Deep arteriovenous malformations of the basal ganglia and thalamus: natural history

2003 ◽  
Vol 98 (4) ◽  
pp. 747-750 ◽  
Author(s):  
Ian G. Fleetwood ◽  
Mary L. Marcellus ◽  
Richard P. Levy ◽  
Michael P. Marks ◽  
Gary K. Steinberg

Object. Patients with arteriovenous malformations (AVMs) in a deep location and with deep venous drainage are thought to be at higher risk for hemorrhage than those with AVMs in other locations. Despite this, the natural history of AVMs of the basal ganglia and thalamus has not been well studied. Methods. The authors retrospectively evaluated a cohort of 96 patients with AVMs in the basal ganglia and thalamus with respect to the tendency of these lesions to hemorrhage between the time of detection and their eventual successful management. The 96 patients studied had a mean age of 22.7 years at diagnosis, and 51% were male. Intracranial hemorrhage (ICH) was the event leading to clinical detection in 69 patients (71.9%), and 85.5% of these patients were left with hemiparesis. After diagnosis, 25 patients bled a total of 49 times. The cumulative clinical follow up after detection but before surgical management was 500.2 patient-years. The risk of hemorrhage after detection of an AVM of the basal ganglia or thalamus was 9.8% per patient-year. Conclusions. The rate of ICH in patients with AVMs of the basal ganglia or thalamus (9.8%/year) is much higher than the rate in patients with AVMs in other locations (2–4%/year). The risk of incurring a neurological deficit with each hemorrhagic event is high. Treatment of these patients at specialized centers is recommended to prevent neurological injury from a spontaneous ICH.

1991 ◽  
Vol 75 (5) ◽  
pp. 715-722 ◽  
Author(s):  
Timothy B. Garner ◽  
O. Del Curling ◽  
David L. Kelly ◽  
D. Wayne Laster

✓ Cerebral venous angiomas are congenital anomalies of the intracranial venous drainage. Many believe that they are associated with a high risk of hemorrhage and neurological dysfunction, but newer neurodiagnostic imaging techniques are showing not only that they are more common than previously known but also that many have no associated symptoms. In this retrospective study, the natural history of venous angiomas was examined in 100 patients (48 males and 52 females) with radiographically identifiable lesions treated over a 14-year period. Information on the natural history of the lesion was obtained from clinical records and follow-up data. Imaging studies included angiography, computerized tomography, and magnetic resonance imaging. Angioma locations were classified as frontal (42 cases), parietal (24 cases), occipital (4 cases), temporal (2 cases), basal or ventricular (11 cases), cerebellar (14 cases), or brain stem (3 cases); 47 lesions were on the left side. Headache as a presenting symptom was common (36 patients) and often led to other radiographic studies, but this appeared to be related to the vascular lesion in only four patients. Other possibly related complications were hemorrhage in one patient, seizures in five, and transient focal deficits in eight. Fifteen patients had no neurological signs or symptoms. The mean patient age at last contact was 45.3 years (range 3 to 94 years). All patients have been managed without surgery. It is concluded that significant complications secondary to venous angiomas are infrequent and that surgical resection of these lesions and of surrounding brain is rarely indicated.


2008 ◽  
Vol 63 (suppl_1) ◽  
pp. ONS63-ONS68 ◽  
Author(s):  
Edward A.M. Duckworth ◽  
Bradley Gross ◽  
H. Hunt Batjer

Abstract Deep Arteriovenous Malformations of the basal ganglia and thalamus have an aggressive natural history and present a therapeutic challenge. More often than not, these lesions are deemed “inoperable” and are treated expectantly or with stereotactic radiosurgery. In some cases, clinical details combined with an opportune route of access dictate surgical resection. History of hemorrhage, small lesion size, and deep venous drainage each add to the aggressive natural history of these malformations. Interestingly, these same factors can point toward surgery. We present a discussion of the microsurgical techniques involved in managing these lesions, with an emphasis on situations that allow these lesions to be approached surgically.


1985 ◽  
Vol 62 (3) ◽  
pp. 321-323 ◽  
Author(s):  
John A. Jane ◽  
Neal F. Kassell ◽  
James C. Torner ◽  
H. Richard Winn

✓ The authors summarize the findings of previous studies relating to the natural history of aneurysms and arteriovenous malformations (AVM's). Ruptured aneurysms have their highest rate of rebleeding on Day 1, and at least 50% will rebleed during the 6 months after the first hemorrhage. Thereafter, the rate drops to at least 3% a year. This is the same rate as seen in anterior and posterior communicating artery aneurysms treated by anterior cerebral artery clipping and carotid ligation; these operations provide immediate protection but do not result in long-term diminution of the risk of rebleeding. Patients with unruptured incidental and unruptured multiple aneurysms rebleed at a rate of 1% per year, as do patients with subarachnoid hemorrhage of unknown etiology. The risk of rebleeding for AVM's is 3% a year.


1992 ◽  
Vol 76 (2) ◽  
pp. 239-243 ◽  
Author(s):  
Yoshio Miyasaka ◽  
Kenzoh Yada ◽  
Takashi Ohwada ◽  
Takao Kitahara ◽  
Akira Kurata ◽  
...  

✓ The authors studied the venous drainage system and its impairment in relation to risk of hemorrhage in 108 cases of supratentorial arteriovenous malformation (AVM). The proportion of AVM's undergoing hemorrhage (hemorrhagic rate) was calculated in relation to: 1) the number of draining veins (one, two, or three or more); 2) the presence or absence of impairment in venous drainage (severe stenosis or occlusion in draining veins); and 3) the location of draining veins (deep venous drainage alone, superficial venous drainage alone, or a combination of the two). Statistical analysis demonstrated that AVM's with the following characteristics had a high risk of hemorrhage: 1) one draining vein (hemorrhagic rate 89% in 54 patients); 2) severely impaired venous drainage (hemorrhagic rate 94% in 18 patients); and 3) deep venous drainage alone (hemorrhagic rate 94% in 32 patients). The present study suggests that the venous drainage system of AVM's is significantly associated with the risk of hemorrhage of these lesions. Therefore, careful preoperative angiographic evaluation of the venous drainage system is mandatory for decision making in the management of patients with AVM's.


2003 ◽  
Vol 98 (1) ◽  
pp. 3-7 ◽  
Author(s):  
Patrick P. Han ◽  
Francisco A. Ponce ◽  
Robert F. Spetzler

Object. In this study the authors quantified a subgroup of patients with Spetzler—Martin Grades IV and V arteriovenous malformations (AVMs) recommended for complete, partial, or no treatment, and calculated the retrospective hemorrhage rate for these lesions. Methods. Between July 1997 and May 2000, 73 consecutive patients with Grades IV and V AVMs were evaluated prospectively by the cerebrovascular team at Barrow Neurological Institute. Treatment recommendations given to the patients or referring physicians were classified as complete treatment, partial treatment, and no treatment. Retrospectively, the hemorrhage rates associated with these treatment groups were also calculated. In the prospective portion of the study (the intention-to-treat analysis), no treatment of the AVM, was recommended for 55 patients (75%) and partial treatment was recommended for seven patients (10%). Aneurysms associated with an AVM were obliterated by surgical or endovascular treatment in seven patients (10%), and complete surgical removal was recommended for four patients (5%). The overall hemorrhage rate for Grades IV and V AVMs was 1.5% per year. The annual risk of hemorrhage was 10.4% among patients who previously had received incomplete treatment, compared with patients without previous treatment. Conclusions. The hemorrhage risk of 1.5% per year, which was associated with Grades IV and V AVMs appears to be lower than that reported for Grades I through III AVMs. The authors recommend that no treatment be given for most Grades IV and V AVMs. No evidence indicates that partial treatment of an AVM reduces a patient's risk of hemorrhage. In fact, partial treatment may worsen the natural history of an AVM. The authors do not support palliative treatment of AVMs, except in the specific circumstances of arterial or intranidal aneurysms or progressive neurological deficits related to vascular steal. Complete treatment is warranted for patients with progressive neurological deficits caused by hemorrhage of the AVM. This selection process plays a significant role in the relatively low combined morbidity and mortality rates for Grade IV and Grade V AVMs (17 and 22%, respectively) reported by the cerebrovascular group in both retrospective and prospective studies.


1974 ◽  
Vol 41 (1) ◽  
pp. 63-70 ◽  
Author(s):  
Julian L. Robinson ◽  
Christopher S. Hall ◽  
Carol B. Sedzimir

✓ The authors report a retrospective study of 146 patients to assess the extent to which aneurysms, arteriovenous malformations, and pregnancy interact. The natural history of the lesions was modified if the women became pregnant. Clinical syndromes, diagnosis, neurosurgical and obstetrical management, and treatment are discussed.


1988 ◽  
Vol 68 (3) ◽  
pp. 352-357 ◽  
Author(s):  
Robert D. Brown ◽  
David O. Wiebers ◽  
Glenn Forbes ◽  
W. Michael O'Fallon ◽  
David G. Piepgras ◽  
...  

✓ The authors conducted a long-term follow-up study of 168 patients to define the natural history of clinically unruptured intracranial arteriovenous malformations (AVM's). Charts of patients seen at the Mayo Clinic between 1974 and 1985 were reviewed. Follow-up information was obtained on 166 patients until death, surgery, or other intervention, or for at least 4 years after diagnosis (mean follow-up time 8.2 years). All available cerebral arteriograms and computerized tomography scans of the head were reviewed. Intracranial hemorrhage occurred in 31 patients (18%), due to AVM rupture in 29 and secondary to AVM or aneurysm rupture in two. The mean risk of hemorrhage was 2.2% per year, and the observed annual rates of hemorrhage increased over time. The risk of death from rupture was 29%, and 23% of survivors had significant long-term morbidity. The size of the AVM and the presence of treated or untreated hypertension were of no value in predicting rupture.


2002 ◽  
Vol 97 (1) ◽  
pp. 113-117 ◽  
Author(s):  
Kevin L. Stevenson ◽  
Matthew Wetzel ◽  
Ian F. Pollack

✓ Delayed complications associated with sublaminar and interspinous wiring in the pediatric cervical spine are rare. The authors present a case of delayed complication in which a cervical fusion wire migrated into the cerebellum, causing subsequent cerebellar abscess 2 years after posterior cervical arthrodesis. A craniotomy was required to remove the wire and drain the abscess. Despite their history of safety and successful fusion, procedures involving sublaminar and interspinous wiring carry a risk of neurological injury secondary to wire migration. A thorough neuroimaging evaluation is required in patients who have undergone fusion and who have neurological complaints to detect late instrumentation-related sequelae.


2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 34-37 ◽  
Author(s):  
Masahiro Izawa ◽  
Motohiro Hayashi ◽  
Mikhail Chernov ◽  
Koutarou Nakaya ◽  
Taku Ochiai ◽  
...  

Object. The authors analyzed of the long-term complications that occur 2 or more years after gamma knife surgery (GKS) for intracranial arteriovenous malformations (AVMs). Methods. Patients with previously untreated intracranial AVMs that were managed by GKS and followed for at least 2 years after treatment were selected for analysis (237 cases). Complete AVM obliteration was attained in 130 cases (54.9%), and incomplete obliteration in 107 cases (45.1%). Long-term complications were observed in 22 patients (9.3%). These complications included hemorrhage (eight cases), delayed cyst formation (eight cases), increase of seizure frequency (four cases), and middle cerebral artery stenosis and increased white matter signal intensity on T2-weighted magnetic resonance imaging (one case of each). The long-term complications were associated with larger nidus volume (p < 0.001) and a lobar location of the AVM (p < 0.01). Delayed hemorrhage was associated only with incomplete obliteration of the nidus (p < 0.05). Partial obliteration conveyed no benefit. Delayed cyst formation was associated with a higher maximal GKS dose (p < 0.001), larger nidus volume (p < 0.001), complete nidus obliteration (p < 0.01), and a lobar location of the AVM (p < 0.05). Conclusions. Incomplete obliteration of the nidus is the most important factor associated with delayed hemorrhagic complications. Partial obliteration does not seem to reduce the risk of hemorrhage. Complete obliteration can be complicated by delayed cyst formation, especially if high maximal treatment doses have been administered.


2003 ◽  
Vol 98 (1) ◽  
pp. 82-94 ◽  
Author(s):  
John E. Wanebo ◽  
Russell R. Lonser ◽  
Gladys M. Glenn ◽  
Edward H. Oldfield

Object. The goals of this study were to define the natural history and growth pattern of hemangioblastomas of the central nervous system (CNS) that are associated with von Hippel—Lindau (VHL) disease and to correlate features of hemangioblastomas that are associated with the development of symptoms and the need for treatment. Methods. The authors reviewed serial magnetic resonance images and clinical histories of 160 consecutive patients with VHL disease who harbored CNS hemangioblastomas and serially measured the volumes of tumors and associated cysts. Six hundred fifty-five hemangioblastomas were identified in the cerebellum (250 tumors), brainstem (64 tumors, all of which were located in the posterior medulla oblongata), spinal cord (331 tumors, 96% of which were located in the posterior half of spinal cord), and the supratentorial brain (10 tumors). The symptoms were related to a mass effect. A serial increase in hemangioblastoma size was observed in cerebellar, brainstem, and spinal cord tumors as patients progressed from being asymptomatic to symptomatic and requiring surgery (p < 0.0001). Twenty-one (72%) of 29 symptom-producing cerebellar tumors had an associated cyst, whereas only 28 (13%) of 221 nonsymptomatic cerebellar tumors had tumor-associated cysts (p < 0.0001). Nine (75%) of 12 symptomatic brainstem tumors had associated cysts, compared with only four (8%) of 52 nonsymptomatic brainstem lesions (p < 0.0001). By the time the symptoms appeared and surgery was required, the cyst was larger than the causative tumor; cerebellar and brainstem cysts measured 34 and 19 times the size of their associated tumors at surgery, respectively. Ninety-five percent of symptom-producing spinal hemangioblastomas were associated with syringomyelia. The clinical circumstance was dynamic. Among the 88 patients who had undergone serial imaging for 6 months or longer (median 32 months), 164 (44%) of 373 hemangioblastomas and 37 (67%) of 55 tumor-associated cysts enlarged. No tumors or cysts spontaneously diminished in size. Symptomatic cerebellar and brainstem tumors grew at rates six and nine times greater, respectively, than asymptomatic tumors in the same regions. Cysts enlarged seven (cerebellum) and 15 (brainstem) times faster than the hemangioblastomas causing them. Hemangioblastomas frequently demonstrated a pattern of growth in which they would enlarge for a period of time (growth phase) and then stabilize in a period of arrested growth (quiescent phase). Of 69 patients with documented tumor growth, 18 (26%) harbored tumors with at least two growth phases. Of 160 patients with hemangioblastomas, 34 patients (median follow up 51 months) were found to have 115 new hemangioblastomas and 15 patients new tumor-associated cysts. Conclusions. In this study the authors define the natural history of CNS hemangioblastomas associated with VHL disease. Not only were cysts commonly associated with cerebellar, brainstem, and spinal hemangioblastomas, the pace of enlargement was much faster for cysts than for hemangioblastomas. By the time symptoms appeared, the majority of mass effect—producing symptoms derived from the cyst, rather than from the tumor causing the cyst. These tumors often have multiple periods of tumor growth separated by periods of arrested growth, and many untreated tumors may remain the same size for several years. These characteristics must be considered when determining the optimal timing of screening for individual patients and for evaluating the timing and results of treatment.


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