Timing of surgery for ruptured supratentorial arteriovenous malformations

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. To analyze factors influencing outcomes for ruptured supratentorial AVMs after surgery, paying particular attention to the timing of the surgery. 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). 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 cm(3) 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 cm(3) 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 cm(3) 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. 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.Peer reviewe

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

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.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

Lasten aivojen valtimolaskimoepämuodostumat

Brain arteriovenous malformations (BAVMs or AVMs) are high-flow cerebrovascular lesions consisting of a tangle of abnormal blood vessels, the nidus, which acts as an arteriovenous (AV) shunt. The incidence of newly diagnosed AVMs is around 1 case per 100 000 person-years. The incidence peaks between 20 and 40 years of age. The average risk of arteriovenous malformation hemorrhage is estimated to be 2-3% per year. Around 10-20% of AVM patients are under 18 years of age at the time of diagnosis. Despite their relative rarity, AVMs are the most common cause of non-traumatic intracranial hemorrhage (ICH) in the pediatric population. The goal of treatment is to eliminate subsequent rupture risk with minimal neurological morbidity. Treatment options comprise microneurosurgery, radiosurgery, endovascular embolization, or any combination of these. This thesis aimed to clarify the special characteristics, long-term outcomes, and health-related quality of life (HRQOL) in patients treated for AVM in childhood or adolescence. We also analyzed the risk of AVM recurrence in patients treated under 21 years of age, evaluated by late digital subtraction angiography (DSA) follow-up. The data on patients were drawn from the Helsinki AVM database. Consisting of 805 AVM patients treated at the Helsinki University Hospital Department of Neurosurgery between 1942 and 2014, the database has been retrospectively collected from medical records and images. In 2016, questionnaires were sent to all living patients over 18 years of age (n = 432). The database was updated with the information obtained from the questionnaires and by reviewing all the relevant medical records, images, and death certificates. The questionnaires contained the 15D HRQOL instrument as well as separate questions on symptoms, comorbid conditions, lifestyle, educational level, and self-sufficiency/independence. Of the 432 patients sent questionnaires, 325 (75%) replied. In Study I, pediatric AVM patients presented with intracranial hemorrhage more often than adults. At the same time, children presented with epileptic seizures (not associated with hemorrhage) less frequently than adults. Further, children’s AVMs were more often deep-located and less often cortical-located than those of adults. We theorized that gradual microhemorrhages from AVMs and the consequent build-up of hemosiderin in the adjacent brain parenchyma might lead to the development of epilepsy over time. Cortical epileptogenic AVMs would therefore come to clinical attention later in life, and their proportion would be lower in children than in adults whereas the proportion of hemorrhagically presenting deep AVMs would be higher. In Study II, we measured how the Spetzler-Ponce Classification (SPC), admission age, symptomatic epilepsy, and repeated ruptures affect the long-term quality of life, as measured with the 15D HRQOL instrument in patients with ruptured AVMs treated in childhood (&lt;20 years of age at the time of admission). The SPC did not predict the long-term HRQOL score. Repeated ruptures affected the HRQOL in several domains. Symptomatic epilepsy significantly reduced the long-term HRQOL. Admission age had a significant effect on one dimension, namely, the patients youngest at admission were not able to carry out their usual daily activities as well as the other patients. Study III analyzed the risk of AVM recurrence in patients treated when under 21 years of age. We found three de-novo AVMs, but one of the patients had been diagnosed with hereditary hemorrhagic telangiectasia (HHT) years after the primary treatment. There were thus two actual recurrent sporadic AVMs giving a recurrence rate of 4.9%. Pediatric patients seem to recover better in the long term, favoring a more aggressive treatment approach. The more frequent hemorrhage presentation does not imply increased hemorrhage risk in the pediatric population; rather it is related to a lower proportion of AVMs coming to clinical attention because of epilepsy. Patients treated for AVMs in childhood or early adulthood should have an imaging follow-up to detect possible later recurrences.Aivojen valtimo-laskimoepämuodostumat (arteriovenoosi malformaatiot, AV-malformaatiot, AVM:t) ovat aivoverisuoniperäisiä oikovirtausmuutoksia. Kolme makroskooppista piirrettä ovat AVM:lle ominaisia: syöttövaltimot, ulosvirtauslaskimot sekä näiden välissä poikkeavista mutkittelevista verisuonista muodostunut suonirykelmä eli ”nidus”. Korkeapaineinen valtimoveri oikovirtaa niduksen läpi suoraan laskimopuolelle ohittaen hiusverisuonet. AV-malformaatioiden ilmaantuvuus on n. 1 / 100 000 henkilövuotta, ilmaantuvuushuippu on 20–40-vuotiailla. Merkittävin AVM:n aiheuttama riski on sen repeytyminen ja aivoverenvuoto, mikä on myös edelleen yleisin AVM:n löytymiseen johtava syy (40–60 % tapauksista). Noin 10–20 % AVM-potilaista on lapsia (<18-vuotiaita) diagnoosihetkellä. Suhteellisesta harvinaisuudestaan huolimatta AVM:t ovat yleisin syy lasten ja nuorten ei-traumaattiseen kallonsisäiseen vuotoon. Tutkimuksen tavoitteena oli selventää lapsuudessa / nuoruudessa hoidettujen AVM-potilaiden erityispiirteitä, pitkänajan ennustetta ja terveyteen liittyvää elämänlaatua sekä arvioida AVM:n uusiutumisriskiä. Tutkimuksessa käytettiin Helsingin neurokirurgian klinikan AVM-tietopankkia, johon on kerätty tiedot kaikista klinikassa 1942–2014 hoidetuista AVM-potilaista (805 potilasta). Tiedot on kerätty jälkikäteen potilaskertomusmerkinnöistä ja kuvista. Vuonna 2016 kaikille elossa oleville > 18-vuotiaille potilaille (n=432) lähetettiin kyselykirjeet. Näistä potilaista 325 (75 %) palautti kyselykirjeen täytettynä. Kirjeet sisälsivät 15D-elämänlaatukyselyn sekä erillisiä kysymyksiä muista oireista, sairauksista, elintavoista, koulutusasteesta ja toimintakyvystä. Kyselykirjeiden vastauksista saatu informaatio yhdistettiin sairaskertomusmerkinnöistä ja kuvantamistutkimuksista saataviin muuttujiin. Lisäksi kuolleiden kuolinsyyt selvitettiin kuolintodistuksista. Ensimmäisessä osatyössä osoitettiin lasten AV-malformaatioiden tulevan useammin aivoverenvuodon ja harvemmin epilepsian vuoksi esille. Lisäksi lapsuudessa esille tulleet AVM:t sijaitsivat useammin aivojen syvissä osissa ja harvemmin aivokuoren seudussa. AV-malformaatioiden ympärillä olevaan aivokudokseen kerääntyy hiljalleen hemosideriiniä mikrovuodoista, mikä lisää epilepsian kehittymisen riskiä aikariippuvaisesti. Tämän vuoksi epilepsiaherkät aivokuoren seudun AVM:t tulevat usein vasta myöhemmällä iällä esille, jolloin syvissä aivojen osissa sijaitsevian AV-malformaatioiden osuus on lapsuudessa suurempi. Toisessa osatyössä analysoitiin 15D-elämänlaatumittarin avulla 10 vuotiaana) diagnosoidut potilaat. Kolmannessa osatyössä tutkittiin alle 21-vuotiaana hoidettujen AV-malformaatioiden uusiutumisriskiä DSA-kuvantamisella. Tutkimuksessa löydettiin kolme uusiutunutta AV-malformaatioita. Yhdellä näistä potilaista oli diagnosoitu alkuperäisen AVM-leikkauksen jälkeen hereditaarinen hemorraginen telangiektasia (HHT). Täten varsinaisia sporadisia AV-malformaatioita todettiin kahdella potilaalla ja näin ollen uusiutumisriski oli 4,9 %. Lapsipotilaat näyttävät toipuvan paremmin kuin aikuiset, minkä vuoksi aktiivisempi hoitolinja on perusteltua. Lasten AVM:t tulevat aikuisia useammin vuodon vuoksi esille, mutta vuotoriski ei ole aikuisia suurempi. AVM:n aiheuttama epilepsia puhkeaa myöhemmällä iällä, minkä vuoksi vuotona esille tulleiden AV-malformaatioiden osuus on suurempi lapsilla kuin aikuisilla. Lapsuudessa tai varhaisaikuisuudessa hoidetuille AVM-potilaille tulisi järjestää kuvantamisseuranta mahdollisten uusiutuneiden AV-malformaatioiden todentamiseksi

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

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

Peer reviewe

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

BackgroundPreviously 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.MethodsControl 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.ResultsA 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.ConclusionsPediatric and adolescent patients are prone to develop recurrent AVMs, even after complete AVM obliteration verified by angiography. Therefore, imaging follow-up is recommended.Peer reviewe