Pial arteriovenous fistula (AVF) is a rare cerebrovascular malformation. According to a series reported by Halbach, pial AVF accounts for 1.6% of all intracranial vascular malformations. Herein, we report a rare case of pial AVF in a 67-year-old male on medication for alcoholic liver cirrhosis (Child-Pugh A) with esophageal varix, who presented with left-side motor weakness and mental fogginess. Digital subtraction angiography revealed contrast leakage in the left internal carotid artery and confirmed pial AVF. After angiography, brain computed tomography confirmed contrast leakage due to a ruptured fistulous point. Therefore, a Guglielmi detachable coil was placed and Onyx embolization to the fistula point was performed. In the present report, we describe this rare case and provide a review of the literature.
Pial arteriovenous fistula (AVF) is a rare cerebrovascular malformation. Intracranial pial AVFs have single or multiple arterial connections to a single venous channel. Pial AVFs differ from brain arteriovenous malformations in that they lack a true nidus. Pial AVFs differ from dural AVFs in that they derive their arterial supply from pial or cortical arteries that are not located within the dura mater. Because pial AVF has a poor natural history, clinical consideration of pial AVF followed by prompt appropriate treatment is important. We report a rare case of a 67-year-old male with intracranial hematoma caused by pial AVF along with a review of the literature.
A 67-year-old male with a history of alcoholic liver cirrhosis (Child-Pugh A) with esophageal varix presented to the emergency department with left-side motor weakness and mental cloudiness. He had been treated with conservative therapy at our hospital for alcoholic liver cirrhosis with esophageal varix. His past history included two surgical procedures for esophageal varices. Five hours before admission to our hospital, he was found unconscious in bed. The initial neurological examination revealed that he was in a drowsy state (Glasgow Coma Scale was E3M4V6) and left hemiparesis was noted. The platelet count was as low as 43,000, but coagulation factors were in the normal range. The lab values for liver function test were above the normal range (total bilirubin, 1.96 mg/dL; serum glutamic-pyruvic transaminase, 42.5 U/L; serum glutamic-oxaloacetic transaminase, 194.3 U/L). There was no history of head trauma or head injury. Brain computed tomography revealed the presence of an acute intracranial hematoma in the right medial frontal lobe with a significant mass effect (
Pial AVF accounts for 1.6% of intracranial vascular malformations and has a poor natural history [
Pial AVFs can be congenital or result from a traumatic injury. Little is known about their pathophysiological mechanisms. Abnormal angiogenesis may play a role in the formation of pial AVFs [
The patient in this study is currently on medication for alcoholic liver cirrhosis with esophageal varices and underwent two surgical procedures for esophageal varices. The patient presented with left-side motor weakness and mental cloudiness. Intracranial hemorrhage was observed on brain CT, and pial AVF was confirmed using DSA. It is possible that liver disease predisposes to both intracranial hemorrhage and thrombosis via a disordered and unstable coagulation system, as some studies have found an association between cirrhotic liver disease and venous thromboembolism [
Venous varices commonly associated with AVFs are produced by the high turbulent flow caused by arteriovenous shunting. Shunt disconnection can be accomplished surgically or endovascularly [
Although pial AVF is usually asymptomatic, it may cause severe hemorrhage and neurological deficits that require endovascular and surgical treatment, especially in patients with liver problems. Because pial AVFs have a poor natural history and relatively good clinical outcome with prompt treatment, it is important to establish a clinical diagnosis. Once the diagnosis has been made, a neurosurgical and neuroendovascular team should determine the most appropriate treatment modality.
No potential conflict of interest relevant to this article was reported.
This study was supported by a research fund from Chosun University Hospital, 2020.
Computed tomography (CT) angiography showing an acute intracranial hematoma in the right medial frontal lobe and contrast leakage (A,B) (arrow) and drained into the vein (C,D).
(A) Digital subtraction angiography (DSA). Anterioposterior (left) and lateral (right) views from the arterial phase to the venous phase showing that the fistulous point (circle) was fed by the callosomarginal artery (arrowhead) and drained into the vein (arrow). (B) Postoperative DSA image showing excision of the pial arteriovenous fistula.
Follow-up computed tomography (CT) scans of the brain taken 1 month (A) and 1 year (B) after the patient’s presentation to the emergency department. CT shows a resolved hematoma in the right medial frontal lobe.