Cavernous malformations of the brainstem: experience with 100 patients

1999 ◽  
Vol 90 (1) ◽  
pp. 50-58 ◽  
Author(s):  
Randall W. Porter ◽  
Paul W. Detwiler ◽  
Robert F. Spetzler ◽  
Michael T. Lawton ◽  
Jonathan J. Baskin ◽  
...  
Keyword(s):  
Natural History ◽  
Skull Base ◽  
Surgical Patients ◽  
Cavernous Malformations ◽  
Content Type ◽  
History Of ◽  
Lost To Follow Up ◽  
Pontomedullary Junction ◽  
Fine Print

Object. In this study the authors review surgical experience with cavernous malformations of the brainstem (CMBs) in an attempt to define more clearly the natural history, indications, and risks of surgical management of these lesions.Methods. The authors retrospectively reviewed the cases of 100 patients (38 males and 62 females; mean age 37 years) harboring 103 lesions at treated a single institution between 1984 and 1997. Clinical histories, radiographs, pathology records, and operative reports were evaluated. The brainstem lesions were distributed as follows: pons in 39 patients, medulla in 16, midbrain in 16, pontomesencephalic junction in 15, pontomedullary junction in 10, midbrain—hypothalamus/thalamus region in two patients, and more than two brainstem levels in five. The retrospective annual hemorrhage rate was most conservatively estimated at 5% per lesion per year. Standard skull base approaches were used to resect lesions in 86 of the 100 patients. Intraoperatively, all 86 patients were found to have a venous anomaly in association with the CMB. Follow up was available in 98% (84 of 86) of the surgical patients. Of these, 73 (87%) were the same or better after surgical intervention, eight (10%) were worse, and three (4%) died. Two surgical patients were lost to follow-up review. Incidences of permanent or severe morbidity occurred in 10 (12%) of the surgically treated patients. The average postoperative Glasgow Outcome Scale score for surgically treated patients was 4.5, with a mean follow-up period of 35 months.Conclusions. The natural history of CMBs is worse than that of cavernous malformations in other locations. These CMBs can be resected using skull base approaches, which should be considered in patients with symptomatic hemorrhage who harbor lesions that approach the pial surface. Venous anomalies are always associated with CMBs and must be preserved.

The natural history of cerebral cavernous malformations

1995 ◽  
Vol 83 (5) ◽  
pp. 820-824 ◽  
Author(s):  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
John R. W. Kestle
Keyword(s):  
Natural History ◽  
Management Strategies ◽  
Operative Management ◽  
Neurological Deficits ◽  
Cavernous Malformations ◽  
Content Type ◽  
Increased Risk ◽  
History Of ◽  
Fine Print

✓ To determine the natural history of brain cavernous malformations, the authors entered patients referred to their center into a prospective registry between 1987 and 1993. All patients underwent magnetic resonance imaging, which showed the typical appearance of this lesion, and conservative management was recommended in all. Patients or their referring physicians were contacted for follow-up data. The purpose of the study was to define the rate of symptomatic hemorrhage and to determine the outcome in those patients who had suffered seizures. Follow-up data were available for 122 patients with a mean age at entry of 37 years (range 4–82 years). The malformation was located in the brainstem in 43 cases (35%), the basal ganglia/thalamus in 20 (17%), and a hemispheric area in 59 (48%). Fifty percent of patients had never had a symptomatic hemorrhage, 41% had one bleed, 7% had two, and 2% had three. Seizures were reported in 23% of patients and headaches in 15%. Lesions were solitary in 80% of patients and multiple in 20%. The retrospective annual hemorrhage rate (61 bleeds/4550.6 patient-years of life) was 1.3%. The mean prospective follow-up period was 34 months. There were nine bleeds during this time, six with new neurological deficits. In patients without a prior bleed, the prospective annual rate of hemorrhage was 0.6%. In contrast, patients with prior hemorrhage had an annual bleed rate of 4.5% (p = 0.028). Patient sex (p = 0.97) or the presence of seizures (p = 0.11), headaches (p = 0.06), or solitary versus multiple lesions (p = 0.15) were not significant predictors of later hemorrhage. There was no difference in the rate of bleeds between brain locations. Four patients with seizures became seizurefree and four patients without seizures later developed seizures; only one patient developed intractable seizures. Fourteen patients (11%) underwent surgery (two after hemorrhage, five with seizures, and seven with progressive deficits), and five had radiosurgery. No patient died in the follow-up period. This study indicates that conservative versus operative management strategies may need to be redefined, especially in patients who present with hemorrhage and who appear to have a significantly increased risk of subsequent rehemorrhage.

Long-term natural history of hemorrhagic type moyamoya disease in 42 patients

2000 ◽  
Vol 93 (6) ◽  
pp. 976-980 ◽  
Author(s):  
Eiichi Kobayashi ◽  
Naokatsu Saeki ◽  
Hiromichi Oishi ◽  
Shinji Hirai ◽  
Akira Yamaura
Keyword(s):  
Risk Factors ◽  
Natural History ◽  
Moyamoya Disease ◽  
Bypass Surgery ◽  
Bleeding Episode ◽  
Content Type ◽  
History Of ◽  
Fine Print

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.

Natural history of postoperative aneurysm rests

1987 ◽  
Vol 66 (1) ◽  
pp. 30-34 ◽  
Author(s):  
Isaac Feuerberg ◽  
Christer Lindquist ◽  
Melker Lindqvist ◽  
Ladislau Steiner
Keyword(s):  
Natural History ◽  
Content Type ◽  
Follow Up Studies ◽  
History Of ◽  
Microsurgical Techniques ◽  
Fine Print ◽  
Saccular Aneurysms

✓ 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.

The natural history of familial cavernous malformations: results of an ongoing study

1994 ◽  
Vol 80 (3) ◽  
pp. 422-432 ◽  
Author(s):  
Joseph M. Zabramski ◽  
Thomas M. Wascher ◽  
Robert F. Spetzler ◽  
Blake Johnson ◽  
John Golfinos ◽  
...  
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Ongoing Study ◽  
Mr Images ◽  
Cavernous Malformations ◽  
Content Type ◽  
Imaging Characteristics ◽  
Familial Form ◽  
Fine Print

✓ Cavernous malformations are congenital abnormalities of the cerebral vessels that affect 0.5% to 0.7% of the population. They occur in two forms: a sporadic form characterized by isolated lesions, and a familial form characterized by multiple lesions with an autosomal dominant mode of inheritance. The management of patients with cavernous malformations, particularly those with the familial form of the disease, remains a challenge because little is known regarding the natural history. The authors report the results of an ongoing study in which six families afflicted by familial cavernous malformations have been prospectively followed with serial interviews, physical examinations, and magnetic resonance (MR) imaging at 6- to 12-month intervals. A total of 59 members of these six families were screened for protocol enrollment; 31 (53%) had MR evidence of familial cavernous malformations. Nineteen (61%) of these 31 patients were symptomatic, with seizures in 12 (39%), recurrent headaches in 16 (52%), focal sensory/motor deficits in three (10%), and visual field deficits in two (6%). Twenty-one of these 31 patients underwent at least two serial clinical and MR imaging examinations. A total of 128 individual cavernous malformations (mean 6.5 ± 3.8 lesions/patient) were identified and followed radiographically. During a mean follow-up period of 2.2 years (range 1 to 5.5 years), serial MR images demonstrated 17 new lesions in six (29%) of the 21 patients; 13 lesions (10%) showed changes in signal characteristics, and five lesions (3.9%) changed significantly in size. The incidence of symptomatic hemorrhage was 1.1% per lesion per year. The results of this study demonstrate that the familial form of cavernous malformations is a dynamic disease; serial MR images revealed changes in the number, size, and imaging characteristics of lesions consistent with acute or resolving hemorrhage. It is believed that the de novo development of new lesions in this disease has not been previously reported. These findings suggest that patients with familial cavernous malformations require careful follow-up monitoring, and that significant changes in neurological symptoms warrant repeat MR imaging. Surgery should be considered only for lesions that produce repetitive or progressive symptoms. Prophylactic resection of asymptomatic lesions does not appear to be indicated.

Cerebral cavernous malformations: natural history and prognosis after clinical deterioration with or without hemorrhage

1997 ◽  
Vol 87 (2) ◽  
pp. 190-197 ◽  
Author(s):  
Phillip J. Porter ◽  
Robert A. Willinsky ◽  
William Harper ◽  
M. Christopher Wallace
Keyword(s):  
Natural History ◽  
Cerebellar Nuclei ◽  
Neurological Deterioration ◽  
Focal Neurological Deficit ◽  
Cavernous Malformations ◽  
Content Type ◽  
Lesion Location ◽  
Neurological Event ◽  
Fine Print

✓ Despite recent studies of the natural history of cavernous malformations, there remains significant uncertainty concerning hemorrhage rates and the importance of lesion location. Controversy arises over varying definitions of “hemorrhage.” What is ultimately important to the patient is the occurrence of a neurological event, which may or may not be associated with radiologically documented hemorrhage, as well as the chance of recovery after such an event. The purpose of this study was to determine the rates of occurrence and sequelae of neurological events in 173 patients referred to our vascular malformation clinic with cavernous malformations. All patient data were entered into a database. The mean age at presentation for the 173 patients was 37.5 years. The lesion location was deep (brainstem, cerebellar nuclei, thalamus, or basal ganglia) in 64 patients (37%) and superficial in 109 (63%). Thirty-one patients (18%) had multiple lesions. Disease presentation was due to seizures in 62 patients (36%), hemorrhage in 44 (25%), focal neurological deficit without documented hemorrhage in 35 (20%), headache alone in 11 (6%), and incidental findings in 21 patients (12%). The results obtained in the 110 patients eligible for follow-up review were used to derive information on the rates of hemorrhage and neurological events. An interval event (neurological deterioration) required both symptoms and signs. The total mean follow-up period was 46 months, the majority (65%) of which was prospective. There were 18 interval events in 427 patient-years of follow-up review, for an overall annual event rate of 4.2%. Location was the most important factor for predicting interval event occurrence, with significantly higher rates for deeply located (10.6%/year) compared with superficially located lesions (0%/year) (p = 0.0001). Of patients suffering a neurological event, only 37% had complete resolution of their deficits. This largely prospective study indicates that deep cavernous malformations carry a worse prognosis than superficial lesions with respect to annual rates of neurological deterioration. The alarming rate of adverse clinical events occurring in patients with deep lesions is punctuated by the fact that less than one-half of them recover fully during long-term follow-up review.

scholarly journals Natural history of unruptured intracranial aneurysms: a long-term follow-up study

1993 ◽  
Vol 79 (2) ◽  
pp. 174-182 ◽  
Author(s):  
Seppo Juvela ◽  
Matti Porras ◽  
Olli Heiskanen
Keyword(s):  
Natural History ◽  
Unruptured Aneurysm ◽  
Content Type ◽  
Unruptured Aneurysms ◽  
Multiple Aneurysms ◽  
Follow Up Study ◽  
Risk Of Rupture ◽  
History Of ◽  
Fine Print

✓ To investigate the natural history of unruptured aneurysms and predictive risk factors determining subsequent rupture, the authors followed 142 patients with 181 unruptured aneurysms until death or subarachnoid hemorrhage intervened, or for at least 10 years after the unruptured aneurysm was diagnosed. Six patients had a symptomatic aneurysm, five had an incidentally discovered aneurysm, and 131 had multiple aneurysms, of which the ruptured lesion was clipped at the beginning of the follow-up study. The median follow-up time was 13.9 years (range 0.8 to 30.0 years). During 1944 patient-years of follow-up study there were 27 first episodes of hemorrhage from a previously unruptured aneurysm, giving an average annual rupture incidence of 1.4%. Fourteen of these bleeding episodes were fatal. The cumulative rate of bleeding was 10% at 10 years, 26% at 20 years, and 32% at 30 years after the diagnosis. The only predictor for the rupture was the size of the aneurysm (p = 0.036). However, in patients with multiple aneurysms (the main subgroup) the only variable that tended to predict rupture was the age of the patient: risk of rupture was inversely associated with age (p = 0.080). The median diameter of the aneurysms was 4 mm at the beginning of the follow-up period, both in those with and those without a later hemorrhage. During the angiographic monitoring period, a ruptured aneurysm significantly (p < 0.001) increased in size in 17 patients with hemorrhage but aneurysms did not increase significantly in 14 patients without hemorrhage. In addition, a new aneurysm was found in six of 31 patients. The authors conclude that an unruptured aneurysm should be operated on, irrespective of its size, if it is technically possible and the patient's age and concurrent diseases are not contraindications to surgery.

Brown tumors of the skull base

2003 ◽  
Vol 98 (2) ◽  
pp. 417-420 ◽  
Author(s):  
Mubarak Al-Gahtany ◽  
Michael Cusimano ◽  
William Singer ◽  
Juan Bilbao ◽  
Kalman Kovacs ◽  
...  
Keyword(s):  
Skull Base ◽  
Brown Tumor ◽  
Benign Condition ◽  
Content Type ◽  
Long Term Follow Up ◽  
History Of ◽  
Brown Tumors ◽  
Fine Print

✓ The natural history of brown tumor of the skull base, a rare and benign condition, has not been adequately documented, particularly after nonsurgical treatment and over a long follow-up period. The authors report a case of brown tumor of the ethmoidal sinus, documenting its clinical, neuroimaging, and pathological features with long-term follow-up results, and review previous reports on this disorder.

Natural history of asymptomatic colloid cysts of the third ventricle

1999 ◽  
Vol 91 (3) ◽  
pp. 364-369 ◽  
Author(s):  
Bruce E. Pollock ◽  
John Huston
Keyword(s):  
Natural History ◽  
Third Ventricle ◽  
Benign Tumors ◽  
Content Type ◽  
The Third ◽  
History Of ◽  
Colloid Cysts ◽  
The Mean ◽  
Fine Print

Object. To determine the natural history of colloid cysts of the third ventricle in patients in whom the cysts were incidentally discovered, the authors retrospectively reviewed cases observed during the modern neuroimaging era (1974–1998).Methods. During this 25-year interval, 162 patients with colloid cysts were examined and cared for at our center. Sixty-eight patients (42%) were thought to be asymptomatic with regard to their colloid cyst and observation with serial neuroimaging was recommended. The mean patient age was 57 years at the time of diagnosis (range 7–88 years) and the mean cyst size was 8 mm (range 4–18 mm). Computerized tomography scanning revealed a hyperdense cyst in 49 (84%) of 58 patients. Three patients were excluded from the study because they died of unrelated causes within 6 months of scanning and seven patients were lost to follow-up review. Clinical follow-up evaluation was available at a mean of 79 months (range 7–268 months) in the remaining 58 patients. The numbers of patients who participated in follow-up review at 2, 5, and 10 years after diagnosis were 40, 28, and 14, respectively. The incidences of symptomatic progression related to the cyst were 0%, 0%, and 8% at 2, 5, and 10 years, respectively. No patient died suddenly during the follow-up interval. Two (6%) of 34 patients in whom follow-up imaging was performed either exhibited cyst growth (one patient) or experienced hydrocephalus (one patient) at a mean of 41 months after diagnosis (range 4–160 months).Conclusions. Patients in whom asymptomatic colloid cysts are diagnosed can be cared for safely with observation and serial neuroimaging. If a patient becomes symptomatic, the cyst enlarges, or hydrocephalus develops, prompt neurosurgical intervention is necessary to prevent the occurrence of neurological decline from these benign tumors.

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
Keyword(s):  
Natural History ◽  
Imaging Techniques ◽  
Venous Drainage ◽  
Vascular Lesion ◽  
Neurological Dysfunction ◽  
Content Type ◽  
History Of ◽  
Clinical Records ◽  
Venous Angiomas ◽  
Fine Print

✓ 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.

Coexistence of intracranial and spinal cavernous malformations: a study of prevalence and natural history

2006 ◽  
Vol 104 (3) ◽  
pp. 376-381 ◽  
Author(s):  
Aaron A. Cohen-Gadol ◽  
Jeffrey T. Jacob ◽  
Diane A. Edwards ◽  
William E. Krauss
Keyword(s):  
Family History ◽  
Natural History ◽  
Mr Imaging ◽  
Neurological Deficits ◽  
Cavernous Malformations ◽  
Recurrent Hemorrhage ◽  
Long Term Follow Up ◽  
History Of ◽  
Clinically Significant

Object The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients’ mean age at presentation was 50 years (range 13–82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.

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