A congenital external carotid artery-external jugular
vein arteriovenous fistula was successfully treated by coil
embolization (case report and literature review)
Received: 15 December 2016 /Accepted: 8 June 2017 /Published online: 29 June 2017
Springer-Verlag GmbH Germany 2017
Object Congenital arteriovenous fistula involving the external
carotid system is rare.
Methods This paper reports a case of congenital external
carotid artery-external jugular vein arteriovenous fistula
admitted to Xuanwu Hospital, and reviews the
Results The patient was a boy, 9 years old, with a his-
tory of pulsatile mass and thrill in the right neck since
his birth. External carotid artery-external jugular vein
fistula was confirmed by the digital subtraction angio-
graphy. And coil embolization was done later.
Postoperative immediate angiography confirmed the
complete occlusion of the fistula, and partial branch of
the external carotid artery can be seen. The abnormal
clinical manifestation disappeared after the procedure
without any complications.
Conclusion This case and relevant literatures remind us
that congenital external carotid artery-external jugular
vein arteriovenous fistula has its unique features, and
it can be treated by coil embolization safely and
Keywords External carotid artery
External jugular vein
Arteriovenous fistulas (AVFs) in the head and neck region are
rare. The majority of the AVFs are of traumatic origin, or
secondary to intragenic causes . And the relative reports
of congenital AVFs involving the external carotid system are
much less . We reported a case of congenital external ca-
rotid artery-external jugular vein arteriovenous fistula (ECA-
EJV AVF) and reviewed the literature in this paper.
A boy was found with the pulsatile mass and thrill around the
right mandibular angle 1 month after birth. The patient did not
suffered from headache, dizziness, tinnitus, or hearing loss in
the following 9 years. The lower activity tolerance turned up
obviously when he was 9 years old. His mother denied the
history of trauma or surgery during her pregnancy. No neuro-
logical dysfunction was found in the admission examination.
Magnetic resonance image (MRI) showed the abnormal flow
void signal in the right craniocervical junction with no abnor-
mal changes in the brain parenchyma (Fig. 1). ECA-EJVAVF
was confirmed by the digital subtraction angiography (DSA)
(Fig. 2). The cardiac ultrasound indicated that the heart struc-
ture and blood flow were normal, and the ejection fraction was
66%. The chest X-ray indicated a slight expansion of his heart.
Surgical procedure Under general anesthesia, right ECA an-
giography indicated the significantly expanded ECA, commu-
nicating to the EJV directly. The high-flow fistula formed
between them, and the EJV also expanded obviously.
Because of the high flow, we compressed the EJV to decrease
the fistula flow; then, a few coils were filled into the proximal
trunk of the fistula. Rechecked DSA showed the fistula flow
Deqiu Cui and Jingwei Li contributed equally to this work.
* Gao Zeng
Department of Neurosurgery, Xuanwu Hospital, Capital Medical
University, 45 Chang Chun St., Beijing 100053, China
Childs Nerv Syst (2017) 33:1583–1587