Recurrence of brain arteriovenous malformations in pediatric patients: a long-term follow-up study.

BACKGROUND: Previously thought to be congenital, AVMs have shown evidence of de-novo formation and continued growth, thus shifting thoughts on their pathophysiology. Pediatric AVM patients have been reported to be more prone to develop AVM recurrence after a seemingly complete cure. Therefore, we assessed the risk of AVM treated in childhood to recur in adulthood after a long-term follow-up in our own cohort. METHODS: Control DS-angiography was arranged during 2021-2022 as part of a new protocol for all AVM patients who were under 21 years of age at the time of their treatment and in whom the treatment had occurred at least five years earlier. Angiography was offered only to patients under 50 years of age at the time of the new protocol. The complete eradication of AVM after the primary treatment had been originally confirmed with DSA in every patient. RESULTS: A total of 42 patients participated in the late DSA control, and 41 of them were included in this analysis after excluding the patient diagnosed with HHT. The median age at the time of admission for AVM treatment was 14.6 (IQR 12-19, range 7-21 years) years. The median age at the time of the late follow-up DSA was 33.8 years (IQR 29.8-38.6, range 19.4-47.9 years). Two recurrent sporadic AVMs and one recurrent AVM in a patient with hereditary hemorrhagic telangiectasia (HHT) were detected. The recurrence rate was 4.9% for sporadic AVMs and 7.1% if HHT-AVM was included. All the recurrent AVMs had originally bled and been treated microsurgically. The patients with sporadic AVM recurrence had been smoking their whole adult lives. CONCLUSIONS: Pediatric and adolescent patients are prone to develop recurrent AVMs, even after complete AVM obliteration verified by angiography. Therefore, imaging follow-up is recommended.

Correlation between arteriovenous malformation nidus size and intraparenchymal hematoma volume in the event of rupture.

•Our study did not support the hypothesis that small AVMs lead to larger hematoma volumes in the event of a rupture.•AVM size did not correlate with the clinical severity of the bleeding as measured with HH and WFNS scores.•Larger supratentorial hematomas were associated with a more severe clinical manifestation and a poorer outcome.•In the event of a rupture, the AVM size did not correlate with the 2-4-month GOS.•AVICH score showed a good correlation with the 2-4-month GOS score.

Long-term health-related quality of life in patients with ruptured arteriovenous malformations treated in childhood.

OBJECTIVE: The aim of this study was to reveal the long-term health-related quality of life (HRQOL), educational level, and impact on occupation in 55 patients who experienced ruptured brain arteriovenous malformations (AVMs) that were treated during childhood. METHODS: In 2016, questionnaires including the 15D instrument were sent to all living patients older than 18 years (n = 432) in the Helsinki AVM database. The cohort was further specified to include only patients with ruptured AVMs who were younger than 20 years at the time of diagnosis (n = 55). Educational level was compared with the age-matched general population of Finland. The mean 15D scores were calculated for independent variables (Spetzler-Ponce classification, admission age, symptomatic epilepsy, and multiple bleeding episodes) and tested using the independent-samples t-test or ANCOVA. Linear regression was used to create a multivariate model. Bonferroni correction was used with multiple comparisons. RESULTS: The mean follow-up time from diagnosis to survey was 24.2 (SD 14.7) years. The difference in the mean 15D scores between Spetzler-Ponce classes did not reach statistical significance. The youngest age group (< 10 years at the time of diagnosis) performed less well on the dimension of usual activities than the older patients. Symptomatic epilepsy significantly reduced the long-term HRQOL. Multiple hemorrhages significantly reduced the scores on three dimensions: mobility, speech, and sexual activity. In the regression model, symptomatic epilepsy was the only significant predictor for a lower 15D score. The educational level of the cohort was for the most part comparable to that of the general population in the same age group. AVM was the reason for early retirement in 11% of the patients, while lowered performance because of the AVM was reported by 37% of the patients. CONCLUSIONS: Patients with ruptured AVMs treated in childhood can live an independent and meaningful life, even in the case of the highest-grade lesions. Symptomatic epilepsy significantly reduced the long-term HRQOL, highlighting the need for continuing support for these patients.

Method for the Intraoperative Detection of IDH Mutation in Gliomas with Differential Mobility Spectrometry.

Isocitrate dehydrogenase (IDH) mutation status is an important factor for surgical decision-making: patients with IDH-mutated tumors are more likely to have a good long-term prognosis, and thus favor aggressive resection with more survival benefit to gain. Patients with IDH wild-type tumors have generally poorer prognosis and, therefore, conservative resection to avoid neurological deficit is favored. Current histopathological analysis with frozen sections is unable to identify IDH mutation status intraoperatively, and more advanced methods are therefore needed. We examined a novel method suitable for intraoperative IDH mutation identification that is based on the differential mobility spectrometry (DMS) analysis of the tumor. We prospectively obtained tumor samples from 22 patients, including 11 IDH-mutated and 11 IDH wild-type tumors. The tumors were cut in 88 smaller specimens that were analyzed with DMS. With a linear discriminant analysis (LDA) algorithm, the DMS was able to classify tumor samples with 86% classification accuracy, 86% sensitivity, and 85% specificity. Our results show that DMS is able to differentiate IDH-mutated and IDH wild-type tumors with good accuracy in a setting suitable for intraoperative use, which makes it a promising novel solution for neurosurgical practice.

Comparing health-related quality of life in modified Rankin Scale grades: 15D results from 323 patients with brain arteriovenous malformation and population controls.

BACKGROUND: We wanted to understand how patients with different modified Rankin Scale (mRS) grades differ regarding their health-related quality of life (HRQoL) and how this affects the interpretation and dichotomization of the grade. METHODS: In 2016, all adult patients in our brain arteriovenous malformation (AVM) database (n = 432) were asked to fill in mailed letters including a questionnaire about self-sufficiency and lifestyle and the 15D HRQoL questionnaire. The follow-up mRS was defined in 2016 using the electronic patient registry and the questionnaire data. The 15D profiles of each mRS grade were compared to those of the general population and to each other, using ANCOVA with age and sex standardization. RESULTS: Patients in mRS 0 (mean 15D score = 0.954 ± 0.060) had significantly better HRQoL than the general population (mean = 0.927 ± 0.028), p < 0.0001, whereas patients in mRS 1-4 had worse HRQoL than the general population, p < 0.0001. Patients in mRS 1 (mean = 0.844 ± 0.100) and mRS 2 (mean = 0.838 ± 0.107) had a similar HRQoL. In the recently published AVM research, the most commonly used cut points for mRS dichotomization were between mRS 1 and 2 and between mRS 2 and 3. CONCLUSIONS: Using 15D, we were able to find significant differences in the HRQoL between mRS 0 and mRS 1 AVM patients, against the recent findings on stroke patients using EQ-5D in their analyses. Although the dichotomization cut point is commonly set between mRS 1 and 2, patients in these grades had a similar HRQoL and a decreased ability to continue their premorbid lifestyle, in contrast to patients in mRS 0.

Cigarette Smoking Is More Prevalent in Patients With Brain Arteriovenous Malformations Compared to General Population: A Cross-Sectional Population-Based Study.

BACKGROUND: Research on the prevalence of smokers in patients with brain arteriovenous malformation (AVM) remains nonexistent, even though smoking is a well-known risk factor for intracranial aneurysms. OBJECTIVE: To examine the prevalence and smoking habits of AVM patients. METHODS: Data on smoking habits were collected with a quality-of-life questionnaire mailed in 2016 to all patients in our large AVM database. These smoking data were supplemented with registry data derived from medical records. The prevalence of smokers was compared to that of the general population, derived from statistics of National Institute for Health and Welfare. Logit transformation of proportions and Students t distribution were used to calculate the 95% CIs for prevalence estimates. RESULTS: Of the 384 patients aged over 18 yr on admission, 277 (72.1%) returned the questionnaires in 2016. When compared to age, sex, and admission year matched general population, the proportion of smokers in AVM patients was 48% (CI = 41%-55%) and 19% (CI = 16%-21%) in the general population. The difference increased in older age groups; in those aged 65 to 77 yr, the percentage of smokers reached 73% (CI = 46%-90%), while the corresponding percentage in the general population was 7% (CI = 5%-9%). CONCLUSION: We observed considerably higher rates of smoking among AVM patients when compared to age, sex, and admission year matched general population. Our results suggest that in the development of AVMs, the role played by nicotine and other substances in tobacco smoke should be examined. Cigarette smoking could potentially be a common cerebrovascular risk factor.

Long-term health-related quality of life in 262 patients with brain arteriovenous malformation.

OBJECTIVE: To discover the health-related quality of life (HRQOL) of patients with treated arteriovenous malformation (AVM), we used the self-applicable HRQOL instrument, the 15D, and analyzed the scores in both in the whole study population and specified cohorts. METHODS: The 15D questionnaires were mailed to adult patients with AVM alive in 2016 (n = 432) in our database. Patients with completely eradicated AVM (n = 262) were included in a subsequent analysis. The results were compared with those of the general population standardized for age and sex. Subgroup analyses were conducted for epilepsy, number of bleeding episodes, location of the lesion, modified Rankin Scale score, and Spetzler-Ponce classification (SPC) using independent-samples t test or analysis of covariance. Tobit regression was used to explain the variance in the 15D score. RESULTS: Patients had impaired HRQOL compared to the reference population (p < 0.0001). Deep location, multiple bleeding episodes, and refractory epilepsy were associated with impaired HRQOL. Patients in SPC A and B had similar posttreatment 15D scores, whereas those in class C had an impaired HRQOL. Significant explanatory variables in the regression model were age, sex, number of bleeding episodes, refractory epilepsy, and SPC. CONCLUSIONS: With careful patient selection, patients in SPC B can reach as favorable HRQOL as those in SPC A provided the operation is successful. Multiple bleeding episodes should be prevented with effective treatment aiming at complete AVM obliteration. The postoperative treatment of patients with AVM should focus on preventing depressive symptoms, anxiety, and epileptic seizures. We encourage other research groups to use HRQOL instruments to fully understand the consequences of neurologic and neurosurgical diseases on patients' HRQOL.

Characteristics and Long-Term Outcome of 127 Children With Cerebral Arteriovenous Malformations.

BACKGROUND: Population-based long-term data on pediatric patients with cerebral arteriovenous malformations (AVMs) are limited. OBJECTIVE: To clarify the characteristics and long-term outcome of pediatric patients with AVM. METHODS: A retrospective analysis was performed on 805 consecutive brain AVM patients admitted to a single center between 1942 and 2014. The patients were defined as children if they were under 18 yr at admission. Children were compared to an adult cohort. Changing patterns of presentation were also analyzed by decades of admission. RESULTS: The patients comprised 127 children with a mean age of 12 yr. The mean follow-up time was 21 yr (range 0-62). Children presented more often with intracerebral hemorrhage (ICH) but less often with epilepsy than adults. Basal ganglia, cerebellar, and posterior paracallosal AVMs were more common in pediatric than in adult patients. Frontal and temporal AVMs, in contrast, were more common in adult than in pediatric patients. As the number of incidentally and epilepsy-diagnosed AVMs increased, ICH rates dropped in both cohorts. In total, 22 (82%) pediatric and 108 (39%) adult deaths were assessed as AVM related. After multivariate analysis, small AVM size and surgical treatment correlated with a favorable long-term outcome. CONCLUSION: Hemorrhagic presentation was more common in children than in adults. This was also reflected as lower prevalence of epileptic presentation in the pediatric cohort. Lobar and cortical AVM locations were less frequent, whereas deep and cerebellar AVMs were more common in children. Hemorrhagic presentation correlated negatively with incidentally and epilepsy-diagnosed AVMs. In children, AVM was a major cause of death, but in adults, other factors contributed more commonly to mortality.

The Application of the Novel Grading Scale (Lawton-Young Grading System) to Predict the Outcome of Brain Arteriovenous Malformation.

BACKGROUND: A supplementary grading scale (Supplemented Spetzler-Martin grade, Supp-SM) was introduced in 2010 as a refinement of the SM system to improve preoperative risk prediction of brain arteriovenous malformations (AVMs). OBJECTIVE: To determine the ability to predict surgical outcomes using the Supp-SM grading scale. METHODS: This retrospective study was conducted on 200 patients admitted to the Helsinki University Hospital between 2000 and 2014. The validity of the Supp-SM and SM grading systems was compared using the area under the receiver operating characteristic (AUROC) curves, with respect to the change between preoperative and early (3-4 mo) as well as final postoperative modified Rankin Scale (mRS) scores. RESULTS: The performance of the Supp-SM was superior to that of the SM grading scale in the early follow-up (3-4 mo): AUROC = 0.57 (95% confidence interval [CI]: 0.49-0.65) for SM and AUROC = 0.67 (95% CI: 0.60-0.75) for Supp-SM. The Supp-SM performance continued improving over SM at the late follow-up: AUROC = 0.63 (95% CI: 0.55-0.71) for SM and AUROC = 0.70 (95% CI: 0.62-0.77) for Supp-SM. The perforating artery supply, which is not part of either grading system, plays an important role in the early follow-up outcome (P = .008; odds ratio: 2.95; 95% CI: 1.32-6.55) and in the late follow-up outcome (P < .001; odds ratio: 5.89; 95% CI: 2.49-13.91). CONCLUSION: The Supp-SM grading system improves the outcome prediction accuracy and is a feasible alternative to the SMS, even for series with higher proportion of high-grade AVMs. However, perforators play important role on the outcome.

Multiple meningiomas in two male-to-female transsexual patients with hormone replacement therapy: A report of two cases and a brief literature review.

BACKGROUND: Exogenous sex hormones may play a role in meningioma development and growth. Thus, transsexual patients being on long-standing hormone replacement therapy (HRT) may be at particular risk for meningioma development and growth. Here we present two cases of two male-to-female transsexual patients taking HRT for an extended period of time with both patients requiring surgical treatment at our institution due to multiple growing meningiomas. CASE DESCRIPTION: The first patient was a 50-year-old genetic male (male-to-female transsexual) who presented 7 years after an extensive sex-change operation due to progressive bitemporal visual defects. The patient had been on HRT for approximately a decade. Radiological examinations showed a total of four meningiomas, one being a large suprasellar meningioma causing the symptoms. Three of the four meningiomas were operated on, but the patient's vision could not be saved. Immunohistochemical (IH) analysis of the meningiomas showed both estrogen and progesterone receptor expression. The patient continued HRT and during follow-up regrowth of the meningiomas was noted. The second patient was a 48-year-old genetic male (male-to-female transsexual) who had been on HRT for two decades and also presented due to left-sided visual loss. Radiological examination showed four meningiomas, one being a left-sided sinus cavernous meningioma causing compression of the visual apparatus. This patient had a previous normal head computerized tomography scan dating back 10 years before his current presentation. Three of the four meningiomas were operated on with a slight improvement in visual acuity. IH analysis showed positive progesterone receptor expression but negative estrogen receptor expression. CONCLUSION: Radiological screening may be justifiable for transsexual patients with a history of long-standing HRT and special focus should be paid to transsexual patients displaying new neurological symptoms or those already diagnosed with a meningioma.

Arteriovenous Malformations of the Posterior Fossa: Focus on Surgically Treated Patients Presenting with Hemorrhage.

BACKGROUND: Arteriovenous malformations (AVMs) of the posterior fossa are demanding lesions that often present with rupture. Studies including outcome analyses in surgically operated patients with ruptured infratentorial AVMs are scarce. Certain anatomic and demographic features have shown associations with postoperative outcomes. METHODS: Eighty-six patients with infratentorial AVM were collected from our AVM database. Fifty-four patients were admitted from 1990 onward, and their demographic, lesion, and treatment characteristics were analyzed. The cohort was further refined to 38 consecutive patients with surgically treated ruptured infratentorial AVM admitted to our center between 1990 and 2014, and statistical analyses of factors influencing outcomes were conducted. RESULTS: Twenty-seven patients (69%) had a favorable outcome at early follow-up and 24 (67%) had a favorable outcome at final follow-up. Factors associated with poor outcome in early recovery on univariate analyses were deep venous drainage of the lesion (odds ratio [OR], 5.3; P = 0.037) and high Hunt & Hess score (P = 0.003). In the multivariate model, independent predictors for poor outcome were deep venous drainage (OR, 14.5; P = 0.010) and older age at admission (OR, 1.06; P = 0.028). The sole independent predictor for poor outcome at last follow-up was deep venous drainage (OR, 5.00; P = 0.046). The total follow-up time was 370 person-years. CONCLUSIONS: AVMs of the posterior fossa usually present with rupture and thus require prompt clinical treatment. The majority of surgically treated patients recover favorably. Our data show that venous drainage patterns have the greatest influence on the patient's postoperative condition. Other influencing factors include the severity of hemorrhage and patient age at admission.

Timing of surgery for ruptured supratentorial arteriovenous malformations.

BACKGROUND: There are conflicting opinions regarding the optimal waiting time to perform surgery after rupture of supratentorial arteriovenous malformations (AVMs) to achieve the best possible outcome. OBJECTIVE: To analyze factors influencing outcomes for ruptured supratentorial AVMs after surgery, paying particular attention to the timing of the surgery. METHODS: We retrospectively investigated 59 patients admitted to our center between 2000 and 2014 for surgical treatment of ruptured supratentorial AVMs. We evaluated the effect of timing of surgery and other variables on the outcome at 2-4 months (early outcome), at 12 months (intermediate outcome) after surgery, and at final follow-up at the end of 2016 (late outcome). RESULTS: Age over 40 years (OR 18.4; 95% CI 1.9-172.1; p = 0.011), high Hunt and Hess grade (4 or 5) before surgery (OR 13.5; 95% CI 2.1-89.2; p = 0.007), hydrocephalus on admission (OR 12.9; 95% CI 1.8-94.4; p = 0.011), and over 400 cm3 bleeding during surgery (OR 11.5; 95% CI 1.5-86.6; p = 0.017) were associated with an unfavorable early outcome. Age over 40 years (OR 62.8; 95% CI 2.6-1524.9; p = 0.011), associated aneurysms (OR 34.7; 95% CI 1.4-829.9; p = 0.029), high Hunt and Hess grade before surgery (OR 29.2; 95% CI 2.6-332.6; p = 0.007), and over 400 cm3 bleeding during surgery (OR 35.3; 95% CI 1.7-748.7; p = 0.022) were associated with an unfavorable intermediate outcome. Associated aneurysms (OR 8.2; 95% CI 1.2-55.7; p = 0.031), high Hunt and Hess grade before surgery (OR 5.7; 95% CI 1.3-24.3; p = 0.019), and over 400 cm3 bleeding during surgery (OR 5.8; 95% CI 1.2-27.3; p = 0.027) were associated with an unfavorable outcome at last follow-up. Elapsed time between rupture and surgery did not affect early or final outcome. CONCLUSIONS: Early surgery in patients with ruptured supratentorial arteriovenous malformation is feasible strategy, with late results comparable to those achieved with delayed surgery. Many other factors than timing of surgery play significant roles in long-term outcomes for surgically treated ruptured supratentorial AVMs.

Surgical Clipping of Very Small Unruptured Intracranial Aneurysms: A Multicenter International Study.

BACKGROUND: Treatment of very small unruptured intracranial aneurysms (VSUIAs, defined as ≤3 mm) can be indicated in selected circumstances. The feasibility and outcomes of endovascular therapy for VSUIAs have been recently published; however, the efficacy and complication rate of surgical clipping has not been reported in any large series to date. OBJECTIVE: We conducted a multicenter study to examine surgical outcomes for VSUIAs. METHODS: All consecutive patients undergoing surgery for a VSUIA in 4 neurosurgical centers between October 2001 and December 2012 were retrospectively analyzed. RESULTS: In the study, 183 patients (128 women, mean age 51.3 years) were treated with 190 procedures for a total of 228 aneurysms. Most were anterior circulation aneurysms (n = 215). The majority were directly clipped (n = 222, 97.4%), with coagulation or wrapping in the remainder. After 1 reoperation for incomplete clipping, postoperative imaging of 225 aneurysms confirmed complete occlusion in 221 (98.2%), 1 neck remnant (0.44%), and 3 partial occlusions (1.3%). Mortality was 0%. Early postoperative neurological deficit developed in 12 patients (6.6%); posterior circulation location was a significant risk factor for early neurological deficit (P < .001). Middle cerebral artery aneurysms had the lowest rate of postoperative deficits at 1.5% (P = .023). After the initial 30-day perioperative period, all deficits related to treatment of posterior circulation aneurysms recovered; overall neurological morbidity decreased to 2.7% with no mortality. CONCLUSION: VSUIA clipping is highly effective and is associated with a low morbidity rate. For VSUIAs selected for treatment, our data support surgical clipping as the modality of choice.

Seventy aneurysms of the posterior inferior cerebellar artery: anatomical features and value of computed tomography angiography in microneurosurgery.

OBJECTIVE: Aneurysms at the junction of the vertebral artery (VA) and posterior inferior cerebellar artery (PICA) are relatively rare. Their treatment is challenged by the diverse anatomy of the VA and PICA, close involvement of the PICA in the aneurysm neck, and scant space deep in the posterior fossa next to the cranial nerves, brain stem, and obstructing structures of the skull base. Computed tomography angiography (CTA) visualizes bony structures in addition to the vasculature, and being noninvasive and easily available, it can serve for planning microsurgical treatment. We analyzed the anatomy of PICA aneurysms imaged by CTA to promote planning the treatment. METHODS: We examined the CTA images of 70 consecutive patients with a saccular PICA aneurysm who were all treated in the Department of Neurosurgery, Helsinki, Finland, 2001 to 2011. RESULTS: Each of the 70 patients had 1 aneurysm at the VA-PICA junction. The proportion of small aneurysms (<7 mm) was high, 67%. The dome-to-neck ratio was <1.2 in 24 (34%) of the aneurysms. In the coronal view, 58 (83%) aneurysms projected cranially, and only 2 (3%) caudally. The aneurysms were located from 1 mm below the foramen magnum to 31 mm above it, most arising at the level of the jugular tubercle. The median distance to the aneurysm from the midline was 6 mm, ranging from 7.5 mm contralateral to the origin of the parent artery to 14 mm ipsilateral. Compared with the right VA, the left VA was larger, it harbored the most aneurysms, and its aneurysms were more often ruptured. CONCLUSIONS: Relation of PICA aneurysms to skull base structures is highly variable; the aneurysms can also be extracranial, or lie on the site of the skull contralateral to the origin of the parent artery. These anatomical variations demand meticulous study of the angiography in each individual case, especially before surgical treatment of the aneurysm.

De novo and recurrent aneurysms in pediatric patients with cerebral aneurysms.

BACKGROUND AND PURPOSE: Long-term angiographic follow-up studies on pediatric aneurysm patients are scarce. METHODS: We gathered long-term clinical and angiographic follow-up data on all pediatric aneurysm patients (≤ 18 years at diagnosis) treated at the Department of Neurosurgery, Helsinki University Central Hospital, between 1937 and 2009. RESULTS: Fifty-nine patients with cerebral aneurysms in childhood had long-term clinical and radiological follow-up (median, 34 years; range, 4-56 years). Twenty-four patients (41%) were diagnosed with altogether 25 de novo and 11 recurrent aneurysms, with 9 (25%) of the aneurysms being symptomatic. New subarachnoid hemorrhage occurred in 7 patients; 4 of these patients died. Eight patients (33%) had multiple new aneurysms. The annual rate of hemorrhage was 0.4%, and the annual rate for the development of de novo or recurrent aneurysm was 1.9%. There were no de novo aneurysms in 7 patients with previously unruptured aneurysms. However, 1 recurrent aneurysm was diagnosed. Current and previous smoking (risk ratio, 2.44; 95% confidence interval, 1.07-5.55) was the only statistically significant risk factor for de novo and recurrent aneurysm formation in patients with previous subarachnoid hemorrhage, whereas hypertension, sex, or age at onset had no statistically significant effect. Smoking was also a statistically significant risk factor for new subarachnoid hemorrhage. CONCLUSIONS: Patients with ruptured intracranial aneurysms in childhood have a high risk for new aneurysms and new subarachnoid hemorrhage, especially if they start to smoke as adults. Life-long angiographic follow-up is mandatory.

Long-term excess mortality in pediatric patients with cerebral aneurysms.

BACKGROUND AND PURPOSE: Knowledge of the long-term excess mortality in pediatric aneurysm patients is lacking. The aim of this study was to assess the long-term excess mortality of 102 pediatric patients with cerebral aneurysm treated at the department of neurosurgery at Helsinki University Central Hospital between 1937 and 2009. METHODS: Patients were followed from diagnosis until death or the end of the year 2010. Relative survival ratio provided the measure of excess mortality in these patients compared with mortality of the general Finnish population matched by age, sex, and calendar time. RESULTS: A majority of the patients (n=89) presented with subarachnoid hemorrhage. Aneurysms (n=118) were treated operatively (n=79), endovascularly (n=1), or conservatively (n=36). The mean follow-up time was 26.8 years (range, 0-55.6 years). By the end of follow-up, 34 of the 102 patients had died; 26 of these deaths (76%) were aneurysm-related. There was overall excess mortality of 10% (cumulative relative survival ratio, 0.90; 95% CI, 0.80-0.96) and 19% (cumulative relative survival ratio, 0.81; 95% CI, 0.66-0.91) at 20 and 40 years after the diagnosis among the 1-year subarachnoid hemorrhage survivors, respectively. The excess mortality was particularly high in boys. There was no long-term excess mortality among patients with unruptured aneurysms. Aneurysm-related deaths included rebleedings from open or partially occluded aneurysms, epileptic seizures, de novo and recurrent aneurysms, or sequelae of subarachnoid hemorrhage. CONCLUSIONS: There is long-term excess mortality in pediatric patients with aneurysm even decades after successful treatment of a ruptured aneurysm, especially among boys. The excess mortality is mainly aneurysm-related.

Long-term outcome of 114 children with cerebral aneurysms.

OBJECT: Population-based data on pediatric patients with aneurysms are limited. The aim of this study is to clarify the characteristics and long-term outcomes of pediatric patients with aneurysms. METHODS: All pediatric patients (≤ 18 years old) with aneurysms among the 8996 aneurysm patients treated at the Department of Neurosurgery in Helsinki from 1937 to 2009 were followed from admission to the end of 2010. RESULTS: There were 114 pediatric patients with 130 total aneurysms during the study period. The mean patient age was 14.5 years (range 3 months to 18 years). The male:female ratio was 3:2. Eighty-nine patients (78%) presented with subarachnoid hemorrhage. The majority of the aneurysms (116 [89%]) were in the anterior circulation, and the most common location was the internal carotid artery bifurcation (36 [28%]). The average aneurysm diameter was 11 mm (range 2-55 mm) with 16 giant aneurysms (12%). Eighty aneurysms (62%) were treated microsurgically, and 37 (28%) were treated conservatively due to poor medical and neurological status of the patient or due to technical reasons during the early years of the patient series. No connective tissue disorders common to pediatric aneurysm patients were diagnosed in this series, with the exception of 1 patient with tuberous sclerosis complex. The mean follow-up duration was 24.8 years (range 0-55.8 years). At the end of follow-up, 71 patients (62%) had a good outcome, 3 (3%) were dependent, and 40 (35%) had died. Twenty-seven deaths (68%) were assessed to be aneurysm-related. Factors correlating with a favorable long-term outcome were good neurological condition of the patient on admission, aneurysm location in the anterior circulation, complete aneurysm closure, and absence of vasospasm. Six patients developed symptomatic de novo aneurysms after a median of 25 years (range 11-37 years). Fourteen patients (12%) had a family history of aneurysms. There was no increased incidence for cardiovascular diseases in long-term follow-up. CONCLUSIONS: Most aneurysms were ruptured and of medium size. Internal carotid artery bifurcation was the most frequent location of the aneurysms. There was a male predominance of pediatric patients with aneurysms. Most patients experienced good recovery, with 91% of the long-term survivors living at home independently without assistance and meaningfully employed. Altogether, almost a third of these patients finished high school and one-fifth had a college or university degree. Pediatric patients had a tendency to develop de novo aneurysms.

[Alertness in transient visual disturbances of one eye]. / Ohimenevä toisen silmän näköhäiriö vaatii valppautta.

TIA is a brief and transient ictal symptom due to a local disturbance of cerebral or ocular arterial circulation. Transitory blindness or blurred vision in one eye (amaurosis fugax) is a sudden TIA symptom lasting a few minutes, usually underlied by thromboembolism originating from an atherosclerotic plaque of the internal carotid artery, less frequently an embolus from the aorta or the heart, or localized thrombosis of vessels of the optic nerve or the retina. We describe two amaurosis fugax patients.

Microneurosurgical management of middle cerebral artery bifurcation aneurysms.

BACKGROUND: Of the MCA aneurysms, those located at the main bifurcation of the MCA (MbifA) are by far the most frequent. The purpose of this article is to review the practical anatomy, preoperative planning, and avoidance of complications in the microsurgical dissection and clipping of MbifAs. METHODS: This review, and the whole series on intracranial aneurysms, is mainly based on the personal microneurosurgical experience of the senior author (JH) in 2 Finnish centers (Helsinki and Kuopio), which serve without patient selection the catchment area in southern and eastern Finland. RESULTS: These 2 centers have treated more than 10,000 patients with intracranial aneurysm's since 1951. In the Kuopio Cerebral Aneurysm Data Base of 3005 patients with 4253 aneurysms, MbifAs formed 30% of all ruptured aneurysms, 36% of all unruptured aneurysms, 35% of all giant aneurysms, and 89% of all MCA aneurysms. Importantly, in 45%, rupture of MbifA caused an ICH. CONCLUSIONS: Middle cerebral artery bifurcation aneurysms are often broad necked and may involve one or both branches of the bifurcation (M2s). The anatomical and hemodynamic features of MbifAs make them usually more favorable for microneurosurgical treatment. In population-based services, MbifAs are frequent targets of elective surgery (unruptured), acute surgery (ruptured), and emergency surgery (large ICH), even advanced approaches (giant). The challenge is to clip the neck adequately, without neck remnants, while preserving the bifurcational flow.