Publish in this journal
Journal Information
Vol. 15. Num. 5.January 2004Pages 433-489
Share
Share
Download PDF
More article options
Vol. 15. Num. 5.January 2004Pages 433-489
DOI: 10.1016/S1130-1473(04)70458-3
Unruptured dural arteriovenous malformation of the transverse-sigmoid sinus presenting with focal symptoms and coma
Malformación arteriovenosa dural del seno transverso-sigmoide no sangrante manifestada por síntomas focales y coma
Visits
1970
J.C. Palomino
, R.D. Lobato, J. Campollo, J. Fernández-Alen, A. Lagares
Service of Neurosurgery and Section of Neuroradiology. Hospital “12 de Octubre”. Faculty of Medicine. University Complutense. Madrid
Visits
1970
Article information
Abstract
Bibliography
Download PDF
Statistics
Summary

We are presenting the case of a 63 year-old man with a dural arteriovenous malformation of the transverse sigmoid sinus who developed focal deficits followed by less localized symptoms such a disorientation, lethargy and eventually comatose status. Initial cerebral angiography showed retrograde filling of the cortical and deep cerebral venous system with marked delay in venous empting. Following embolization clinical symptoms completely cleared at the time that control angiography showed retrograde venous flow turning anterograde. Patient's symptoms recurred four months later when there was a relapse of retrograde cerebral venous drainage at the time he developed thrombosis of the superior longitudinal and right transverse sinuses. Sinus thrombosis and thrombosis of the central retinal artery were coincidental with hypercoagulability rela-ted to hyperhomocysteinemia. Since control angiography still showed persistence of the AV shunting radical excision of the involved dural sinuses was performed. The final outcome was excellent.

The physiopathological mechanism responsible for neurological deficits in our patient most likely was ischemia of venous origin secondary to venous hyper-tension resulting from retrograde cerebral venous drainage. The clinical and angiographic presenta-tion in few similar cases reported in the literature is reviewed.

Key words:
Arteriovenous malformation
Dural arteriovenous malformation
Venous sinuses
Ischemia
Intra-cranial pressure
Venous thrombosis
Coma
Endovascular embolization
Hyperhomocysteinemia
Abreviaturas:
AVM
CBF
CBV
DAVM
HC
HHCs
MTHFR
SLS
Resumen

Se presenta el caso de un hombre de 63 años con una malformación arteriovenosa dural de la región del seno transverso-sigmoide que debutó con síntomas focales para desarrollar después déficit de funciones superiores y finalmente estupor y coma. La angiografía cerebral inicial mostró drenaje venoso retrogrado hacia venas cerebrales corticales y profundas con un marcado retardo del tiempo angiográfico. Tras la práctica de embolización transarterial los síntomas revirtieron por completo, al tiempo que la angiografia de control mostró una normalización del drenaje venoso que se hizo anterógrado. Cuatro meses después los síntomas recurrieron coincidiendo con la reaparición de drenaje venoso cerebral retrógrado cuando el paciente presentó trombosis del seno longitudinal superior, del seno transverso-sigmoide derecho y de la arteria central de la retina en el contexto de hipercoagulabilidad secundaria a hiperhomocisteinemia. La angiografía cerebral de control mostró persistencia de fístula AV y drenaje venoso retrógrado por lo que se procedió a resecar las estructuras durales implicadas, logrando la curación definitiva.

El mecanismo fisiopatológico responsable de los síntomas en nuestro paciente fue probablemente isquémico de origen venoso secundaria a la hipertensión del sector venoso causada por la fistula AV dural. Se revisa la presentación clínica y angiográfica en casos similares reportados en la literatura.

Palabras clave:
Malformación arteriovenosa
Fístula dural arteriovenosa
Seno venoso
Isquemia
Presión intracraneal
Trombosis
Coma
Embolización
Cirugía
Hiperhomocisteinemia
El Texto completo solo esta disponible en PDF
References
[1.]
L.A. Awad,J.R. Little,W.P. Akarawi,J. Ahí
Intracranial dural arteriovenous malformations: factors predisposing to an aggressive neurological course
J. Neurosurg., 72 (1990), pp. 839-850 http://dx.doi.org/10.3171/jns.1990.72.6.0839
[2.]
S.L. Barnwell,V.V. Halbach,R.T. Higashida,G. Hieshima,C.B. Wilson
Complex dural arteriovenous fistulas. Results of combined endovascular and neurosurgical treat ment in 16 patients
J. Neurosurg., 71 (1989), pp. 352-358 http://dx.doi.org/10.3171/jns.1989.71.3.0352
[3.]
D.L. Barrow
Unruprured cerebral arteriovenous malformations presenting with intracranial hypertension
Neurosurgery, 23 (1988), pp. 484-490
[4.]
W.vd. Berg,E.D. Verbraak,P.I. Bos
Homocystinuria presenting as central retinal artery occlusion and longstanding thromboembolic disease
Br J Ophthalmol, 74 (1990), pp. 696-697
[5.]
V. Biousse,A. Ameri,M.G. Bousser
Isolated intracra nial hypertension as the only sign of cerebral venous thrombosis
Neurology, 53 (1999), pp. 1537-1542
[6.]
V. Biousse,N.J. Newman,R. Sternberg Jr.
Retinal vein occlusion and transient monocular visual loss associated with hyperhomocystinemia
Am. J. Ophthalmol., 124 (1997), pp. 257-260
[7.]
G.H. Boers
Carriership for homocystinuria in juvenile vascular disease
Haemostasis, 19 (1989), pp. 29-34
[8.]
G.H. Boers,A.G. Smals,Trijbels
Heterozygosity for homocystinuria in premature peripheral and cerebral occlusive arterial disease
N. Engl. J. Med., 313 (1985), pp. 709-715 http://dx.doi.org/10.1056/NEJM198509193131201
[9.]
J.A. Borden,J.K. Wu,W.A. Shucart
A proposed classification for spinal and cranial dural arteriovenous fistulous malformations and implications for treatment
J. Neurosurg., 82 (1995), pp. 166-179 http://dx.doi.org/10.3171/jns.1995.82.2.0166
[10.]
M.L. Chimowitz,J.R. Little,L.A. Awad,C.A. Sila,G. Kosmorsky,A.J. Furlan
Intracranial hypertension associated with unruptured cerebral arteriovenous malformations
Ann. Neurol., 27 (1990), pp. 474-479 http://dx.doi.org/10.1002/ana.410270504
[11.]
C. Cognard,Y.P. Gobin,L. Pierot
Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage
[12.]
M. Collice,G. D'Aliberti,O. Arena,C. Solaini,R.A. Fontana,G. Talamonti
Surgical treatment of intracranial dural arteriovenous fistulae: role of venous drainage
Neurosurgery, 47 (2000), pp. 56-66
[13.]
J.R. Cruysberg,G.H. Boers,J.M. Trijbels,A.F. Deutman
Delay in diagnosis of homocystinuria: retrospective study of consecutive patients
BMJ, 313 (1996), pp. 1037-1040
[14.]
J.L. Cummings
Vascular subcortical dementias: clinical aspects
Dementia, 5 (1994), pp. 177-180
[15.]
A. D'Angelo,G. Mazzola,L. Crippa,I. Ferino,D.S. Vigano
Hyperhomocysteinemia and venous thromboembolic disease
Haematologica, 82 (1997), pp. 211-219
[16.]
C.A. David,S.J. Peerless
Pseudotumor syndrome resulting from a cerebral arteriovenous malformation: case report
Neurosurgery, 36 (1995), pp. 588-590
[17.]
U. DeGirolami,M.L. Haas,E.P. Richardson Jr.
Subacute diencephalic angioencephalopathy. A clinicopathological case study
J. Neurol. Sci., 22 (1974), pp. 197-210
[18.]
A. Esack,G. Thompson,H. Burmester
Benign intracranial hypertension and essential thrombocythaemia
J. Neurol. Neurosurg. Psychiatry, 52 (1989), pp. 914
[19.]
M.J. Gelwan,I.S. Choi,A. Berenstein,J.M. Pile-Spellman,M.J. Kupersmith
Dural arteriovenous malformations and papilledema
Neurosurgery, 22 (1988), pp. 1079-1084
[20.]
Y.P. Gobin,E. Houdart,A. Rogopoulos,A. Casasco,A.L. Bailly,J.J. Merland
Percutaneous transvenous embolization through the thrombosed sinus in transverse sinus dural fistula
AJNR Am. J. Neuroradiol., 14 (1993), pp. 1102-1105
[21.]
A. Guberman,D. Stuss
The syndrome of bilateral paramedian thalamic infarction
Neurology, 33 (1983), pp. 540-546
[22.]
J. Handa,S. Yoneda,H. Handa
Venous sinus occlusion with a dural arteriovenous malformation of the posterior fossa
Surg. Neurol., 4 (1975), pp. 433-437
[23.]
K. Hinokuma,E. Ohama,F. Ikuta,K. Tanimura,S. Kameyama
Dural arteriovenous malformation with abnormal parenchymal vessels: an autopsy study
Acta Neuropathol. (Berl), 80 (1990), pp. 656-659
[24.]
O.W. Houser,J.K. Campbell,R.J. Campbell,T.M. Sundt Jr.
Arteriovenous malformation affecting the transverse dural venous sinus an acquired lesion
Mayo Clin. Proc., 54 (1979), pp. 651-661
[25.]
R.W. Hurst,D.B. Hackney,H.I. Goldberg,R.A. Davis
Reversible arteriovenous malformation-induced venous hypertension as a cause of neurological deficits
Neurosurgery, 30 (1992), pp. 422-425
[26.]
M. Ito,T. Sonokawa,H. Mishina,K. Sato
Reversible dural arteriovenous malformation-induced venous ischemia as a cause of dementia: treatment by surgical occlusion of draining dural sinus: case report
Neurosurgery, 37 (1995), pp. 1187-1191
[27.]
T. Iwama,N. Hashimoto,Y. Takagi
Hemodynamic and metabolic disturbances in patients with intracranial dural arteriovenous fistulas: positron emission tomography evaluation before and after treatment
J. Neurosurg., 86 (1997), pp. 806-811 http://dx.doi.org/10.3171/jns.1997.86.5.0806
[28.]
H.S. Kang,D.G. Kim,B.W. Yoon
Superior sagittal sinus thrombosis with homocystinuria and deficiency of antithrombin III and factor VII: case report
Acta Neurochir. (Wien), 140 (1998), pp. 196-198
[29.]
T. Kawaguchi,S. Fujita,H. Yamada,Y. Nishida,E. Mori
Hemodynamics before and after the total removal of a dural arteriovenous malformation of the posterior fossa. Case report
Surg. Neurol., 30 (1988), pp. 457-461
[30.]
H. Kinney,P.C. Burger,F.S. Vogel
Subacute diencephalic angioencephalopathy report of an additional case
J. Neurol. Sci., 45 (1980), pp. 73-81
[31.]
E.J. Kosnik,W.E. Hunt,C.A. Miller
Dural arteriove nous malformations
J. Neurosurg., 40 (1974), pp. 322-329 http://dx.doi.org/10.3171/jns.1974.40.3.0322
[32.]
A. Kurata,Y. Miyasaka,T. Yoshida,M. Kunii,K. Yada,S. Kan
Venous ischemia caused by dural arteriovenous malformation. Case report
J. Neurosurg., 80 (1994), pp. 552-555 http://dx.doi.org/10.3171/jns.1994.80.3.0552
[33.]
A.K. Lalwani,C.F. Dowd,V.V. Halbach
Grading venous restrictive disease in patients with dural arteriovenous fistulas of the transverse/sigmoid sinus
J. Neurosurg., 79 (1993), pp. 11-15 http://dx.doi.org/10.3171/jns.1993.79.1.0011
[34.]
E. Lamas,R.D. Lobato,J. Esparza,L. Escudero
Dural posterior fossa AVM producing raised sagittal simus pressure. Case report
J. Neurosurg., 46 (1977), pp. 804-810 http://dx.doi.org/10.3171/jns.1977.46.6.0804
[35.]
P. Lasjaunias,M. Chiu,K. ter Brugge,A. Tolia,M. Hurth,M. Bernstein
Neurological manifestations of intracranial dural arteriovenous malformations
J. Neurosurg., 64 (1986), pp. 724-730 http://dx.doi.org/10.3171/jns.1986.64.5.0724
[36.]
R. Leblanc,J. Little
Hemodynamics of Arteriovenous Malformations
Clin Neurosurg, 36 (1989), pp. 299-317
[37.]
Legnani, C, Palareti, G., Grauso, E., et al: Hyperhomocyst(e)inemia and a common methylenete-trahydrofolate reducíase mutation (Ala223 Val MIHER) in patients with inherited thrombophilic coagulation defects. A 1997; 17: 2924–2929.
[38.]
S.R. Lentz
Mechanisms of thrombosis in hyperhomocysteinemia
Curr. Opin. Hematol., 5 (1998), pp. 343-349
[39.]
R.D. Lobato,P.A. Gómez,R. Alday
Malformaciones arteriovenosas tentoriales. Presentación de tres casos y revisión de la literatura
Neurocirugía, 7 (1996), pp. 208-218
[40.]
C.P. Lucas,J.M. Zabramski,R.F. Spetzler,R. Jacobowitz
Treatment for intracranial dural arteriovenous malformations: a meta-analysis from the English language literature
Neurosurgery, 40 (1997), pp. 1119-1130
[41.]
M. Makris
Hyperhomocysteinemia and thrombosis
Clin. Lab Haematol., 22 (2000), pp. 133-143
[42.]
G.M. Malik,J.E. Pearce,J.L. Ausman,B. Mehta
Dural arteriovenous malformations and intracranial hemorrhage
Neurosurgery, 15 (1984), pp. 332-339
[43.]
E.W. Molicka,H. Van Slooten,L.A Van der,M.S. Suttorp-Schulten
Delayed diagnosis of homocystinuria as a cause of vascular retinal occlusion in young adults
Br. J. Ophthalmol., 83 (1999), pp. 1310-1311
[44.]
S. Mullan
Reflections upon the nature and management of intracranial and intraspinal vascular malformations and fistulae
L Neurosurg., 80 (1994), pp. 606-616
[45.]
K.J. Murphy,P. Gailloud,A. Venbrux,H. Deramond,D. Hanley,D. Rigamonti
Endovascular treatment of a grade IV transverse sinus dural arteriovenous fistula by sinus recanalization, angioplasty, and stent placement: technical case report
Neurosurgery, 46 (2000), pp. 497-500
[46.]
T. Nakada,I.L. Kwee,W.G. Ellis,J.N. St John
Subacute diencephalic necrosis and dural arteriovenous malformation
Neurosurgery, 17 (1985), pp. 653-656
[47.]
S. Obrador,M. Soto,J. Silvela
Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus
J. Neurol. Neurosurg. Psychiatry, 38 (1975), pp. 436-451
[48.]
L. Pierot,J. Chiras,C. Duyckaerts,M. Jason,N. Martin
Intracranial dural arteriovenous fistulas and sinus thrombosis. Report of five cases
J. Neuroradiol., 20 (1993), pp. 9-18
[49.]
R. Rozen
Genetic predisposition to hyperhomocys teinemia: deficiency of methylenetetrahydrofolate reducíase (MTHFR)
Thromb. Haemost., 78 (1997), pp. 523-526
[50.]
T. Sakaki,T. Morimoto,H. Nakase,T. Kakizaki,K. Nagata
Dural arteriovenous fistula of the posterior fossa developing after surgical occlusion of the sigmoid sinus. Report of five cases
J Neurosurg., 84 (1996), pp. 113-118 http://dx.doi.org/10.3171/jns.1996.84.1.0113
[51.]
A. Tawakol,T. Omland,M. Gerhard,J.T. Wu,M.A. Creager
Hyperhomocyst(e)inemia is associated with impaired endothelium-dependent vasodilation in humans
Circulation, 95 (1997), pp. 1119-1121
[52.]
J. Vassilouthis
Cerebral arteriovenous malformation with intracranial hypertension
Surg. Neurol., 11 (1979), pp. 402404
[53.]
F. Viñuela,A.J. Fox,D.M. Pelz,C.G. Drake
Unusual clinical manifestations of dural larteriovenous malformations
J. Neurosurg., 64 (1986), pp. 554-558 http://dx.doi.org/10.3171/jns.1986.64.4.0554
[54.]
E.M. Wenzler,A.J. Rademakers,G.H. Boers,J.R. Cruysberg,C.A. Webers,A.E. Deutman
Hyperhomo cysteinemia in retinal artery and retinal vein occlusion
Am J Ophthalmol, 115 (1993), pp. 162-167
[55.]
C.J. Wrobel,E.H. Oldfield,G. Di Chiro,E.C. Tarlov,R.A. Baker,J.L. Doppman
Myelopathy due to intracra nial dural arteriovenous fistulas draining intrathecally into spinal medullary veins
Report of three cases. J. Neurosurg., 69 (1988), pp. 934-939
[56.]
J.M. Zabramski,R.F. Spetzler
AVMs and Intracranial Hypertension (Comments)
Neurosurgery, 23 (1988), pp. 489

Palomino. J.C.; Lobato, R.D.; Campollo, J.; Fernández-Alen, J.; Lagares, A.: Unruptured dural arteriovenous malformation of the transversesigmoid sinus presenting with focal symptoms and coma. Neurocirugía 2004; 15: 458–467.

Copyright © 2004. Sociedad Española de Neurocirugía
Neurocirugía (English edition)

Subscribe to our Newsletter

Article options
Tools
Cookies policy
To improve our services and products, we use cookies (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here.