Postoperative hyperperfusion in dural arteriovenous fistula associated with venous ischemia: case report

BACKGROUND: It is well known that carotid endarterectomy and extracranial-intracranial arterial bypass sometimes cause postoperative hyperperfusion, and vasoparalysis attributable to long-standing ischemia has been suggested as the cause. It is also well known that dural arteriovenous fistula (AVF) sometimes causes cerebral ischemia attributable to venous hypertension. However, there are few reports regarding the postoperative changes of regional cerebral blood flow (rCBF). METHODS: We report a case of dural AVF of the left transverse/sigmoid sinuses, occurring in a 64-year-old man. Intraoperative transvenous embolization combined with transarterial embolization was performed, and the rCBF was measured pre- and postoperatively using 99mTc-hexamethyl-propylene amine oxime and single-photon emission computed tomography (SPECT). RESULTS: Preoperative SPECT disclosed a marked rCBF reduction in the left temporal, parietal, and occipital lobes. Complete obliteration of the AVF was attained after the intraoperative transvenous embolization, without any neurological deterioration. However, postoperative SPECT demonstrated temporary hyperperfusion in these regions. CONCLUSIONS: Sudden resolution of venous ischemia can lead to postoperative hyperperfusion, and pre- and post-treatment rCBF studies are important to prevent complications related to hyperperfusion.     

Histopathological aspects of dural arteriovenous fistulas in the transverse-sigmoid sinus region in nine patients

OBJECTIVE: In recent years, dural arteriovenous fistulas (DAVFs) have been primarily thought to be acquired lesions, formed after sinus thrombosis. The pathogenesis of DAVF, however, is still controversial. We have studied histopathological aspects of DAVFs in resected specimens obtained from nine patients, to obtain clues to the pathogenesis of DAVFs. METHODS: Histological comparison was made among nine DAVF cases and five control cases without venous sinus disease. In addition, the relationship between the clinical course and histological aspects was investigated. RESULTS: The essential abnormality found was a connection between the dural arteries and the dural veins within the venous sinus wall, through small vessels averaging approximately 30 microns in diameter. By using several staining methods, we confirmed that the vessels were part of the venous system; we named these dilated venules "crack-like vessels." CONCLUSIONS: The development of abnormal communications between dural arteries and dural veins (crack-like vessels) is regarded as the essential part of the pathogenesis of DAVFs, and sinus thrombus is not thought to be an essential lesion of DAVFs. It might be postulated that sinus hypertension caused by stenocclusive disease of the venous sinuses triggers the development of fistulous connections between arteries and veins in the dural wall, which may result in increasingly dilated venules and the formation of DAVFs.     

Hypothalamic hamartoma: report of a case with unusual histologic features

A rare case of hypothalamic hamartoma with unusual radiologic and histopathological features is described, possibly the first of its type in English literature. A 1.5-year-old female child presented with precocious puberty. MR scan of the brain revealed a pedunculated hypothalamic mass, most of which was isointense with normal brain on T1- and T2-weighted images. However, a sizeable component of the lesion was hyperintense on T1-weighted images, suggestive of adipose tissue. Microscopically, the lesion was a hamartoma composed of an admixture of neuroectodermal elements, namely glial cells, neurons, and nerve bundles along with mesenchymal elements in the form of fibroadipose tissue.     

Venous hypertension associated with a posterior fossa dural arteriovenous fistula: another cause of bithalamic lesions on MR images

We report a case of a posterior fossa arteriovenous fistula (AVF) with bithalamic hyperintensity of MR images. The thalamic abnormality improved after surgery, suggesting reversible venous hypertension as the pathogenesis of the finding, as opposed to infarction. This manifestation of a posterior fossa AVF should be considered in the differential diagnosis of bilateral thalamic disease.     

Endocarditis of the tricuspid valve associated with congenital coronary arteriovenous fistula

A 17 year old girl with a congenital right coronary artery to coronary sinus fistula presented with recurrent septic pulmonary embolism secondary to tricuspid valve endocarditis. The diagnosis was made on the basis of echocardiography and cardiac angiography.     

Acquired dural arteriovenous malformations of the lumbar spine: case report

OBJECTIVE AND IMPORTANCE: Although dural arteriovenous malformations (AVMs) are considered to be acquired, no conclusive evidence has been obtained. The first case of dural AVMs of the lumbar spine that showed evidence supporting their being acquired lesions is described. CLINICAL PRESENTATION: A 27-year-old man was admitted to our hospital in November 1994, presenting with increasing pain and numbness in the left buttock to the left lower limb. Myelography and arteriography showed dural AVMs of the lumbar spine. However, the myelogram obtained before surgery for lumbar disc herniation in 1987 provided no findings suggestive of intradural vascular anomalies. INTERVENTION: Embolization procedures via the left iliolumbar arteries were performed. However, there was no improvement of the symptoms, and the patient underwent surgical treatment. CONCLUSION: The present report documents a case of dural AVMs of the lumbar spine that showed evidence supporting their being acquired lesions.     

Reversible dementia due to dural arteriovenous fistula: a simple surgical option

Dural AVMs can produce a wide variety of symptoms related to raised intracranial pressure, venous congestion, and cerebral ischaemia. We present a unique case of reversible dementia, due to venous hypertension. The cerebral ischaemia was caused by extensive bilateral arteriovenous malformations of the external carotid system, which drained into the superior sagittal and transverse sinuses and resulted in venous hypertension. Although partial occlusion by endovascular embolization and ligation procedures had some effect, only 'scalping and silastic implantation' gave satisfactory amelioration of symptoms.     

Spinal epidural arteriovenous fistula with intramedullary reflux. Case report

A spinal epidural arteriovenous fistula with secondary reflux into the perimedullary veins is a rare entity. The authors present such a case with a discussion of its pathophysiology and treatment. The mechanism for formation of a spinal dural arteriovenous fistula is outlined based on the anatomical substrates in this region.     

Paralysis of the hypoglossal nerve in the presentation of dural arteriovenous fistula of the posterior fossa

BACKGROUND: Left-ventricular assist device implantation (LVAD) is still associated with thromboembolism as the optimal anticoagulation is still unclear. We report on the effects of adding platelet inhibitors to our anticoagulation regimen in our Novacor LVAD program. METHODS: Oral platelet aggregation inhibitors (aspirin 330 mg + dipyridamole 75 mg, three times per day) were added to the heparin/phenprocoumon treatment in 9 patients starting on postoperative day 3 to 7 (group A). Of the previous 41 patients, the last 20 patients served as a control group (group B), to reduce any learning curve effect. RESULTS: The mean interval of mechanical support between the two groups was comparable (group A vs B: 148 +/- 127 vs 104 +/- 61 days, n.s.). Accordingly, the cumulative support was much lower in group A (1051 days) as compared to group B (2091 days). In group B, 10 patients (50%) developed clinically evident thromboembolism. The number of events ranged from 1 to 10 (mean 1.4 +/- 2.3), with a total of 32. With addition of platelet inhibitors, the incidence of cerebral embolism dramatically dropped, as only one patient presented with transient ischemic attacks in group A (p < 0.05). Thoracic bleeding as defined by excessive drainage losses requiring redo thoracotomy did not increase (group A vs B: 22% vs 20%, n.s.). CONCLUSION: Addition of platelet inhibitors to heparin/phenprocoumon effectively prevents thromboembolism. However, platelet inhibitors should be postponed until sufficient hemostasis is achieved, since too early administration is associated with an increased risk of bleeding.     

A case of cavernous dural arteriovenous fistula resulting in intracerebral extravasation during transvenous embolization

Cortical venous drainage has been described as one of the major risk factors for dural arteriovenous fistula, which may induce venous hypertension leading to venous ischemia or intracerebral hemorrhage. However, it is rather rare to observe cortical venous drainage manifesting in this way in the cavernous sinus region. We report a case of a 55-year-old gentleman with a right cavernous dural arteriovenous fistula, presenting with conjunctival chemosis, exophthalmus and ocular hypertension on the affected side. Magnetic resonance imaging showed a small intracerebral hemorrhage in the right frontal lobe. Cerebral angiography revealed a dural arteriovenous fistula in the right cavernous sinus draining into the right olfactory vein via the uncal vein, as well as into the superior and inferior ophthalmic veins. This unusual cortical venous reflux was thought to be consistent with the intracerebral hemorrhage found on the magnetic resonance imaging. The patient underwent transvenous embolization for the dural arteriovenous fistula using an inferior petrosal catheterization into the uncal vein was difficult, and the cortical venous reflux through the vein seemed to be slight. However, extravasation of the contrast material occurred in the right frontal lobe after obliteration of the ophthalmic veins during the procedure. The cause of the extravasation was suspected to be the same olfactory vein that had been involved in the previous intracerebral hemorrhage. The obliteration of the dural fistula was continued rapidly, and the fistula disappeared after the embolization. Neurologically, the patient had no noticeable troubles, except for a mild headache. The pretreatment symptoms were alleviated within several days, and the patient was discharged in a week. We emphasize the following points from this rare case in order to facilitate a safer procedure during transvenous embolization for cavernous dural arteriovenous fistula. It is important to obliterate the cortical venous drainage as early as possible, even if the reflux is small or the catheterization is difficult. Repeated, careful sinography is useful for the evaluation of the drainage pattern at certain stages during the transvenous embolization procedure.     

Hypothalamic hamartoma associated with multiple congenital abnormalities. Two patients and a review of reported cases

We report 2 patients with hypothalamic hamartoma associated with multiple congenital abnormalities and analyze 42 (including our own) reported cases, including our 2 cases, of hypothalamic hamartoma or hypothalamic hamartoblastoma with multiple congenital abnormalities, to understand the timing of their occurrence and clarify the prognosis. To this end, we classified them into lethal and nonlethal cases. We found poly- and syndactyly, cleft or high-arched palate and nose abnormalities to be important manifestations of this syndrome. Major organ abnormalities and CNS and endocrine abnormalities occurred frequently among the lethal cases, very likely indicative of a disturbance of embryogenesis between gestational days 34-37 and thus implicated in a negative prognosis.     

Dural arteriovenous fistula caused by jugular vein stenosis--case report

This paper considers ways of improving patient co-operation. It is divided into two parts. Firstly, communication with the patient (i.e. the child and their parents) and secondly, the use of simple reward charts that are relevant to orthodontics.     

A case of dural arteriovenous fistula in the transverse and sigmoid sinus which developed over five years

We report a case of dural arteriovenous fistula (dAVF) of the transverse and sigmoid sinus which developed over five years. The patient was a 64-year-old man, who had a history of congestive heart failure and transient ischemic attack (TIA). No abnormal lesions were detected in the cerebral angiograms five years prior to the time the etiology of his TIA was investigated. He suddenly suffered from left hemiparesis and the CT scan showed right parietal subcortical hemorrhage. Right carotid angiogram demonstrated dAVF of the transverse and sigmoid sinus supplied by the right occipital and ascending pharyngeal arteries. We thought the reason for the subcortical hemorrhage in this patient was due to the disturbance of venous return in the right cerebral hemisphere. We underwent direct embolization of the right transverse sinus by interlocking detachable coils. Postoperative cerebral angiograms demonstrated the disappearance of dAVF and he was discharged. Whether dAVF is a congenital anomaly or not, has been controversial. Since the dAVF in this case had newly developed over five years, it is suggested that dAVF is an acquired lesion.     

Symptomatic hamartoma of the spinal cord associated with neurofibromatosis type 1. Case report

The authors present a case in which a symptomatic hamartoma was found in the spinal cord of a patient with neurofibromatosis type 1 (NF-1). This 52-year-old woman presented with painful urinary incontinence. Magnetic resonance (MR) imaging revealed an intramedullary lesion within the lower thoracic spinal cord and conus medullaris, which was surgically removed. Pathological investigation showed a hamartomatous lesion consisting of glial cells, ganglion cells, abundant disoriented axons, and thin-walled vessels. This case provides a pathological correlate to the hamartomatous lesions demonstrated on MR imaging in patients with NF-1 and illustrates that these benign lesions may become symptomatic and require neurosurgical intervention.     

Hypothalamic hamartoma with skeletal malformations, gelastic epilepsy and precocious puberty

A child is described who has skeletal malformations, gelastic epilepsy, precocious puberty and a hypothalamic hamartoma. The skeletal abnormalities were detected at birth, she developed gelastic epilepsy at the age of 3 years 5 months and precocious puberty at 3 years 8 months. A hypothalamic hamartoma was found on MRI. The precocious puberty has been successfully medically managed, though her seizures are difficult to control. The combination of all four features has not been described previously.     

Pulmonary arteriovenous fistula with cerebral arteriovenous malformation without hereditary hemorrhagic telangiectasia. Unusual case report and literature review

An unusual and rare case of a central pulmonary arteriovenous fistula (PAVF) with concomitant cerebral arteriovenous malformation (CAVM) in the absence of hereditary hemorrhagic telangiectasia is reported. Presentation was in the form of a cerebrovascular accident (CVA). Clinical presentations and management strategies of PAVF with associated CAVM are broadly discussed along with a pertinent literature review.     

Progressive aneurysmal degeneration of cortical venous drainage of dural arteriovenous malformations: case report

OBJECTIVE AND IMPORTANCE: The development of a venous aneurysm associated with dural arteriovenous malformations rarely has been documented in the literature. CLINICAL PRESENTATION: A 64-year-old man with known dural arteriovenous malformations developed a venous aneurysms, as shown on sequential angiograms obtained during 2 years. INTERVENTION: The dural arteriovenous malformations were treated with neuroendovascular embolization and then surgical excision. CONCLUSION: The clinical presentation, diagnosis, and treatment of this unusual case are presented.     

Cranial dural arteriovenous fistulas--current status of therapy

Spontaneous dural arteriovenous fistulas (DAVF) are abnormal arteriovenous shunts with a nidus within the dura mater. The exact etiology of the spontaneous DAVFs remains uncertain. Arterial inflow is from the meningeal branches in the region, which can be from an extracranial or an intracranial source. The venous drainage is through the dural sinuses, although cortical or deep venous drainage may be recruited. The critical symptoms are highly dependent on the venous drainage. Computed tomography and/or magnetic resonance imaging demonstrates the epiphenomena rather than the DAVF itself. Angiography is the most important modality in evaluating a DAVF. Based on the patterns of the venous drainage, a classification of the DAVF is possible. This classification enables decision-making about the appropriate therapy. Also the risk of each DAVF may be determined.