The natural history of intracranial venous angiomas

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.

1995 ◽  
Vol 83 (2) ◽  
pp. 222-224 ◽  
Author(s):  
William C. Olivero ◽  
J. Richard Lister ◽  
Patrick W. Elwood

✓ Little is known about the natural history and growth rate of asymptomatic meningiomas. To better delineate this problem, the authors reviewed the clinical records and imaging studies of the last 60 patients diagnosed with asymptomatic meningiomas at their institution. There were 45 women and 15 men, whose ages ranged from 38 to 84 years, with a mean age of 66 years. The most common tumor location was convexity (25 patients), but virtually all locations were represented. Three patients were lost to follow up. The average clinical follow-up review of the remaining 57 patients was 32 months (range 6 months to 15 years). None of the patients became symptomatic from an enlarging tumor during their follow-up period. Typically, once a meningioma was diagnosed, follow-up scans were obtained at 3 months, 9 months, and then yearly or every other year thereafter. Forty-five patients underwent follow-up scans, with comparison of tumor size to that found on the initial scan, over a period ranging from 3 months to 15 years. Thirty-five patients have shown no growth in their tumor size, with an average imaging follow up of 29 months (range 3–72 months). Ten patients have shown tumor growth calculated as an increase in the maximum diameter of the tumor. This growth ranged from 0.2 cm over 180 months to 1 cm over 12 months, with an average of 0.24 cm per year. Average imaging follow up for these patients was 47 months (range 6 months to 15 years). The authors conclude that patients with asymptomatic meningiomas need close clinical and radiological follow up to rule out other disease processes and to rule out rapidly enlarging tumors. Although the average follow-up time was short, the vast majority of these tumors appeared to show minimal or no growth over periods of time measured in years. With modern noninvasive imaging techniques, these tumors can be safely observed until they enlarge significantly or become symptomatic.


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.


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.


1990 ◽  
Vol 73 (4) ◽  
pp. 560-564 ◽  
Author(s):  
Daniele Rigamonti ◽  
Robert F. Spetzler ◽  
Marjorie Medina ◽  
Karen Rigamonti ◽  
David S. Geckle ◽  
...  

✓ Although cerebral venous malformations have been reported to cause epilepsy, progressive neurological deficits, and hemorrhage, their clinical significance remains controversial. In an attempt to clarify the natural history of the lesion and suggest an appropriate management strategy, the authors review their experience with 30 patients. In four patients with cerebellar venous angioma, an acute episode of ataxia was documented. The coexistence of a cavernous malformation was pathologically confirmed in the two patients who underwent surgery for bleeding presumed caused by the venous angioma. Infarction was shown in two patients and a tumor in two others. Follow-up periods ranged between 18 and 104 months, with only five patients symptomatic at the time of this report. Rebleeding had not occurred, nor had acute episodes of neurological dysfunction been documented. This clinical experience suggests that a venous malformation is frequently associated with other, more symptomatic conditions and is often erroneously identified as the source of the symptoms. Because the nature of the relationship between the venous malformation and the allied conditions remains ambiguous, it is recommended that patients harboring a “symptomatic” venous malformation undergo high-field magnetic resonance imaging to rule out underlying pathology, and that any such pathology be treated independently of the venous malformation.


2001 ◽  
Vol 95 (2) ◽  
pp. 256-262 ◽  
Author(s):  
Hiroshi Nakaguchi ◽  
Takeo Tanishima ◽  
Norio Yoshimasu

Object. Factors affecting the postoperative recurrence of chronic subdural hematomas (CSDHs) have not been sufficiently investigated. The authors have attempted to determine features of CSDHs that are associated with a high or low recurrence rate on the basis of the natural history of these lesions and their intracranial extension. Methods. One hundred six patients (82 men and 24 women) harboring 126 CSDHs who were treated at Tokyo Kosei Nenkin Hospital between January 1989 and April 1998 were studied. Types of CSDHs were classified according to hematoma density and internal architecture, and the intracranial extension of the hematomas were investigated. The postoperative recurrence rate was calculated for each factor. Based on the internal architecture and density of each hematoma, the CSDHs were classified into four types, including homogeneous, laminar, separated, and trabecular types. The recurrence rate associated with the separated type was high, whereas that associated with the trabecular type was low. Chronic subdural hematomas are believed to develop initially as the homogeneous type, after which they sometimes progress to the laminar type. A mature CSDH is represented by the separated stage and the hematoma eventually passes through the trabecular stage during absorption. Based on the intracranial extension of each hematoma, CSDHs were classified into three types, including convexity, cranial base, and interhemispheric types. The recurrence rate of cranial base CSDHs was high and that of convexity CSDHs was low. Conclusions. Classification of CSDHs according to the internal architecture and intracranial extension may be useful for predicting the risk of postoperative recurrence.


2000 ◽  
Vol 93 (6) ◽  
pp. 976-980 ◽  
Author(s):  
Eiichi Kobayashi ◽  
Naokatsu Saeki ◽  
Hiromichi Oishi ◽  
Shinji Hirai ◽  
Akira Yamaura

Object. The purpose of this study was to delineate the long-term natural history of hemorrhagic moyamoya disease (MMD).Methods. A retrospective review was conducted among 42 patients suffering from hemorrhagic MMD who had been treated conservatively without bypass surgery. The group included four patients who had undergone indirect bypass surgery after an episode of rebleeding. The follow-up period averaged 80.6 months. The clinical features of the first bleeding episode and repeated bleeding episodes were analyzed to determine the risk factors of rebleeding and poor outcome.Intraventricular hemorrhage with or without intracerebral hemorrhage was a dominant finding on computerized tomography scans during the first bleeding episode in 29 cases (69%). During the follow-up period, 14 patients experienced a second episode of bleeding, which occurred 10 years or longer after the original hemorrhage in five cases (35.7%). The annual rebleeding rate was 7.09%/person/year. The second bleeding episode was characterized by a change in which hemisphere bleeding occurred in three cases (21.4%) and by the type of bleeding in seven cases (50%). After rebleeding the rate of good recovery fell from 45.5% to 21.4% and the mortality rate rose from 6.8% to 28.6%. Rebleeding and patient age were statistically significant risk factors of poor outcome. All four patients in whom there was indirect revascularization after the second bleeding episode experienced a repeated bleeding episode within 8 years.Conclusions. The occurrence of rebleeding a long time after the first hemorrhagic episode was not uncommon. Furthermore, the change in which hemisphere and the type of bleeding that occurred after the first episode suggested the difficulty encountered in the prevention of repeated hemorrhage.


1987 ◽  
Vol 66 (1) ◽  
pp. 30-34 ◽  
Author(s):  
Isaac Feuerberg ◽  
Christer Lindquist ◽  
Melker Lindqvist ◽  
Ladislau Steiner

✓ In a series of 715 patients operated on by microsurgical techniques for intracranial saccular aneurysms between 1970 and 1980, part of the aneurysmal sac was not obliterated in 28 aneurysms in 27 patients (3.8% of 715 cases). Clinical follow-up evaluation for 8 years (range 4 to 13 years) and angiographic follow-up studies for 6 years (range 2 to 10 years) in these 27 cases revealed that one aneurysm rest increased in size and bled twice, five were spontaneously obliterated, two decreased in size, 13 remained unchanged, and in seven cases no late follow-up angiography was performed. The incidence of rebleeding from an aneurysm rest was 3.7% of the 27 in whom the sac was not obliterated and 0.14% of all 715 patients who were operated on.


1993 ◽  
Vol 79 (1) ◽  
pp. 16-27 ◽  
Author(s):  
Francis H. Tomlinson ◽  
Daniel A. Rüfenacht ◽  
Thoralf M. Sundt ◽  
Douglas A. Nichols ◽  
Nicolee C. Fode

✓ Arteriovenous (AV) fistulas of cerebral and spinal arteries are characterized angiographically by an immediate AV transition without a capillary bed or “nidus” as occurs in AV malformations (AVM's). The clinical presentation, morphology, radiology, and treatment of 12 patients with cerebral AV fistulas and of 12 patients with spinal AV fistulas are reviewed. In the patients with cerebral lesions, headache and seizure disorders were the most common presentations followed by subarachnoid hemorrhage, cardiac failure, progressive neurological dysfunction, and incidental detection on prenatal ultrasound study. In patients with spinal AV fistulas, weakness and sensory disturbance in the lower extremities were the most frequent clinical presentations followed by back pain, disturbances of micturition, and grand mal seizure. The etiology of the symptom complex produced by AV fistulas in each of these locations differed, with venous hypertension being important in spinal cord lesions. Of the patients with cerebral lesions, nine had a single AV fistula, one had two fistulas, and two had multiple fistulas. An AVM was observed in five patients with fistulas (two large, three small). Nine patients exhibited extramedullary AV fistulas of the spine, of whom eight had a single fistula and one had three fistulas; three patients had intramedullary spinal AV fistulas. An arterial aneurysm was found in association with two fistulas, one cerebral and one spinal. Venous ectasias or varices, frequently exhibiting mural calcification, were observed to be prominent in all AV fistulas involving cerebral arteries and in two involving spinal arteries. The location and size of the venous complexes reflected the diameter of the fistula. In addition to conventional imaging techniques (cerebral angiography, computerized tomography, and magnetic resonance (MR) imaging), MR angiography was a helpful adjunct in the evaluation of fistulas. Treatment strategies employed for AV fistulas in both locations included open surgical and endovascular procedures, frequently used in combination. A satisfactory outcome was observed in all patients.


1977 ◽  
Vol 46 (6) ◽  
pp. 731-734 ◽  
Author(s):  
Duke S. Samson ◽  
Richard M. Hodosh ◽  
W. Kemp Clark

✓ The natural history of unruptured asymptomatic aneurysms is unclear. Because of this uncertainty regarding risk of ultimate enlargement and/or hemorrhage, and in view of the significant mortality and morbidity traditionally involved in aneurysm surgery, clinicians have varied in their advocacy of surgical management of such lesions. Forty-nine consecutive patients harboring 52 such aneurysms were treated surgically over a 57-month period. There were no surgical deaths and morbidity was within acceptable limits. Patient population characteristics and surgical technique are discussed.


1987 ◽  
Vol 66 (4) ◽  
pp. 618-620 ◽  
Author(s):  
Steven A. Goldman ◽  
Samuel E. Gandy

✓ Primary intracerebral squamous cell carcinoma is a rare sequela of benign cerebral epidermoid cysts. A case of presumed malignant transformation in a lateral ventricular epidermoid cyst is described, in which squamous cell carcinoma arose 33 years after the surgical resection of a benign epidermoid cyst. Three years after diagnosis of malignancy, the patient remains functional with a slowly invasive tumor. This case provides a rare description of the natural history of forebrain squamous cell carcinoma, which would appear to be a more indolent tumor than previously realized.


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