Vertebro-epidural lumbosacral vascular malformations. An unusual cause of lumbo-sciatic pain

Sciatica is most commonly caused by nerve root compression secondary to herniated disk. Rarely, it can be due to a lumbosacral vascular malformation. We present five cases with such a malformation, presenting as a chronic lumboradiculagia. The patients were explored with computed tomography, MRI and selective spinal angiography. Polymorphic anatomic and hemodynamic aspect of these cases are reported: 1. One vertebral hemangioma with epidural extension; 2. Three purely epidural malformations: a) one epidural cavernous hemangioma, b) one epidural arteriovenous malformation, c) one epidural varix; 3. One paravertebral arteriovenous fistula with epidural venous drainage. Diagnosis of these rare malformations may be difficult. A multiplanar cross-sectional magnetic resonance and computed tomography scan with contrast enhancement can show characteristic abnormalities and may assist in recognition these malformations. Selective spinal angiography confirms the diagnosis, allows to classify the malformation and is required to evaluate endovascular therapeutic possibilities.     

A case of a large neonatal arteriovenous malformation with heart failure. Color Doppler sonography,MRI and MR angiography as early non-invasive diagnostic procedures

We report a case of a large arteriovenous malformation (AVM) of neonatal onset with heart failure. Transfontanel color Doppler sonography revealed abnormal vessels in the early stage of the investigation. Magnetic resonance imaging (MRI) revealed numerous flow voids suggesting abnormal vessels, and magnetic resonance angiography (MRA) disclosed numerous bizarre abnormal vessels. Color Doppler sonography is a convenient and appropriate procedure for the early bedside diagnosis of neonatal AVMs. MRI and MRA can replace cerebral angiography for the diagnosis of neonatal AVMs.     

Incidence of vascular malformations in spontaneous intra-cerebral haemorrhage in children

Aim To assess the incidence of vascular malformations in children presenting with non-traumatic intra-cerebral haemorrhage and outline the need for radiological investigations. Materials and methods Between 1993 and 2002, 26 children presented with spontaneous intra-cerebral haemorrhage at a mean age of 26 months (range 1–192 months). Results Twenty-two children had, as first treatment, surgical removal of the haematoma and any malformation found. One patient with no evidence of vascular malformation had conservative treatment, two had embolisation and one had stereotactic radiosurgery of arteriovenous malformations (AVMs). The diagnosis of vascular malformation was confirmed histologically and/or radiologically in 16 (61%) patients. Of these 16 patients, 7 were AVMs, 1 thrombosed middle cerebral artery (MCA) aneurysm, 1 cavernous angioma, 6 aggregates of abnormal vessels, 1 vein of Galen aneurysm. Pre-operatively, 12 patients had magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and 7 were positive for vascular malformation (1 false positive) with no false negatives. Digital subtraction angiography (DSA) was performed pre-operatively in 7 patients, 4 were positive with no false negatives. There were no re-bleeds at the follow-up period. Five (19%) patients died from the haemorrhage, 7 (27%) had severe neurological deficit and 14 (54%) had no neurological deficit. Residual malformation after surgery requiring additional treatment was found in 3 (16%) patients. All patients with confirmed malformations were followed-up with DSA. Conclusion MRI/MRA has high sensitivity and specificity in identifying vascular malformations in children presenting acutely with spontaneous intra-cerebral haemorrhage. This may prove useful when pre-operative DSA is not promptly available. After acute clot evacuation, there is high incidence of residual malformation and such patients should be followed-up with DSA. This paper was presented at the XX Congress of the European Society for Pediatric Neurosurgery, Martinique, French Antilles, March 3–9, 2006.     

Moyamoya disease: importance of the MRI-MRA combination and difficulties in management and follow-up in 7 cases

PURPOSE: To point out the importance of both MRI and MR angiography for the diagnosis and follow-up of moyamoya disease. Materials and methods: We report seven cases. MRI was performed in the initial phase in three cases. MR angiography without conventional angiography led to the diagnosis in one case. Conventional angiography was performed in six cases. Follow-up MRI and MR angiography were obtained in two. RESULTS: The initial MRI revealed ischemic lesions in the sylvian territory in two cases, and a cerebral malformation in one, after-effect lesions in two, and cerebral atrophy and ventricular dilatation in one. Conventional angiography provided the diagnosis of moyamoya disease in all cases, associated with cerebral arteriovenous malformation in two. CONCLUSION: MRI and MRA should be performed for the diagnosis and follow-up of moyamoya disease.     

Spinal vascular malformations--typical and atypical findings

Vascular malformations of the spinal cord and its meninges are rare diseases which comprise true inborn cavernomas and arteriovenous malformations (AVM), including perimedullary fistulae, glomerular and juvenile AVMs, and presumably acquired dural arteriovenous fistulae. This article gives an overview of the imaging features on magnetic resonance imaging (MRI) and digital subtraction angiography of both typical and atypical findings to describe the wide variety of possible pathological entities encountered. Clinical differential diagnoses, the neurological symptomatology and potential therapeutic approaches of these diseases, which may vary depending on the underlying pathology, are given. Although MRI constitutes the first choice diagnostic modality for suspected spinal vascular malformations, we conclude that the definite diagnosis of the disease and thus the choice of the appropriate therapeutic approach rests on selective spinal angiography which should be performed at a specialized center. Treatment in symptomatic patients offers an improvement in prognosis. Microsurgical treatment is recommended for symptomatic spinal cord cavernomas. Dural arteriovenous shunts can either be treated by microsurgical or endovascular approaches, the former being a simple, quick and secure approach to obliterate the fistula while the latter is technically demanding. In spinal arteriovenous malformations of both the fistulous and the glomerular type, the endovascular approach is the method of first choice; in selected cases, surgery or a combined therapy may be necessary.     

Imaging in spinal vascular disease

Spinal vascular diseases are rare and constitute only 1% to 2% of all vascular neurologic pathologies. In this article, the following vascular pathologies of the spine are described: spinal arterial infarcts, spinal cavernomas, and arteriovenous malformations (including perimedullary fistulae and glomerular arterivenous malformations), and spinal dural arteriovenous fistulae. This article gives an overview about their imaging features on MRI, MR angiography, and digital subtraction angiography. Clinical differential diagnoses, the neurologic symptomatology, and the potential therapeutic approaches of these diseases, which might vary depending on the underlying pathologic condition, are given.     

Preoperative evaluation of spinal vascular malformation by MR angiography: how reliable is the technique: case report and review of literature

Contrast-enhanced MR angiography (MRA) has been increasingly used in the evaluation of spinal vascular malformations. Even though MR spinal angiography has several advantages over catheter spinal angiography (DSA), however, spinal DSA must never be omitted before operation, even if the vascular malformation is nicely demonstrated by MR angiography. We report a case of spinal vascular malformation in which MR angiography provided great images which almost convinced everyone about the type and site of malformation/fistula. The images were so convincing that it was almost decided to skip catheter based angiography, citing reason of disadvantages of catheter based angiography over MR angiography. However, spinal DSA was luckily done which completely changed the type and site of malformation and helped in avoiding failed surgery. We conclude that even though catheter based spinal angiography has disadvantages over MRA, it should never be omitted from the diagnostic protocol.     

Brain Hemangiomas of Infancy

Vascular birthmarks of the skin can be associated with hemangiomas or arteriovenous malformations of different organs. Computed tomography and magnetic resonance imaging (MRI) combined with angiography are the diagnostic tools used in children, adolescents, and adults to depict vascular malformation or bleeding. In infants, ultrasound examination through the anterior fontanelle is possible. This report describes 2 mature infants with brain hemangiomas primarily detected by real-time sonography: In both the brain lesions were associated with cutaneous and other vascular birthmarks. MRI was used to verify the brain hemangiomas. Subsequent follow-up evaluations during therapy were easily performed by ultrasonography.     

Dynamic MRA in the evaluation of intracranial vascular diseases

Conventional catheter angiography (CCA) remains the gold standard for the evaluation of most intracranial vascular malformations. MRA techniques such as Time of Flight, Phase Contrast or 3D contrast-enhanced MRA, provide anatomic evaluation but without hemodynamic information. Recently developed, dynamic MRA is based on dynamic acquisition of images and image subtraction; these two principal characteristics produce images comparable to those obtained by CCA. The purpose of this review is to explain the principles, advantages and drawbacks of this technique in the evaluation of arteriovenous malformations, arteriovenous fistulas, aneurysms and venous thrombosis.     

Spontaneous resolution of an idiopathic cervical direct vertebral arteriovenous fistula after partial coil embolization in a patient presenting with myeloradiculopathy

A 53-year old female presented with paresis of the left upper extremity. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) disclosed a single high-flow vertebral arteriovenous fistula (AVF) with vertebral artery (VA) transection. The AVF was also fed by steal flow from the contralateral VA. The left posterior inferior cerebellar artery (PICA) branched just distal to the fistula. The fistula drained into the neighboring paravertebral veins and refluxed into the intradural venous systems. The dilated drainers compressed the spinal cord. Embolization was attempted at the drainer just behind the fistula orifice using platinum coils. The fistula was still fed slightly by right VA after the embolization, but spontaneous complete obliteration was achieved after one week. The clinical symptoms and signs disappeared. Although, detachable balloon embolization is the quickest and most effective procedure to obliterate a fistula, stepwise embolization using GDC can be considered, and may avoid the normal pressure perfusion break-through phenomenon. Spontaneous obliteration of the fistula after partial embolization in our case may result from intravenous embolization just behind the fistula orifice. It may therefore be a useful approach to the embolization of an AVF to begin the embolization at the venous side of the fistula.     

Neuroimaging features and pathology of mixed glioblastoma-AVM complex: A case report

This report is of a rare case of glioblastoma coexisting with an arteriovenous malformation in a 65-year-old man. Multimodal magnetic resonance imaging (MRI) performed at 3T revealed a necrotic and cystic lesion in the left hemisphere; morphological and metabolic findings were consistent with an infiltrating high-grade glioma, but the presence of dark vessel-like signals on T2* and susceptibility-weighted imaging (SWI) suggested the coexistence of a vascular malformation. The arteriovenous malformation was confirmed by MR angiography and cerebral angiography. The patient was operated on, and histological examination revealed atypical cells characteristic of glioblastoma multiforme and, in the same area, arteriovenous malformation. The possible role of angiogenic factors in this case is also addressed.     

Comparisons of DSA and MR angiography with digital subtraction angiography in 151 patients with subacute spontaneous intracerebral hemorrhage

To exclude underlying vascular abnormalities in patients with spontaneous intracerebral hemorrhage, the traditional paradigm requires investigation using digital subtraction angiography (DSA) in both the acute and subacute phases. We investigated whether MRI and magnetic resonance angiography (MRA), in the subacute stage of intracerebral hematoma, had high positive predictive values (PPV) and negative predictive values (NPV) in screening for vascular abnormality in the routine clinical setting. In a regional neurosurgical center in Hong Kong, we retrospectively reviewed 151 patients investigated with both MRI and DSA for underlying structural vascular abnormalities during the subacute phase. Sensitivity, specificity, and intermodality agreement were assessed. A total of 70/151 (46%) vascular lesions accountable for the hemorrhage were found. Patients with vascular abnormalities tended to be younger (mean age ± standard deviation [SD], 33 ± 15 years), less likely to be hypertensive (6.3%), and the lesion was more likely to be accompanied by intraventricular hemorrhage (22%). In terms of cerebral arteriovenous malformation and dural arteriovenous fistulas, MRI/MRA had a PPV of 0.98 and a NPV of 1.00. We concluded that MRI/MRA was able to detect most structural vascular abnormalities in the subacute phase in most patients and, thus, its use is recommended as the screening test.     

A case of cervical dural arteriovenous malformation showing the dumbbell type growth]

A rare case with cervical dural arteriovenous malformation was reported. A 46-year-old female was admitted to our hospital because of progressive gait disturbance. Neurological examination revealed muscle weakness and sensory disturbance in the upper and lower limbs. Cervical X-ray showed destruction of the C5 vertebral body an enlarged C5-6 intervertebral foramen. Cervical CT showed a dumbbell type lesion resembling spinal tumor. On MRI, a signal area was observed posterior to the C5 spine on T1 and T2 weighted images. Myelography showed worm-like appearance. Selective spinal angiography revealed dural arteriovenous malformation fed by bilateral radicular arteries, thyrocervical and costocervical trunks. The authors emphasize significance of dural arteriovenous malformation as a cause of tumor-like lesions.     

Computed tomography (CT) angiography with multidetector row helical CT for detecting spinal dural arteriovenous fistula]

A 59-year-old man had have dysuria and left thigh pain for 2 months. He also experienced transient weakness of both legs twice, which recovered within a few hours. One month ago, he had bowel disturbance. Lumbar MRI showed a swelling of the conus medullaris. Enlarged and tortuous vessels behind the spinal cord on lumbar MRA and CT myelography was demonstrated. Though angiogram through intercostal and lumbar arteries revealed no abnormal findings, CT angiography with multidetector row helical CT (MDCT) showed abnormal vessel in intradural space at sacral level. Angiogram through left internal iliac artery revealed abnormal vessel fed by branches of the lateral sacral artery at the early arterial phase. Thus, diagnosis of dural arteriovenous fistula (DAVF) at the left S1 level was confirmed. It was indicated that CT angiography with MDCT was useful in detecting spinal DAVF especially at sacral level.     

Acute spinal cord infarction: vascular steal in arteriovenous malformation.

Central nervous system arteriovenous malformations typically present with chronic neurologic impairment. An 8-year-old boy presented with acute spinal cord infarction associated with a spinal arteriovenous malformation. Vascular steal phenomenon suggested by spinal angiography happens to underly the pathogenic mechanism.     

Spinal extradural arteriovenous fistulas with retrograde intradural venous drainage: Diagnostic features in digital subtraction angiography and time-resolved magnetic resonance angiography

Spinal extradural arteriovenous fistulas (AVFs) may be more difficult to prospectively identify than dural AVFs because they are less common than dural AVFs. The primary purpose was to further characterize the diagnostic imaging of spinal extradural AVFs with intradural retrograde venous drainage. The magnetic resonance (MR) imaging and angiographic results of 23 patients with suspected spinal dural AVFs were analyzed in order to distinguish dural and extradural AVFs. The diagnostic accuracy of MR angiography was retrospectively compared between dural and extradural AVFs. All 23 patients showed high intensity in the spinal cord on T2-weighted MR images. Eighteen out of 23 patients were diagnosed with dural AVFs, while the remaining 5 were diagnosed with extradural AVFs by angiography. Extradural AVFs were fed by a branch of the segmental artery to the vertebral body, characterized by a fistula located in the ventral extradural space, and drained retrogradely via an epidural venous pouch into intradural veins. The segmental artery was localized within 1 vertebral level using MRA in 12 out of 18 patients (67%) with dural AVFs and in 1 out of 5 patients (20%) with extradural AVFs (p = 0.09). The reasons behind the lower accuracy was mainly the image misinterpretation. Congestion of the spinal cord in spinal extradural AVFs with intradural retrograde venous drainage was similar to that in dural AVFs, whereas its angioarchitecture differed from that of dural AVFs. A clearer understanding of the imaging features of extradural AVFs is important for improving the diagnostic accuracy and clarifying treatment targets.     

Functional and topographical considerations in the surgical management of cerebral vascular malformations

Cerebral vascular malformations with potential surgical consequences mainly consist of arteriovenous malformations (AVM) and cavernous malformations. The standard preoperative workup of these lesions includes basic neuroradiological investigation, such as computed tomography, magnetic resonance imaging (MRI), and magnetic resonance angiography, and conventional angiography, to assess the exact neurotopographical relationships of the nidus, arterial feeding pedicles, and venous drainage. In cases where lesions are located near or within eloquent areas, precise documentation of the anatomy can be obtained using various functional tests including functional MRI, activated positron emission tomoqraphy, and magnetoencephalography (MEG), which may then be integrated into a neuronavigational system allowing for selective, image-guided surgery, thus potentially reducing surgical morbidity. Preoperative embolization may in certain cases improve the surqical excision by reducinq blood flow throuqh the AVM. Cavernous malformations may also be removed with minimally invasive and highly selective techniques.     

Spinal intradural arteriovenous fistula with unusual presentation: case report and literature review

Object  The authors present their experience with a Cervical Spinal intradural arteriovenous fistula (AVF) with unusual clinical presentation in a pediatric patient and review of the international literature on this condition. Methods  A 5-year-old girl was found by her parents in bed screaming and talking inappropriately. Later on, she calmed down and complained of occipital, back and neck pain. She was admitted to the CHEO-ICU for work up and observation. The admission head CT scan showed evidence of SAH. Brain MRI showed blood in the third and fourth ventricles. MRA showed a C2 vascular lesion consistent with spinal cord AVF. Conventional angiogram was done which confirmed the AVF fed by a posterior medullary artery originating from the left vertebral artery. An attempt to embolize the AVF with glue was made but proved to be unsuccessful. On December 4, 2002, direct surgery on the AVF was performed, through a midline posterior approach (C2–C3 laminectomies) and microsurgical technique a complete excision of the intradural extramedullary AVF was achieved. The postoperative course was uneventful and the patient remained neurologically intact. Post-operative angiography confirmed complete excision of the AVF. Conclusion  The rarity of a pediatric spinal AVF presenting with spontaneous Intracranial SAH, makes this case very peculiar. This is the first reported pediatric case of cervical cord AVF with this clinical presentation found in the literature.     

Mobility of Intradural Extramedullary Schwannoma at Spine : Report of Three Cases with Literature Review

Although very rare, a few cases of intradural extramedullary (IDEM) spinal tumor migration have been reported since Tomimatsu first reported a mobile schwannoma of the cervical cord in 1974. Schwannoma is a neurogenic tumor which originates from nerve sheath that it is relatively well-marginated tumor with little attachment or adhesion to surrounding tissue. Mobility of tumor in spinal canal sometimes can result in negative exploration at the expected area. We found three interesting cases in which different tumor locations observed in repeated magnetic resonance image (MRI) findings. All tumors were intradural and extramedullary schwannoma. We reviewed the literature about moving tumor in the spine through PUBMED search.     

Spontaneous Intracranial Arterial Dissection and Subarachnoid Hemorrhage

Spontaneous intracranial arterial dissection may cause subarachnoid or rarely intraventricular hemorrhage. The clinical presentation may resemble aneurysmal rupture. Experience using magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) in establishing the diagnosis of intracranial arterial dissection has been limited. We report three patients with intracranial arterial dissection and describe the correlation between findings on conventional angiography and MRI or MRA.