Päivi Koroknay-Pál

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.

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.

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.

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.

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.

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 patients 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.

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

Abstract 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.