An adult case of congenital external carotid-jugular arteriovenous fistula with reversible circulatory insufficiency in the cerebellum and lower brain stem.

A 27-year-old man with congenital external carotid-jugular arteriovenous fistula presented with a diminished level of consciousness and an ataxic gait. Axial fluid-attenuated inversion-recovery (FLAIR) MR imaging revealed venous congestion, a dilated right jugular vein, and an area of high signal intensity in the brain stem and cerebellum. Angiography showed a dilated right external carotid artery and jugular vein and the presence of a fistula. After coil embolization of the fistula, axial MR FLAIR images showed only a few areas of high signal intensity in the brain stem and cerebellum. The causal factor was venous congestion in the inferior petrosal sinus and basilar plexus due to high blood pressure in the jugular vein. This case is presented for its unusual clinical and radiologic findings.

[1]  D. Mikulis,et al.  Venous congestion: an MR finding in dural arteriovenous malformations with cortical venous drainage. , 1994, AJNR. American journal of neuroradiology.

[2]  D. Herbreteau,et al.  Endovascular treatment of external carotid-jugular fistulae in the parotid region. , 1993, Neurosurgery.

[3]  T. Suzer,et al.  Congenital External Carotid-Jugular FistulaReport of Two Cases and a Review of the Literature , 1992 .

[4]  T. Yoshimoto,et al.  [Spontaneous arteriovenous fistula of the external carotid artery treated by a detachable balloon--a case report]. , 1987, No shinkei geka. Neurological surgery.

[5]  H. Vinters,et al.  A pathologic correlate of the 'steal' phenomenon in a patient with cerebral arteriovenous malformation. , 1986, Stroke.

[6]  E. Holman.  Reflections on arteriovenous fistulas. , 1971, The Annals of thoracic surgery.

[7]  J. Prévot,et al.  Congenital cervical jugulo-carotid fistula. , 1970, Journal of pediatric surgery.

[8]  C. Masse,et al.  [Congenital arteriovenous fistula between trunk of the external carotid and the external jugular vein (a case)]. , 1970, Journal de chirurgie.

[9]  N. Nomikos,et al.  Congenital arteriovenous aneurysm in the neck. A case report. , 1965, Journal of neurosurgery.

[10]  D. Szilagyi,et al.  PERIPHERAL CONGENITAL ARTERIOVENOUS FISTULAS. , 1965, Surgery.

[11]  M. Gage.  Congenital arteriovenous aneurysm of right external carotid artery and posterior facial vein with absence of branches of external carotid artery; new method of obliterating inaccessible venous sac and fistula. , 1951, Annals of surgery.

[12]  W. Rienhoff.  CONGENITAL ARTERIOVENOUS FISTULA: AN EMBRYOLOGIC STUDY, WITH REPORT OF CASE , 1924 .

[13]  W. Halsted.  Congenital Arterio-Venous and Lymphatico-Venous Fistulae. Unique Clinical and Experimental Observations. , 1919, Proceedings of the National Academy of Sciences of the United States of America.