OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases.METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well.RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively.CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.
Ambulatory Care Facilities ; Brain Stem Infarctions ; Central Nervous System Vascular Malformations ; Central Nervous System Venous Angioma ; Cerebral Veins ; Follow-Up Studies ; Humans ; Retrospective Studies ; Sinus Pericranii ; Varicose Veins ; Veins

Dural arteriovenous fistula (DAVF) is an uncommon condition. However, it is important for otologists because it is the most common cause of pulsatile tinnitus and may also present with intracranial hemorrhage and neurological disturbance. Many therapeutic approaches, such as surgery and endovascular techniques, have been proposed to obliterate the lesions. Surgery may be accompanied by massive blood loss. Transarterial embolization seldom obliterates transverse-sigmoid sinus DAVFs completely and permanently. In recent years, several authors have reported the effect of transvenous embolization of transverse-sigmoid sinus DAVFs in English literature. We recently experienced a case of sigmoid DAVF treated by transvenous embolization and report it with a review of the literature.
Arteriovenous Fistula ; Central Nervous System Vascular Malformations* ; Colon, Sigmoid ; Endovascular Procedures ; Intracranial Hemorrhages ; Tinnitus

A case of cavernous angioma of the cerebellar vermis is described. Cevernous angioma is rare vascular malformation of the central nervous system and the cerebellar vermis is very unusual site. Exact preoperative diagnosis of this lesion is difficult because cavernous angioma is angiographically silent and generally accepted to be very rare. The relevant literature is reviewed.
Central Nervous System ; Diagnosis ; Hemangioma ; Hemangioma, Cavernous* ; Vascular Malformations

A case of extradural cavernous hemangioma on lumbar spine combined with ruptured disc is reported. Cavernous hemangiomas are uncommon vascular malformation of the central nervous system. In the spine, the majority of these lesions are from secondary extensions of vertebral hemangiomas. Occurrence of a primary extradural cavernous hemangioma is relatively rare. Authors report a case of extradural cavernous hemangioma of the spine with the review of literatures.
Central Nervous System ; Hemangioma ; Hemangioma, Cavernous* ; Spine ; Vascular Malformations

Dural arteriovenous malformations are rare, accounting for 10-15% of all intracranial arteriovenous malformation and are thought to be acquired lesion caused by dural sinus thrombosis. The authors report a rare case of extrasinusal dural arteriovenous fistula in the right middle fossa adjacent to the superior orbital fissure. During the preceding 17 years, this 31-year-old female had complained of proptosis and ocular pain in the right eye. Computed tomography and magnetic resonance imaging showed a retrobular intraconal mass in the right orbit; Angiography revealed an extrasinusal dural arteriovenous fistula in the right middle fossa adjacent to the superior orbital fissure, which drains into the superior ophthalmic and inferior temporal vein. After embolization of the fistula by injection of N-butylcyanoacrylate(NBCA), we intended to remove the retrobulbar intraconal mass seen on CT and MRI by means of a unilateral partial orbitotomy on the supraorbital rim and orbital roof, and to this end, explored the right orbit. We were unable, however, to locate any mass lesion in the orbital cavity, and postoperative CT and MRI showed no such lesion. We were of the opinion that the mass shown on preoperative CT and MRI was a dilated intraorbital vein structure which disappeared after embolization, operative ligation and cutting of the dural arterivenous fistula. Finally, proptosis and ocular pain disappeared, and the patient recovered with any no neurologic deficit.
Adult ; Angiography ; Arteriovenous Malformations* ; Central Nervous System Vascular Malformations ; Exophthalmos ; Female ; Fistula ; Humans ; Intracranial Arteriovenous Malformations ; Ligation ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Orbit ; Sinus Thrombosis, Intracranial ; Veins

The approach to ethmoidal dural arteriovenous fistulas (eDAVFs) is usually via a pterional or a frontal craniotomy. However, the transfrontal sinus is a more direct route to the fistula. The aim of this report is to describe our experience and associated complications occurring as a result of flow diversion in the transfrontal sinus approach for eDAVFs. In this report, we discuss visual field defects occurring after a transfrontal sinus operation. This approach is most direct for surgical treatment of an eDAVF, enabling preservation of neural structures with minimal to no negative effects on the brain. Although the surgery was uneventful, the patient presented with a left side visual field defect. An ophthalmologic exam detected an arterial filling delay in the choroidal membrane and ischemic optic neuropathy was highly suspected. The patient is currently recovering under close observation with no special treatment. The transfrontal sinus approach provides the most direct and shortest route for eDAVFs, while minimizing intraoperative bleeding. However, complications, such as visual field defects may result from a sudden flow diversion or eyeball compression due to scalp traction.
Brain ; Central Nervous System Vascular Malformations* ; Choroid ; Craniotomy ; Fistula ; Frontal Sinus ; Hemorrhage ; Humans ; Intracranial Arteriovenous Malformations ; Membranes ; Optic Neuropathy, Ischemic ; Scalp ; Traction ; Visual Fields*

Intracerebral venous angiomas with arterial components have been reported rarely. A case of venous angioma with arterial component is presented. The patient was a 29-year-old-female who presented with abrupt headache followed by left hemiparesis. Computerized tomography and magnetic resonance imaging revealed intracerebral hematoma in right temporoparietal lobe and basal ganglia with surrounding signal voids. The cerebral angiography showed the characteristic umbrella appearance and single large draining vein of venous angioma in right temporoparietal lobe and fine arterial branches supplying the vascular malformation. At the operation, multiple arteriovenous fistulas without nidus were observed and delicate angiomatous network with red veins were found around the hematoma cavity, Histologic examination of the angiomatous network proved it to be an arteriovenous malformation. It was a variant of mixed angioma, venous angioma combined with arteriovenous malformation. Clinical, angiographic, and pathological features of intracerebral venous angiomas with arterial components were reviewed from literatures.
Arteriovenous Fistula ; Arteriovenous Malformations* ; Basal Ganglia ; Central Nervous System Venous Angioma* ; Cerebral Angiography ; Headache ; Hemangioma ; Hematoma ; Humans ; Magnetic Resonance Imaging ; Paresis ; Vascular Malformations ; Veins

Cavernous hemangiomas were first reported in 1929 by Globus and Doshay, and are defined as benign vascular structures developed between the neural tissues occurring in the central nervous system, consisting of a dilated vascular bed. Cavernous hemangiomas comprise nearly 5-12% of all spinal vascular malformations; however, existence in the epidural space without bone involvement is rare. Only 4% of all cavernous hemangiomas (0.22/1.000.000) are purely epidural cavernous hemangiomas. In this case report, we removed a hemorrhagic thoracic mass presenting with progressive neurological deficits in a 55-year-old male patient. We found this case to be appropriate for presentation due to the rare occurrence of this type of cavernous hemangioma.
Central Nervous System ; Epidural Space ; Hemangioma ; Hemangioma, Cavernous* ; Humans ; Male ; Middle Aged ; Thoracic Vertebrae ; Vascular Malformations

Sudden unexpected death in epilepsy (SUDEP) is a devastating complication in patient with epilepsy. The mechanism of SUDEP is unclear, but several risk factors are reported and various studies are ongoing. Cerebral cavernous hemangioma (CCH) is a kind of vascular malformation that can cause seizure. Seizure caused by CCH is usually progress to intractable epilepsy. Dendriform pulmonary ossification (DPO) is an uncommon and rarely diagnosed disease during life, and there have been no reports of SUDEP with DPO. We recently experienced an autopsy case of a young woman dying suddenly and unexpectedly, who had suffered from epilepsy caused by CCH and in one of them DPO was identified. Herein we report that case and provide a literature review.
Autopsy ; Epilepsy ; Female ; Hemangioma, Cavernous, Central Nervous System ; Humans ; Risk Factors ; Seizures ; Vascular Malformations

OBJECTIVES: Vascular tinnitus is the most common form of pulsatile tinnitus, particularly when the tinnitus corresponds with the pulse of patients. In this study, we reviewed the 10-year clinical data on vascular tinnitus of our tinnitus clinic to investigate the frequency of the underlying etiologies, to introduce a diagnostic protocol, and to evaluate the treatment outcomes. METHODS: We retrospectively collected the data of 57 patients who were diagnosed as vascular tinnitus between April 2001 and December 2011. Careful history taking, otoscopy, thorough physical examinations, audiometry, laboratory tests, as well as radiologic examinations were performed according to our diagnostic protocol to find the origin of pulsatile tinnitus. Treatment options were individualized based on the specific etiology, and the outcomes were assessed using patient's subjective reports at the follow-up interviews. RESULTS: High jugular bulb was the most common cause (47.4%) of vascular tinnitus, and venous hum was the next (17.5%). Dural arteriovenous fistula, intracranial aneurysm, atherosclerotic carotid artery disease, and hypertension were less common causes. Vascular tinnitus was alleviated in most patients after the appropriate treatment: surgical intervention, tinnitus retraining therapy, reassurance, and medications. CONCLUSION: Vascular tinnitus can be successfully diagnosed by the regular use of the suggested protocol. Many patients with vascular tinnitus have treatable underlying etiologies. Treatment of those etiologies or at least counseling about the tinnitus itself can benefit the patients with troublesome vascular tinnitus.
Arteriovenous Fistula ; Audiometry ; Carotid Artery Diseases ; Central Nervous System Vascular Malformations ; Counseling ; Follow-Up Studies ; Humans ; Hypertension ; Intracranial Aneurysm ; Magnetic Resonance Angiography ; Otoscopy ; Physical Examination ; Retrospective Studies ; Tinnitus*