Lasten aivovaltimoaneurysmat

Objectives. Intracranial aneurysms in children are rare and population-based long-term follow-up studies are limited. In this study, a large clinical and angiographic long-term follow-up was carried out. The special characteristics of the patients and their aneurysms were assessed together with factors affecting early and long-term morbidity and mortality. Materials and Methods. All pediatric (≤18 years) aneurysm patients treated at the Department of Neurosurgery in Helsinki during 1937-2009 were followed from diagnosis until death or the end of the year 2011. Later patient data were gathered from the referring hospitals, and a new follow-up was organized. The long-term excess mortality was calculated by using relative survival ratio. Results. Patients comprised 114 individuals with 130 aneurysms. Most aneurysms were ruptured and of medium size. Internal carotid artery bifurcation was the most common location, and boys were predominantly affected. The majority of the aneurysms were treated surgically. After a mean follow-up time of 25 years, 62% of the patients had a good outcome, 3% were dependent and 35% had died. Of the survivors, 91% lived independently at home and were meaningfully employed. Twenty-eight percent of the survivors were high school graduates. Factors correlating with a favorable long-term outcome were aneurysm location in the anterior circulation, operative treatment, and complete aneurysm closure postoperatively. Fourteen patients (12%) had a family history of aneurysms. Fifty-nine patients had long-term angiographic follow-up data (median follow-up 34 yrs). Of these patients, 41% were diagnosed with a total of 36 new aneurysms. Seven new subarachnoid hemorrhages (SAH) occurred. The annual rate of development of new aneurysm was 1.9% and that of hemorrhage 0.4%. Current or previous smoking (odds ratio 3.39, 95% confidence interval [CI] 1.02-11.29, p=0.047) was the only significant risk factor for new aneurysm formation. No independent risk factors for new SAH emerged. Cumulative risk of new SAH 40 years after the initial diagnosis was 15% (95% CI 5-25%). There was an overall excess mortality of 10% and 19% at 20 and 40 years after the diagnosis among the one-year survivors, respectively. The excess mortality was particularly high in male patients, and was mainly aneurysm-related. Conclusions. Most patients had a good recovery, but almost half of them developed new aneurysms during a follow-up of 34 years, with smoking being a major risk factor. A long-term excess mortality exists in pediatric aneurysm patients, especially males, even decades after successful treatment of a ruptured aneurysm. The excess mortality is mainly aneurysm-related. A life-long angiographic follow-up is mandatory in these patients.Aivovaltimopullistuma on aivovaltimoiden haarautumiskohtaan muodostunut pallomainen pullistuma eli aneurysma. Aivovaltimoaneurysman puhkeaminen aiheuttaa hengenvaarallisen lukinkalvonalaisen aivoverenvuodon (SAV). Aivovaltimoaneurysmat ovat harvinaisia lapsilla. Tässä väitöstutkimuksessa suoritettiin pitkäaikaisseuranta Helsingissä Neurokirurgian klinikassa vuosien 1937-2009 aikana hoidetuille alle 18-vuotiaille aivovaltimoaneurysma-potilaille. Potilaiden ja aneurysmien erityispiirteet määritettiin, sekä selvitettiin potilaiden pitkän aikavälin sairastuvuus. Potilaiden ylikuolleisuus, eli kuolleisuuden lisäys verrattuna keskimääräiseen kaltaistettuun väestöön määritettiin. Näiden potilaiden riski saada uusia aneurysmia tai uusi aneurysmavuoto selvitettiin sekä arvioitiin näihin vaikuttavat riskitekijät. Seuranta-aikana hoidettiin 114 lapsianeurysma-potilasta, joilla oli yhteensä 130 aneurysmaa. Useimmat aneurysmista olivat vuotaneita ja keskikokoisia. Sisemmän kaulavaltimon huippu oli aneurysmien yleisin sijaintipaikka. Valtaosa potilaista oli poikia. Suurin osa aneurysmista hoidettiin kirurgisesti. 25 vuoden seurannassa valtaosa potilaista oli toipunut hyvin ja kolmasosa oli kuollut. Eloonjääneistä suurin osa asui itsenäisesti kotona ja oli työelämässä. Kolmasosa eloonjääneitä oli suorittanut vähintään lukion oppimäärän. Suotuisaan pitkän aikavälin ennusteeseen vaikuttivat aneurysman sijainti sisemmän kaulavaltimon alueella, kirurginen hoito ja täydellinen aneurysman sulku leikkauksesta. Neljäntoista (12%) potilaan suvussa oli taipumus aivovaltimoaneurysmiin. Viidelläkymmenelläyhdeksällä potilaalla oli radiologisia seurantatietoja aivovaltimoista. Potilaista 41%:lla todettiin uusi aivovaltimoaneurysma 34 vuoden seurannassa. Seitsemän potilaista oli sairastanut uuden SAV:n. Tupakointi oli ainoa merkittävä riskitekijä uuden aneurysman synnylle. Riski sairastua uuteen SAV:hen 40 vuotta ensimmäisen vuoden jälkeen oli 15%. Potilaiden ylikuolleisuus 20 vuoden seurannassa oli 10% ja 40 vuoden seurannassa 19%. Ylikuolleisuus oli erityisen korkea miespotilailla, ja aiheutui lähinnä aneurysmaan liittyvistä tekijöistä. Yhteenvetona voidaan todeta, että potilaat toipuivat hyvin, mutta lähes puolella heistä todettiin uusi aneurysman yli 34 vuoden seurannassa. Tupakointi oli ainoa merkittävä riskitekijä uuden aneurysman synnylle. Lapsuudessa aivovaltimoaneurysma-diagnoosin saaneilla potilailla on pitkäaikaisseurannassa ylikuolleisuutta vuosikymmeniä onnistuneenkin aneurysman hoidon jälkeen. Tämä ylikuolleisuus johtuu lähinnä aneurysmaan liittyvistä tekijöistä. Näiden potilaiden elinikäinen radiologinen seuranta on perusteltua

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.Peer reviewe

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.Peer reviewe

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

Johdanto Lasten aivojen valtimolaskimoepämuodostumista ei ole julkaistu juurikaan väestöpohjaista pitkän aikavälin seurantaa. Tavoite Selventää lasten valtimolaskimoepämuodostumien ominaisuuksia ja pitkän aikavälin toipuminen. Menetelmät Retrospektiivinen analyysi suoritettiin 805 perättäiselle potilaalle, jotka kirjautuivat Töölön sairaalan neurokirurgian klinikkaan vuosien 1942 ja 2014 välillä ja joilla oli aivojen valtimolaskimoepämuodostuma. Potilaat määriteltiin lapsiksi, jos he olivat alle 18-vuotiaita kirjautumisajankohtana. Lapsia verrattiin aikuiskohorttiin. Taudin muuttuvia ilmenemismalleja tarkasteltiin vuosikymmenittäin. Tulokset Potilaat koostuivat 127 lapsesta, joiden keski-ikä oli 12-vuotta. Keskiseuranta-aika oli 21-vuotta (0-62 välillä). Lasten valtimolaskimoepämuodostumat tulivat aikuisia useammin esille aivoverenvuotona, mutta harvemmin epilepsian takia. Tyvitumakkeen, pikkuaivojen ja posteriorisen aivokurkiaisen viereisen alueen valtimolaskimoepämuodostumat olivat lapsilla aikuisia yleisempiä. Otsalohkon ja ohimolohkon valtimolaskimoepämuodostumat olivat puolestaan aikuisilla yleisempiä. Kun sattumalöydöksinä ja epilepsian takia ilmitulleet valtimolaskimoepämuodostumat yleistyivät, aivoverenvuotojen esiintyvyys pieneni kummassakin kohortissa. Kokonaisuudessaan valtimolaskimoepämuodostuma aiheutti 22 (82%) lasten ja 108 (39%) aikuisten kuolemaa. Multivarianttianalyysin jälkeen valtimolaskimoepämuodostuman pieni koko ja sen kirurginen poisto korreloivat suotuisan pitkänajan tuloksen kanssa. Johtopäätökset Lasten valtimolaskimoepämuodostumat tulivat esille useammin aivoverenvuodon takia kuin aikuisilla. Tätä heijasteli myös epileptisen ilmenemisen pienempi prevalenssi lapsissa. Lasten valtimolaskomoepämuodostumat sijaitsivat aikuisia harvemmin lohkoissa tai kuorikerroksella, mutta useammin aivojen syvissä osissa ja pikkuaivoissa. Aivoverenvuotojen esiintyminen korreloi negatiivisesti sattumalta ja epilepsian takia löydettyjen valtimolaskimoepämuodostumien kanssa.Lapsissa valtimolaskimoepämuodostuma oli tärkein kuolleisuuden aiheuttaja, mutta aikuisissa kuolemat johtuivat useammin muista syistä.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