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A 49-year-old man was admitted to our hospital complaining of severe epigastric pain. Contrast-enhanced abdominal computed tomography revealed an arteriovenous malformation of the pancreatic uncus. Because of unbearable epigastric pain, transcatheter arterial embolization was performed with n-butyl-2-cyanoacrylate. Subsequently, his symptoms fully resolved; no recurrence of the arteriovenous malformation was seen more than 1 year after the embolization.

Paraspinal arteriovenous fistulas (AVFs), with the fistulas draining into the epidural veins alone, are relatively rare and few cases have been reported until now. We reported a case of cervical paraspinal AVFs draining only into the epidural venous plexus without reflux into the intradural venous system. The patient showed myelopathy due to direct compression of the spinal cord by a large varix. A 57-year-old man presented with gait disturbance. Neurological findings on admission revealed that tetraparesis, sensory disturbance of four extremitas below the C5 level and bladder bowel dysfunction. Magnetic resonance imaging and computed tomography at the cervical level disclosed remarkable compression of the spinal cord by a large venous pouch at the C6 level. Digital subtraction angiography (DSA) revealed paraspinal arteriovenous fistulas fed by bilateral C6 radicular arteries, the right ascending cervical arteries, and the right deep cervical artery in the right C6 intervertebral foramen. Three-staged transarterial embolization was performed by selective catheterization of the multiple feeders with n-butylcyanoacrylate, followed by transvenous embolization. During transvenous embolization, motor evoked potential (MEP) monitoring was performed. After retrograde catheterization of the epidural venous plexus, the large varix was occluded with Guglielmi detachable coils. The AV fistulas were completely occluded without any change in MEP monitoring during the procedure. The patient's gait improved well after the procedure and follow up DSA six months later showed no recurrence of the paraspinal AVFs.

We report a rare case of a vertebral arteriovenous fistula that developed as a complication of atlantoaxial transarticular screw fixation. The patient was a 44-year-old male with a history of juvenile rheumatoid arthritis. He had undergone an atlantoaxial transarticular screw fixation for an atlantoaxial dislocation. At 2 months after the surgery, he complained of right-side tinnitus. A selective left vertebral angiography showed a high-flow arteriovenous fistula of the right V2 segment and occulusion of the right vertebral artery at the level of the C3 vertebral body. Endovascular embolization of the arteriovenous fistula was successfully performed using detachable coils. No deficits were observed after the treatment, and the tinnitus disappeared completely. Endovascular coil embolization is currently an effective and safe treatment for the vertebral arteriovenous fistula.

A 44-year-old male with right vertebral arteriovenous fistula accompanied with tinnitus, underwent endovascular treatment using GDC. A digital subtraction angiography clearly showed one fistula flowed from the right vertebral artery (VA) to the vertebral venous plexus, while the right VA close to the fistula was interupped with HyperForm. The tip of the micro catheter was placed in the vertebral venous plexus through fistula from the right VA, and the vertebral venous plexus around the fistula was embolized with 4 GDCs. Blood flow of the right VA was maintained. Follow-up angiography undertaken 6 months after the operation didn't show the recurrence of arteriovenous fistula.

Sclerotherapy with absolute ethanol and/or polidocanol is a well-established therapeutic modality for the treatment of peripheral vascular malformations, although systemic complications such as hemoglobinuria and pulmonary embolism could occur. We report two cases of pulmonary embolism associated with sclerotherapy for peripheral vascular malformations. Two patients, a 17-year-old man and a 17-year-old woman, undergoing absolute ethanol sclerotherapy for vascular malformations of the leg developed pulmonary embolism after injection of ethanol. Pulmonary embolism, suspected by the clinical symptoms such as hypoxia and hypocapnia, was confirmed by the pulmonary scintigraphy showing minimal pulmonary defects. Hemoglobinuria was also observed with injection of ethanol. Patients recovered rapidly with heparin and urokinase therapy. The review of perioperative complications with sclerotherapy for peripheral vascular malformations in our institution for past four years revealed that complications were observed in 18 out of 88 patients (20.5%), and in 32 out of 183 cases (17.5%). Major complications were hemoglobinuria, pulmonary embolism, shivering and delayed emergence from general anesthesia. We conclude that sclerotherapy for vascular malformations under general anesthesia is a risky procedure and this must be carefully managed with keen monitoring of Spo2 and Etco2.

We presented a case of neurofibromatosis type I (NF-1) associated with an extracranial arteriovenous fistula (AVF) fed by the occipital and vertebral artery. A 20-year-old man was referred to our hospital because of an occipital subcutaneous pulsatile mass. A CT scan showed a huge subcutaneous enhanced mass. Angiography revealed that the occipital AVF was fed by bilateral occipital arteries, the left ascending pharyngeal artery, the left middle cerebral artery, and the left vertebral artery with-abundant communication with the subcutaneous veins. Endovascular treatment by using both coil and glue (Eudragid) embolization via the occipital artery successfully obliterated the AVF. Subsequently surgical operation was performed. Postoperative angiography showed the disappearance of the AVF. Combination of intravascular surgery and surgical operation should be considered as an effective treatment for NF-1 associated with AVF.

A 67-year-old woman was admitted to our hospital for a complete medical evaluation of a pulmonary nodule on a chest X-ray film, which was found during an annual check-up. A loud bruit was audible over the left chest and the supraclavicular region. Computerized tomography (CT) scan and magnetic resonance imaging revealed an arteriovenous fistula from a branch of the left subclavian artery into the paravertebral veins. Angiography disclosed that the arteriovenous fistula was fed by the left costocervical artery. Transarterial embolization of the arteriovenous fistula was done with 5 mechanical detachable coils and 2 micro coils. Postembolization angiography showed a marked decrease in blood flow and near-elimination of the arteriovenous fistula. The shrinkage of the arteriovenous fistula was also observed on CT scan. This patient had been in a traffic accident, and had been clinically well before the accident. Post-traumatic arteriovenous fistula was strongly suspected, because a pulmonary nodule on chest X-ray film and a bruit were found after a traffic accident.

A case of a traumatic vertebral arteriovenous fistula associated with a hangman's fracture is reported. A 45-year-old male fell down about 2 meters and struck his parietooccipital region against the ground. Profuse nasal bleeding developed. He was transferred to a local hospital, where his respiration was ataxic and blood pressure was low. After intubation, he was transferred to our emergency department. Cervical x-p revealed fracture of C1, C2 and subluxation of C2 body. Because of uncontrollable nasal bleeding, the bilateral maxillary arteries were embolized with spongel. At this time, right vertebral angiograms demonstrated a vertebral arteriovenous fistula with an pseudoaneurysm located at C2 level. On the 13th hospital day, direct balloon occlusion of the fistula was attempted; this could not be achieved because the subclavian and vertebral arteries were tortuous and the balloon catheter could not be introduced to the level of the fistula in the vertebral artery. The patient was only observed until follow-up angiogram on the 24th hospital day revealed enlargement of the pseudoaneurysm. We performed trapping of both the proximal and distal ends of the involved vertebral artery; from C5 to C1. Postoperative course was uneventful, hangman's fracture was fixed with a Halo vest. Four months after operation, fistula and pseudoaneurysm were not opacified on angiogram. We believe that transvascular techniques are the treatment of choice for vertebral arteriovenous fistulas. However, as the next best thing, we can use trapping for the patient whose vessels are too tortuous to introduce the balloon catheter to the involved vessel.

A 49-year-old man was admitted to our hospital complaining of clumsiness in his left arm and leg that had 2 months' history with subacute onset. On examination he showed left sided ataxia but no other neurological deficits. A linear lesion was demonstrated in the left cerebellar hemisphere by X-ray computed tomography and magnetic resonance imaging without apparent hemorrhage and mass effect. Digital subtraction angiography revealed a venous malformation in the left cerebellar hemisphere but carotid arteries were normal. Relative distribution of cerebral blood flow was measured using single-photon-emission-computed tomography with rotating gamma camera and 123I-IMP. Reduced uptake was observed in the right frontal lobe (-15- -30%). However, the asymmetry disappeared 8 months later. The decreased blood flow in the contralateral cerebral cortex may be due to destruction of cerebello-thalamo-cortical pathways.

The abnormal midline cyst of diencephalic origin was termed "diencephalic cyst" by Brocklehurst in 1973. Since then, several case reports of so-called "diencephalic cyst" have appeared, but its clinical picture, especially its relation to other midline dysraphisms, does not seem to be well understood. Our experience with four cases of so-called "diencephalic cyst" were reported and their relation to holoprosencephaly as well as simple agenesis of corpus callosum was discussed. (1) In all of these cases, there was an abnormal space in the midline which freely communicated with the lateral ventricle. In the first two cases, their clinical pictures as well as the features of contrast studies were typical of alobar holoprosencephaly. In the third case, the absence of the olfactory tract, the incompletely separated cerebrum, and the monoventricle were confirmed at autopsy. In the last case, the ventricle was also incompletely separated. From these observations, such a malformation should better be considered as a variant of holoprosencephaly, and it would not be appropriate to classify this malformation, as Brocklehurst claimed, as "a maldevelopment lying between the group associated with anterior neuropore closure and prosencephalization (anterior encephaloceles and holoprosencephaly) and the group associated with maldevelopment of the hind brain (cerebellar and fourth ventricle encephaloceles and Dandy-Walker cyst of the rhombencephalon)." (2) This malformation is apparently formed before the 40 mm stage of the embryo, since by this time the diencephalic roof has already sunk to the level of the adult form. This maldevelopment should be clearly differenciated from simple agenesis of the corpus callosum which is formed after the 60 mm stage of the embryo. In simple agenesis of the corpus callosum, the deep venous system might take an abnormal course, but is well formed, whereas in holoprosencephaly or so-called "diencephalic cyst", the internal cerebral vein as well as the straight sinus and the inferior sagittal sinus is absent. (3) In all of our cases, there was an abnormal space posterior and inferior to the elevated lateral sinus. Autopsy done in two cases revealed that this abnormal space was a Dandy-Walker cyst in one case, the the dorsal sack of diencephalic origin in the other case. The latter case tells that in the cases of holoprosencephaly the lateral sinus does not necessarily mark the boundary between the cerebrum and the cerebellum. (4) "Diencephalic cyst" often accompanies severe hydrocephalus. However, even with successful surgical treatment of hydrocephalus, the outcome of intelligence in these cases would be grave.

A 12-year-old man was referred to our clinic because of sanguineous discharge. At the out-patient department, bilateral varicocele was found. Urinalysis showed microscopic hematuria, but no other laboratory data were abnormal. Excretion urography was normal. The retrograde phlebograph showed a rare venous return via Santorini's plexus and lumbar veins. After varicocelectomy, urinalysis improved.

A case of Sturge-Weber syndrome with marked abnormalities in the cerebral venous system was reported. The patient was a 2-year old boy who was admitted to the Department of Neurosurgery with the chief complaints of left hemiparesis and left focal seizures. He had vascular nevi on the forehead and upper lip of his face, scalp, right forearm and thigh (Fig. 1). Neurological examination of admission revealed left hemiparesis. Plain skull films indicated no intracranial calcification. EEG showed paroxysmal focus in the right parieto-temporal area. Plain CT scan showed calcium deposits in the right parietooccipital area and contrast enhancement occurred around the areas of calcification (Fig. 2). Venous phases of bilateral CAGs showed abnormalities of the cortical veins and sinuses and abnormal drainage from the cerebral cortex to the deep veins. It also demonstrated persistence of the primitive straight sinus (Fig. 3, 4). From the neurological and neuroradiological findings, this case was diagnosed as the Sturge-Weber syndrome with marked abnormalities in the cerebral venous system. These abnormal findings of veins and sinuses seemed to be brought about by development abnormalities of veins and sinuses which continuously occurred following Streeter's primordial plexus, which has been considered to be a cause of the Sturge-Weber syndrome.

A case classified to "cerebral venous dysgenesis" (Kagawa et al 1972) is reported. A 16-year-old girl was admitted complaining of psychomotor seizure which began seven years before. At the first admission (seven years ago) she was diagnosed as psychomotor seizure and cerebral venous dysgenesis by CT scan, EEG and cerebral angiography. Then she was treated by medication (anticonvulsants) for seven years. At the second admission (this time) left temporal lobectomy was performed by means of monitoring the cortical EEG. CT scan showed atrophy of the left temporal lobe. Spike and wave complex was seen in EEG. Carotid angiography disclosed the abnormal networks among the fine venules and their connection to tortuous and coiling veins. The abnormality was seen in the frontal, parietal, and temporal area. After operation psychomotor seizure has been controlled by medication. Pathologically tortuous vessels, gliosis and arachnoidal fibrosis were shown. This case should be classified to "cerebral venous dysgenesis" differentiated from the venous angioma. Classification of venous malformation, clinical signs, angiographical findings, and genesis were discussed.