebral tionrhages artery

M 41 - Tinnitus, loss Drowsiness, Ext. and int. Transverse Ext. carotid Unchangedof hearing, papilloedema carotid sinus and ligation 2 m laterheadaches, hemianopia, arteries retrogrademental de- dysphasia drainage toterioration, straight sinusaphasia and cortical

veins, sig-moid sinusoccluded

:3)

ns

[1)

tyrDoidage

Houser et al.(1972)(12 occipitalcases)

(3 posteriorfossa cases)

Senarclens etal. (1972)

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S. Obrador, M. Soto, and J. Silvela

APPENDIX TABLE-continued

Authors Sex Age History Main Clinical Angiography Treatment Evolution(yr) duration symptoms signs

Afferent Venoussupply drainage

M 38 36 h Headaches, Bruit, hemi- Ext. and int. Transverse Excisionvisual fail- anopsia carotid sinus, sig-ure, sub- arteries moid sinus,arachnoid and superiorhaemorrhage longitudinal

sinusM 53 5 yr Headaches, Bruit, papil- Bilat. ext., int. Torcular Craniotor

visual fail- loedema, and verte- occlusicure, sub- blood in csf bral arteries feedingarachnoid vesselshaemor-rhages

F 43 1 yr Tinnitus, head- Bruit Ext. carotid Transverse -

aches artery sinusF 58 6 w Episode of Bruit Ext. carotid Transverse -

loss of con- artery and straightsciousness, sinusesvisual hal-lucinations

F 62 2 d Speech dis- Dysphasia, Ext. and int. Venous sinus Excisionturbances, bilateral carotid drainage tosubarach- Babinski arteries transversenoid haemor- signs, blood sinusrhage in csf

M 42 3-m Tinnitus Bruit Ext. and int. Sigmoid sinus -

Loss of vision2 yr later

my, Recovered afteronof 6m

Unchanged

Unchanged

Focal seizures10 y later

Unchangedcarotid 2 m laterarteries

M 54 12 yr Headaches, Bruit, exoph- Bilat. ext., Bilat. trans- Craniotomy Partial im-seizures, thalmos, int., and verse sinus and excision provementmental de- hemiparesis vertebral of feeding 3 yr laterterioration, ataxia, brain arteries vesselsvisual failure stem syn-

dromeM 48 2 yr Headaches, Bruit, optic

loss of atrophyvision (ven-tricularshunt due topapilloedema9 m earlier)

M 69 - - -

F 50 -

F 65 -

F 59 -

M 50 1 yr Tinnitus, Bruitheadaches,loss of vision

Ext. and int. Transversecarotid and sigmoidarteries sinus, tem-

poral super-ficial veinsand cervicalveins

Died, throm-bosed cere-bral veins

Ext. and int. Transverse - Died (cerebralcarotid sinus metastasisarteries from lung

cancer)Ext. and int. Sigmoid sinus Removal of Died (frontal

carotid frontal astrocytoma)arteries tumour

Ext. carotid Superficial - Died (tubercu-artery vein drain- lous menin-

age to gitis)superiorlongitudinal

Ext. and int. Transverse Removal of Improvingcarotid sinus frontal slowly (fron-arteries tumour, tal menin-

6 m later giema)occlusion ofmalforma-tion afferentveins, ven-tricularshunt

Ext. and int. Sigmoid sinus Embolization, Recoveredcarotid, and and jugular ext. caro- after 2 yrvertebral vein tid ligation,arteries excision of

malforma-tion

450

Aminoff, 1973

Aminoff andKendall

(Associatedwith otherintracranialpathology)

Storrs andKing (1973)

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Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus 451

APPENDIX TABLE-continued

Authors Sex Age History Main Clinical Angiography Treatment Evolution(yr) duration symptoms signs

Afferent Venoussupply drainage

M 36 6 m Tinnitus, Bruit Ext. and int. - - Spontaneousheadache carotid arrest after

vertebral angiography,arteries recovered

after 1 yrF 48 - Bruit Bruit Ext. carotid Transverse Dural section Recovered

artery and sigmoid above andsinuses below trans-

verse sinusand occlu-sion ofafferent veins

M 24 - Bruit Bruit Ext. and int. Transverse Dural section Improvementcarotid and and sigmoid above trans- of bruitvertebral sinuses verse sinus,arteries ligation of

branches ofext. carotidartery

M 52 - Bruit Bruit Ext. and int. Transverse Dural section Recoveredcarotid and and sigmoid above andvertebral sinuses below trans-arteries verse sinus

and occipitaldura mater

M 39 - Bruit Bruit Ext. and int. Transverse Dural scction Improvementcarotid and sigmoid above and of bruitarteries sinuses below trans-

verse sinus,occlusion ofsuperiorpetroussinus. Liga-tion ascend-ing pharyn-geal artery

M 71 - Bruit Bruit Ext. and int. Transverse Occipital Unchangedcarotid and and sigmoid artery liga-vertebral sinuses tionarterires

F 49 2 m Bruit Bruit Ext. carotid Sigmoid sinus Ext. carctid Recoveredand verte- and jugular artery re-bral arteries vein sected, 5 m

later verte-bral arteryligation, sub-occipitalcraniotomy,coagulationof duralveins

F 52 6 m Vomiting, Bruit, loss of Ext. and int. Torcular, Bilat. ext. Bruit dis-headaches, vision, papil- carotid and superior carotid liga- appearedsubarach- loedema, vertebral longitudinal tion, crani-noid haemor- ataxia, arteries sinus, corti- otomy withrhage, neuro- hemianopsia cal veins partiallogical de- coagulationterioration of the

angioma, 1 yrlater ligationof feedingarteries andtransversesinus liga-tion, 9 mlater ligationof longitud.,transverseand straightsinuses,excisionaround torcu-lar, tentor-ium and falx

Six personal cases, see Tables 1, 2, and 3.

Hugosson andBergstrom(1974)

Kosnik et al.(1974)

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