scholarly journals Neurological outcomes of untreated brainstem cavernous malformations in a prospective observational cohort and literature review

2021 ◽  
pp. svn-2020-000608
Author(s):  
Da Li ◽  
Jing-Jie Zheng ◽  
Jian-Cong Weng ◽  
Pan-Pan Liu ◽  
Ze-Yu Wu ◽  
...  
Keyword(s):  
Neurological Status ◽  
Neurological Deficits ◽  
Developmental Venous Anomaly ◽  
Cavernous Malformations ◽  
Neurological Outcomes ◽  
Scale Scores ◽  
Risk Patients ◽  
History Of ◽  
Lost To Follow Up

BackgroundHaemorrhages of brainstem cavernous malformations (CMs) can lead to neurological deficits, the natural history of which is uncertain. The study aimed to evaluate the neurological outcomes of untreated brainstem CMs and to identify the adverse factors associated with worsened outcomes.MethodsFrom 2009 to 2015, 698 patients (321 women) with brainstem CMs were entered into the prospective cohort after excluding patients lost to follow-up (n=43). All patients were registered, clinical data were collected and scheduled follow-up was performed.ResultsAfter a median follow-up of 60.9 months, prospective haemorrhages occurred in 167 patients (23.9%). The mean modified Rankin Scale scores at enrolment and at censoring time were 1.6 and 1.2. Neurological status was improved, unchanged and worsened in 334 (47.9%), 293 (42.0%) and 71 (10.2%) patients, respectively; 233 (33.4%) recovered to normal levels. Lesions crossing the axial midpoint (relative risk (RR) 2.325, p=0.003) and developmental venous anomaly (DVA) (RR 1.776, p=0.036) were independently significantly related to worsened outcomes. The percentage of worsened outcomes was 5.3% (18 of 337) in low-risk patients (neither DVA nor crossing the axial point) and increased to 26.0% (13 of 50) in high-risk patients (with both DVA and crossing the axial point). The percentage of worsened outcomes significantly increased as the number of prospective haemorrhages increased (from 1.5% (8 of 531, if 0 prospective ictus) to 37.5% (48 of 128, if 1 ictus) and 38.5% (15 of 39, if >1 ictus)).ConclusionsThe neurological outcomes of untreated brainstem CMs were improved/unchanged in majority of patients (89.8%) with a fatality rate of 1.7% in our cohort, which seemed to be favourable. Radiological features significantly predicted worsened outcomes. Our results provide evidence for clinical consultation and individualised treatment. The referral bias of our cohort was underlined.

scholarly journals Clinical course of untreated thalamic cavernous malformations: hemorrhage risk and neurological outcomes

2017 ◽  
Vol 127 (3) ◽  
pp. 480-491 ◽  
Author(s):  
Kai-Bing Tian ◽  
Jing-Jie Zheng ◽  
Jun-Peng Ma ◽  
Shu-Yu Hao ◽  
Liang Wang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Clinical Course ◽  
Neurological Status ◽  
Lesion Size ◽  
Initial Presentation ◽  
Neurological Deficits ◽  
Developmental Venous Anomaly ◽  
Cavernous Malformations ◽  
Hemorrhage Risk

OBJECTIVEThe natural history of cerebral cavernous malformations (CMs) has been widely studied, but the clinical course of untreated thalamic CMs is largely unknown. Hemorrhage of these lesions can be devastating. The authors undertook this study to obtain a prospective hemorrhage rate and provide a better understanding of the prognosis of untreated thalamic CMs.METHODSThis longitudinal cohort study included patients with thalamic CMs who were diagnosed between 2000 and 2015. Clinical data were recorded, radiological studies were extensively reviewed, and follow-up evaluations were performed.RESULTSA total of 121 patients were included in the study (56.2% female), with a mean follow-up duration of 3.6 years. The overall annual hemorrhage rate (subsequent to the initial presentation) was calculated to be 9.7% based on the occurrence of 42 hemorrhages over 433.1 patient-years. This rate was highest in patients (n = 87) who initially presented with hemorrhage and focal neurological deficits (FNDs) (14.1%) (χ2 = 15.358, p < 0.001), followed by patients (n = 19) with hemorrhage but without FND (4.5%) and patients (n = 15) without hemorrhage regardless of symptoms (1.2%). The initial patient presentations of hemorrhage with FND (hazard ratio [HR] 2.767, 95% CI 1.336–5.731, p = 0.006) and associated developmental venous anomaly (DVA) (HR 2.510, 95% CI 1.275–4.942, p = 0.008) were identified as independent hemorrhage risk factors. The annual hemorrhage rate was significantly higher in patients with hemorrhagic pres entation at diagnosis (11.7%, p = 0.004) or DVA (15.7%, p = 0.002). Compared with the modified Rankin Scale (mRS) score at diagnosis (mean 2.2), the final mRS score (mean 2.0) was improved in 37 patients (30.6%), stable in 59 patients (48.8%), and worse in 25 patients (20.7%). Lesion size (odds ratio [OR] per 0.1 cm increase 3.410, 95% CI 1.272–9.146, p = 0.015) and mRS score at diagnosis (OR per 1 point increase 3.548, 95% CI 1.815–6.937, p < 0.001) were independent adverse risk factors for poor neurological outcome (mRS score ≥ 2). Patients experiencing hemorrhage after the initial ictus (OR per 1 ictus increase 6.923, 95% CI 3.023–15.855, p < 0.001) had a greater chance of worsened neurological status.CONCLUSIONSThis study verified the adverse predictors for hemorrhage and functional outcomes of thalamic CMs and demonstrated an overall annual symptomatic hemorrhage rate of 9.7% after the initial presentation. These findings and the mode of initial presentation are useful for clinicians and patients when selecting an appropriate treatment, although the tertiary referral bias of the series should be taken into account.

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.

scholarly journals Longitudinal evaluation of corticospinal tract in patients with resected brainstem cavernous malformations using high-definition fiber tractography and diffusion connectometry analysis: preliminary experience

2015 ◽  
Vol 123 (5) ◽  
pp. 1133-1144 ◽  
Author(s):  
Amir H. Faraji ◽  
Kumar Abhinav ◽  
Kevin Jarbo ◽  
Fang-Cheng Yeh ◽  
Samuel S. Shin ◽  
...  
Keyword(s):  
Quantitative Data ◽  
Normal Subjects ◽  
Neurological Status ◽  
Fiber Tractography ◽  
High Definition ◽  
Cavernous Malformations ◽  
Neurological Outcomes ◽  
Complementary Approach ◽  
Relationship Of

OBJECT Brainstem cavernous malformations (CMs) are challenging due to a higher symptomatic hemorrhage rate and potential morbidity associated with their resection. The authors aimed to preoperatively define the relationship of CMs to the perilesional corticospinal tracts (CSTs) by obtaining qualitative and quantitative data using high-definition fiber tractography. These data were examined postoperatively by using longitudinal scans and in relation to patients’ symptomatology. The extent of involvement of the CST was further evaluated longitudinally using the automated “diffusion connectometry” analysis. METHODS Fiber tractography was performed with DSI Studio using a quantitative anisotropy (QA)-based generalized deterministic tracking algorithm. Qualitatively, CST was classified as being “disrupted” and/or “displaced.” Quantitative analysis involved obtaining mean QA values for the CST and its perilesional and nonperilesional segments. The contralateral CST was used for comparison. Diffusion connectometry analysis included comparison of patients’ data with a template from 90 normal subjects. RESULTS Three patients (mean age 22 years) with symptomatic pontomesencephalic hemorrhagic CMs and varying degrees of hemiparesis were identified. The mean follow-up period was 37.3 months. Qualitatively, CST was partially disrupted and displaced in all. Direction of the displacement was different in each case and progressively improved corresponding with the patient’s neurological status. No patient experienced neurological decline related to the resection. The perilesional mean QA percentage decreases supported tract disruption and decreased further over the follow-up period (Case 1, 26%–49%; Case 2, 35%–66%; and Case 3, 63%–78%). Diffusion connectometry demonstrated rostrocaudal involvement of the CST consistent with the quantitative data. CONCLUSIONS Hemorrhagic brainstem CMs can disrupt and displace perilesional white matter tracts with the latter occurring in unpredictable directions. This requires the use of tractography to accurately define their orientation to optimize surgical entry point, minimize morbidity, and enhance neurological outcomes. Observed anisotropy decreases in the perilesional segments are consistent with neural injury following hemorrhagic insults. A model using these values in different CST segments can be used to longitudinally monitor its craniocaudal integrity. Diffusion connectometry is a complementary approach providing longitudinal information on the rostrocaudal involvement of the CST.

scholarly journals Natural history of brainstem cavernous malformations: prospective hemorrhage rate and adverse factors in a consecutive prospective cohort

2020 ◽  
pp. 1-12
Author(s):  
Da Li ◽  
Ze-Yu Wu ◽  
Pan-Pan Liu ◽  
Jun-Peng Ma ◽  
Xu-Lei Huo ◽  
...  
Keyword(s):  
Risk Factor ◽  
Natural History ◽  
Neurological Deficit ◽  
Cox Regression ◽  
Developmental Venous Anomaly ◽  
Focal Neurological Deficit ◽  
Cavernous Malformations ◽  
Cox Regression Analysis ◽  
History Of

OBJECTIVEGiven the paucity of data on the natural history of brainstem cavernous malformations (CMs), the authors aimed to evaluate the annual hemorrhage rate and hemorrhagic risk of brainstem CMs.METHODSNine hundred seventy-nine patients diagnosed with brainstem CMs were referred to Beijing Tiantan Hospital from 2006 to 2015; 224 patients were excluded according to exclusion criteria, and 47 patients were lost to follow-up. Thus, this prospective observational cohort included 708 cases (324 females). All patients were registered, clinical data were recorded, and follow-up was completed.RESULTSSix hundred ninety (97.5%) of the 708 patients had a prior hemorrhage, 514 (72.6%) had hemorrhagic presentation, and developmental venous anomaly (DVA) was observed in 241 cases (34.0%). Two hundred thirty-seven prospective hemorrhages occurred in 175 patients (24.7%) during 3400.2 total patient-years, yielding a prospective annual hemorrhage rate of 7.0% (95% CI 6.2%–7.9%), which decreased to 4.7% after the 1st year. Multivariate Cox regression analysis after adjusting for sex and age identified hemorrhagic presentation (HR 1.574, p = 0.022), DVA (HR 1.678, p = 0.001), mRS score ≥ 2 on admission (HR 1.379, p = 0.044), lesion size > 1.5 cm (HR 1.458, p = 0.026), crossing the axial midpoint (HR 1.446, p = 0.029), and superficially seated location (HR 1.307, p = 0.025) as independent adverse factors for prospective hemorrhage, but history of prior hemorrhage was not significant. The annual hemorrhage rates were 8.3% and 4.3% in patients with and without hemorrhagic presentation, respectively; the rate was 9.9%, 6.0%, and 1.0% in patients with ≥ 2, only 1, and 0 prior hemorrhages, respectively; and the rate was 9.2% in patients with both hemorrhagic presentation and focal neurological deficit on admission.CONCLUSIONSThe study reported an annual hemorrhage rate of 7.0% exclusively for brainstem CMs, which significantly increased if patients presented with both hemorrhagic presentation and focal neurological deficit (9.2%), or any other risk factor. Patients with a risk factor for hemorrhage needed close follow-up regardless of the number of prior hemorrhages. It should be noted that the referral bias in this study could have overestimated the annual hemorrhage rate. This study improved the understanding of the natural history of brainstem CMs, and the results are important for helping patients and physicians choose a suitable treatment option based on the risk factors and stratified annual rates.Clinical trial registration no.: ChiCTR-POC-17011575 (http://www.chictr.org.cn/).

Microsurgery for Small Arteriovenous Malformations of the Brain: Results in 110 Consecutive Patients

Neurosurgery ◽  
2000 ◽  
Vol 47 (3) ◽  
pp. 571-577 ◽  
Author(s):  
Justin H. T. Pik ◽  
Michael K. Morgan
Keyword(s):  
Arteriovenous Malformations ◽  
Postoperative Outcome ◽  
Neurological Status ◽  
Acute Onset ◽  
Neurological Deficits ◽  
Vascular Lesions ◽  
History Of ◽  
The Brain ◽  
Results Of Surgery

ABSTRACT OBJECTIVE To examine the results of surgery in 110 consecutive patients with arteriovenous malformations (AVMs) smaller than 3 cm in diameter. These results are compared with the published results of other microsurgical series as well as with results for patients treated with focused irradiation. METHODS From January 1989 to November 1998, 121 patients with AVMs smaller than 3 cm were treated at our institution. One hundred ten patients underwent microsurgical removal of their AVMs. The presentation, preoperative neurological status, and postoperative outcome were recorded. Follow-up was complete for all surgical cases. RESULTS Of the 110 patients, 109 (99%) had angiographically confirmed obliteration of their AVMs. Two patients (1.8%) required reoperation for residual AVM. Two (4.3%) of 46 patients with AVMs in eloquent brain areas experienced worsening of their neurological status after surgery. One (1.6%) of 64 patients was worse neurologically after removal of an AVM in a noneloquent area. CONCLUSION Microsurgical removal is a safe and effective treatment for the majority of AVMs smaller than 3 cm in diameter. Although the treatment is accompanied by a risk of acute onset of neurological deficits, this tends to be transient in the majority of cases. Furthermore, microsurgical excision of small AVMs offers patients immediate protection from the natural history of their vascular lesions.

LONG-TERM OUTCOME OF PATIENTS WITH MULTIPLE CEREBRAL CAVERNOUS MALFORMATIONS

Neurosurgery ◽  
2009 ◽  
Vol 65 (3) ◽  
pp. 450-455 ◽  
Author(s):  
Juri Kivelev ◽  
Mika Niemelä ◽  
Riku Kivisaari ◽  
Reza Dashti ◽  
Aki Laakso ◽  
...  
Keyword(s):  
Family History ◽  
De Novo ◽  
Good Condition ◽  
Neurological Deficits ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Long Term Outcome ◽  
History Of ◽  
The Mean

Abstract OBJECTIVE Multiple cerebral cavernous malformations (MCCMs) typically occur in patients with a family history of these lesions. Literature on MCCMs is scarce, and little is known about their natural history. METHODS Of 264 consecutive patients with cerebral cavernomas treated at the Department of Neurosurgery, Helsinki University Central Hospital, in the past 27 years, 33 patients had MCCMs. Lesions were categorized according to the Zabramski classification scale. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale, and amelioration of epilepsy was assessed using the Engel scale. All clinical data were analyzed retrospectively. RESULTS The mean age of patients at diagnosis was 44 years. Sex presentation was almost equal. Nine percent of all patients had a family history of the disease. Patients presented with epilepsy, acute headache, and focal neurological deficits. MCCMs were incidental findings in 2 patients. Altogether, 416 cavernomas were found: 70% supratentorial and 30% infratentorial. Fifteen patients had symptomatic hemorrhage before admission to our department. Surgery was performed on 18 patients. In most cases, the largest cavernoma was removed. Postoperatively, 1 patient experienced temporary hemiparesis, and another developed permanent motor dysphasia. No mortalities occurred. The mean follow-up time was 7.7 years. Twenty-six patients (79%) were in good condition. Among patients with epilepsy who underwent lesionectomy, 70% had an Engel class I outcome. On follow-up magnetic resonance imaging, 52 de novo cavernomas were found. CONCLUSION Surgical treatment of patients with MCCMs is safe. An extirpation of the clinically active cavernoma prevents further bleedings and improves outcome of epilepsy.

scholarly journals Clinical Characteristics and Outcome of Staphylococcus aureus Prostate Abscess From Ten Years of Experience at a Tertiary Care Center

2019 ◽  
Vol 6 (10) ◽  
Author(s):  
Bryan Walker ◽  
Eric Heidel ◽  
Mahmoud Shorman
Keyword(s):  
Staphylococcus Aureus ◽  
Rare Complication ◽  
Care Center ◽  
Tertiary Care ◽  
Care Hospital ◽  
Risk Patients ◽  
History Of ◽  
A Site ◽  
Lost To Follow Up

Abstract Objective Prostatic abscess (PA) is an uncommon infection that is generally secondary to Escherichia coli and other members of the Enterobacteriaceae family. In recent years, although rare, more reports of Staphylococcus aureus (S. aureus) PA have been reported, especially with increasing reports of bacteremia associated with injection drug use (IDU). Method This was a retrospective review of adult patients admitted to a tertiary care hospital between 2008 and 2018 and who had a diagnosis of S. aureus PA. Results Twenty-one patients were included. The average age was 46 years. Fourteen (67%) patients presented with genitourinary concerns. Main risk factors included concurrent skin or soft tissue infections (52%), history of genitourinary disease or instrumentation (48%), IDU (38%), and diabetes mellitus (38%). Methicillin-resistant Staphylococcus aureus (MRSA) was identified in 57% and concomitant bacteremia in 81% of patients. Surgical or a radiologically guided drainage was performed in 81% of patients. Antibiotic treatment duration ranged from 3 to 8 weeks. Six patients were lost to follow-up. Clinical resolution was observed in the remaining 15 (81%) patients who had follow-up. Conclusions S. aureus PA continues to be a rare complication of S.aureus infections. In most published reports, MRSA is the culprit. In high risk patients with persistent bacteremia, physicians need to consider the prostate as a site of infection.

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.

Retrosigmoid Suprafloccular Transhorizontal Fissure Approach for Resection of Brainstem Cavernous Malformation

2010 ◽  
Vol 66 (suppl_2) ◽  
pp. ons306-ons313 ◽  
Author(s):  
Shiro Ohue ◽  
Takanori Fukushima ◽  
Allan H. Friedman ◽  
Yoshiaki Kumon ◽  
Takanori Ohnishi
Keyword(s):  
Neurological Status ◽  
Early Postoperative Period ◽  
Neurological Deficits ◽  
Anatomic Study ◽  
Cavernous Malformations ◽  
Entry Zone ◽  
Root Entry Zone ◽  
Microsurgical Resection ◽  
Cerebellar Peduncle

Abstract OBJECTIVE This study examined the usefulness of a surgical approach (retrosigmoid suprafloccular transhorizontal fissure approach) for resection of brainstem cavernous malformations (CMs). METHODS An anatomic study concerning the retrosigmoid suprafloccular transhorizontal fissure approach was performed with 3 cadaveric heads. Clinical course was retrospectively reviewed for 10 patients who underwent microsurgical resection of brainstem CMs with this approach. Medical, surgical, and neuroimaging records of these patients were evaluated. RESULTS In the anatomic study, after standard suboccipital retrosigmoid craniotomy, the horizontal fissure on the petrosal surface of the cerebellum was dissected between the superior semilunar lobule and flocculus. With this approach, the root entry zone of the trigeminal nerve and the middle cerebellar peduncle could be exposed by superior retraction of the superior semilunar lobule. The lateral surface of the pons was then easily visible around the root entry zone. When this approach was used for 10 brainstem CMs, complete resection was achieved in 9 patients (90%). No mortality was encountered in this study. New neurological deficits occurred in the early postoperative period for 4 patients but were transient in 3 patients. Neurological status at final follow-up was improved in 4 patients (40%), unchanged in 5 patients (50%), and worse in 1 patient (10%) compared with preoperative conditions. CONCLUSION The retrosigmoid suprafloccular transhorizontal fissure approach is useful for the resection of lateral pontine CMs.

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.

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