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The role of sinus thrombosis in occipital dural arteriovenous malformations development and spontaneous closure* K. Kutluk' , M. Schumacher' , A. Mironoi/ University ofF re iburg, Section Neuroradiology, Freibur g Kantonspital Aara u. Section of Neu radiology, Aarau . Swltzerla nd

Summary Two cases of dural arterioven ous malformations are pr esent ed . Both were associated with thrombosis ofth e sigmoid sinus . Without anytreatment, recanalizati on of th e sinus occurred in Olle cas e, while the dural arterio venous malformatio n was persisting . In the second case, recana lization of the sinus coincided with the sp onta neous closure of th e malform ation . The interrelati onship of sinus thrombosis and dur al arteriovenous malformation s, and th e possible mechanism of spontaneous closure are discussed . Die Rolle der Sinus t hrombose bei okzipitalen dural en a rteriovenösen Fehlbildung en - En twic klung und spon taner Verschluß Zwei Fälle eine r duralen okzipitalen arte riovenösen Malform ation, beide mit Thrombose des Sinus sigrnoide us , werden vorgestellt. Das Zusammenspiel von spontaner Rekanali sation des Sinus bei fortbeste he nde r arte riovenöser Gefäß malformation im einen Fall und gleichzeitig eintretendem Versc hluß der Gefäßmalformation im zweiten Fall wird beschrieben. Die Zusammenhänge von Sinust hrombose und Entstehung duraler Gefäßmalforma tionen sowie die möglicher weise zugrunde liegend e Pathophysiologie von Spontanheilungen werden diskuti ert Key-Words Dura l art eriovenous malformation - Dural fistula - Spontaneous closure - Sinus thrombosis

Dural arterioven ous malformatio ns (AVM) are not as common as the intr acranial ones , with a Irequeney of 10 % to 15 % of all intracranial AVMs (1- 8). They take blood prim arily from the external earotid eireulation and only seeondarily from bran ehes of the internal earotid and vertebral system (9). The clinical pr esentations are usually headach e, bruits over the mas toid region, tinni tus and

Neurochir urgta 34 (1991) 144-1 47 Georg Thieme Verlag Stuttgart . New York

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less often increased intracran ial pressure and int racranial hem orrh age (10). They may be totally asymptoma tie (6). The treatm ent of choice for these fistulas is embolization by the arterial rout e, in exceptional cas es a tran sveno us approach may be used or operation is necessa ry (7. 9.1 9). Ther e are rep orts of spontane ous closu re or regre ssion of intraeranial AVMs (11-1 5). Spontaneous closur e of dural AVMs are , however , deseribed seldom (11 . 14. 16- 20). Thrombosis of dur al sinuses may be associated with dural AVMs. Thro mbosis ma y aiso be associated with sponta neous closure of dural AVMs. Such cases were reporte d with diiTerent suggestions abo ut the pathomeehani sm (2. 21). To add more inform ation concerning the discussion about the prima ry etiology of dural fistulas. we prese nt tWQcases with cont roversa l findin gs in the relationship between sinus thrombosis and development of dura l AVMs. Case Rep orts Case 1: A öz-year -cld wornan was ad mitte d to the hospital because of hea dache an d inter mittent noise in the left ear. Neurological examlnat ion was normal. Left ca rotid ang iogra phy revealed a dural occipital AVM fed by two branches oft he occipital a rtery, the posterior branch of middle meningea l a rtery and tontorial br an ch of the interna l carotid artery (Fig. 1). There was a n occlusion of thc sigmoid sinus with aretrog rade delayed flow into the confluent sinus. Five rnonths later without anv trea tment, second angicgra phy demonstrated that the thrornb osis of' the sigmoid sin us was recanalized although the dural fistula was persistin g with all branches (Fig. 2), Case 2: A S'l-year-old worna n was admltted to the hospital because of left sided headache and pulsatile beating in the left ea r, since ten years. Neurological exa mination was nor mal. Angiograph y revealed a left occipital dural AVM , supplied by bran ches of the occipital and tentoria l arteries. The main dr ainage was to transverse sinus and the sylvian veins. There was a n occlusion of the whcle sigmoid sinus (Fig. 3- 4). The patient refused to be c perated and was discharged. Though the headache cease d and the ear beating was decreased 12 months later . the patient decided to be treated by embolization. On the control angiograp hy before ernbellzaticn, the spontaneous closure of the malformati on was noticed (Fig. 5). The enla rged tentorial and occipita l a rter ies see n on the previous angiograp hy, were no more visualized. The left verte bral, intern al caroüd. externa l carotid and the right car otid a ngiogra ms revealed normal findings. In the venous phase. a reca nalizatio n of the sigmoid sinus and a para cavern ous drain age syste m as a var iatlon was see n.

* Dedlcated to Prof. Or. W. Wenz at his 65t h birthday.

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The role ojsinus thrombos is in occip ital dural arteriovenous malformations

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Fig.l l eft carctid anglegram(lateralview) revealsaduralAVM fed byseverat branchesof thecccipltal,middlemeningealandtentorialarteries end the occlusion of thesigmoidsinus

Iliscussion

There is a close relationship of dural AVMs to thrombosis of a dural sinus. Some authors suggest that occlusion of a sinus is the cause of development of a dural hlood sup ply during the process of organisation. with subsequent formation ofan AVM to the patent portion ofth e sinus (1.2 . 4.1 0.2 1). May berg and Zimmerman (21) re ported a case of Galen aneurysm associa ted with a dural AVM and straight sinus thrombos is, and the sinus thrombosis was suggested as the primary event in the development of the other lesions. The authors thought that first the sinus was occluded, an d subsequently. organ isation of the straig ht sinus thrombosis produced dilatation of dural arterioles, capillaries, and the development of arteriovenous fistula.

On the other hand, the mechanism of the spontaneous closure of dural AVMs is not certain. In the cases with subarachnoid hemorrhage, hematoma formation, edema, or associated spasm were suggested to promote thro mbosis of the AVM (17), Magidson and Weinberg (17) pr esented a case with dur al AVM oft he right transverse sinus, in which the second angiography demonstrated no AVM. two years later. The enlarged feeding ar teries on the previous angiography were of normal size on the seco nd

Fig.2 Controlangiography halfa year Jaterwith recanalizatton of the sigmoid sinus. The dural malformation ls still persistmg

one and the transverse sinus was occluded. Spontaneous sinus thrombosis involving the ar ea of the AVM was suggested by the authors as a cause ofthe disapp ear ance ofthe AVM . lncrease d venous pressure in the sinuses, seco ndarily increased intracranial pressure and the venous stasis are thought to be the reasons of sinus thrombosis in such cases (6. 22).

In the case reported by Endo et al. (18). dural AVM of the left transverse and sigmoid slnuses . regressed within 25 months without bleeding episodes or surgical intervention and the patient's symptoms disappeared completely over the same period. Olutola et al. (20) presented a case of suprate ntorial dura lAVM associate d with intrace rebral hemorrhage . Four months later , repeat angiogra phy revealed no vascular malformation. The authors believe that spontanoeus regression was due to the thrombosis ofthe actual AVM , not of the draining venous sinus, as there was no evidence of sinus thrombosis. Subarachnoid hemorrhage, edema, mass effect, parenchymal hemorrhage and vascular spasms might be res ponsible for th rombosis. The interrelationship of sinus thrombosis and dur al AVM. and also spontaneous closure ofAVM is not clear . In our first patient, the nat ure of the dural AVM

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K. Kutluk. M. S chumccher. A. Miro110V Fig. 3 l eft common carotid anglegram (lateral view) demonstrates thetentorial and occipital feeders to the duralAVM, arterializing thetrensverse sinus. Reversed venous drainage intothesylvian groupand occlusion ofthe whole sigmoid sinus can bevisualized

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Fig.4 l eft external carotid angiogram demonstrates thepoint offistula (errow) and thrombosis of sigmoid sinus (lateral view [al. paview Ib]l

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cha nge d neither in occlusion, nor in recanalization stage s of the sigmoid sinus. In the sec ond case , it is rnor e-difficult to make a clear expla na tion for the spontane ous closure ofthe dural AVM, since it occurred during the recanalizatio n phase of the thro mbosis of sigmoid sinus . We th ink that the course of dural sinus thrombosis does not always playa definit e role in sponta ne ous disa ppea ra nce of dural AVMs. However , a complete obliteration of a sinus ma y lead to an ocelusion of the fistula as we could observe in three pati ents : The occipita l du ral AVMs disappeared after operative occlusion oft he who le sigrnoid sin us. The controversal findings in

our cases as weil as in the literature conce rn ing development , regression or recurre nce of occipital dural AVMs may allow some assessme nts: 1. Parti al thrombosis of a sinus see ms to be essentia l for th e primary developm ent of a fistula. 2. Parti al sinus thrombosis is not indispe nsa ble to keep a fistula pat ent. Recanali zation ofth e sinus may or may not induce closure of a fistu la . 3. Complete thrombosis ofthe sinus norm allyi s followed by closure of the fistula as see n in operati ve tr eatment.

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The rcle 0/ sinu s thro mbosis in occipilal dural arteriooenous matformations

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c Refercn ces Aminoff. M . J. : Vascular anomallos in the intrac ra nial dura mater . Brain 96 (1973)6 01-602 2 A minoff. M. J. . B, H. Kedall : Asymptomatic du ral vascular anoma lies. Br. J. Rad iol. 46 (1973) 662- 667 3 Bit oh. S .. A'. Arita. AI. Fujuoara. K. Gzaki. Y. l\'aka o: Dural arter tovenous malformat ion near the left sphene parietal slnus. Surg. Neurol. 130 9801345- 349 -I Conf orti. P.: Sponta neous disappea rance of cerebral arte rioven ous angioma. J. Neurosu rg. 34 (1971) 432-4 34 5 Dichga ns. V. J.. M. Gottscha ld t. K. Voigt: Arte riovenöse Duraangiome am Sinus transversus. Klinische Symptome. chara kteristische arterielle Vers orgun g und häu fige venöse Abflußstörun gen. Zentra lbl. Neurochtr. 33 (1972) 1- 18 b Eisenma n. J. I.• A. Alekoumbides. H, Pribram: Spontaneous thrombosis of vascula r malform ations ofthe brain . Acta Hadlol. Diagn . 13 (972) 77-85 7 Endo. 5.. K. Koshu. J. Suzuki: Spona neous regres ston of posterlor fossa dural arteriove nous malfonn ation. J. Neuros urg. 51 (979) 715-71 7 8 Graeb . D. A. C. L. Dolman: Ragiologieal and patho logical aspects of dura l arteriovenous listulas. Case report. J . Neurosu rg. 64 (986) 962-967 s Halbach. v. R. T. IIigas hida. G. B. Hiesh ima. C..\1. Sehringe r. C.1\: Ilar din: Transve nous ernbolizauon of dura l distulas involving the transv erse and sigmoid sinuses . AJl\'R 10 (1989 )385-392 10 Hauser. O. W. 11. L. Baker j r.. A. L. Rhot on jr.: Intracranial dura l arte riovenous malformations. Hadiology 105 (1972) 55-64 11 Kos nik ; E. J. . lV. E. /I un t. C. A. Mil ter: Dura l arte novenous ma Iformations. L x eurosurg. 40 (1974) 322-329 12 Kus hner. J.. J. E. Alexander: Partial spontaneous regressive arteriovenous ma lfor mation. J. l'-:eurosurg. 32 (19701360- 366 13 l.ak ke. J. P. IV. F: Regression of an art eri ovenous ma lforma tion of the brain. J . Neurol. Sei. 11 (1970)489- 496 H Lam as, E.. R. D. Loba to, J. Espa rz a. L. Es cud ero: Dural posterior fossa AVM produ cing ralsed sagittal sinus pressure. Case report . J . Neurosurg. 46 (1977) 804- 808 15 Lasj au n ias, P.. M. Chin. K. Ter Brugge : Neurological manifestations 1

of intra cranial dural arte riovenous malfurm at tcns. J . Neurosurg. 64 119861724- 730 16 Leoine . J. , J. C. Mis ka. 1. L. S eres. U. G. S nodgrass: Spontaneo us angiogra phic disap pearan ce ofa cerebra! arter lovenous malforme tion. Arch. Neurol. 28 (197 3) 195-1 96 17 Magidson. At. A. . P. E. Weinberg: Sponta ncous closure of a dur al arteriovenous malformation. Surg. Xeurol. 8 (197 6) 107- 110 18 Mayberg. M. R.. C. Zi mmerman. Vein ofGale n aneurysm assoclated with dur al AV).! and stra ight sinus thrombosis. J . Neurosurg. 68 (l988) 288- 291 1'1 Neuno n. T. H.• S. Cronooist. Involvement of dural arten es in intra crania l arte riovenous malform ations. Hadiology 93 (1969 ) 1071-1078 20 Netcton. T. II.. W F. Hogt: Dural arte riovenous shu nts in the region ofthe cavemous sinus . Neuroradiology 1 (1970) 71- 81 2 1 Olutola. P. S.. .\1. Eüam. ,\ 1.s tolot. A. Talalla : Spoma neous regression of a du ra l arteriovenous malformation. Xeurosu rgery 12 (l983) 687-690 22 O'Reilly . G. v.. S , B. Ham merschlag. ,\ 1.Ront h al: Aneu rysmal dilatation ofthe Galenic venous syste m ca used by a dura l arte rtovenous malformation. J. Ccmput . Assist. Tomogr. 51198 1)899-903 23 Picard. L.. J. Roland. S. Bracar d. J. Lep oire. J . Mon to nt: Spontaneous dural distulas. Classification - Diagnosis - Endovascular treatment. In: Auer. L. W.. F. Loew Ieds.). The cereb ral veins. Springe r. wien-New York. 1983 H Sundt, Tn. M.. D. G. Piepgras: The surgical a pproach to arterlovenous malformations of the latera l and sigmoid du ral sinuses. J. :\eu ros urg.59{1983)32-39 2.5 vinueta. F,. A. J. Fox. D. M. Pelz. C. G. Drake : Unusua l c1inical ma nifestations ofdu ral arte riovenous malformations. J , Neurosurg. 64 {l9861554 - 558

Prof Dr. AI. Schumache r Untversuy of Fre iburg Sectio n of Neuro radiology Hauptstraße 5 7800 Freiburg

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Fig.5 An~io~rams of theleft intemal (al, estemal caroto (bi, end occipital (c) artenesdemcnstratethe occlusion of thefistula. Recanalization of tnerett sigmoid smusrs seen