Malformacje naczyniowe ośrodkowego układu nerwowego
Rokowania, prognozy i postęp choroby
Malformacje naczyniowe ośrodkowego układu nerwowego (OUN) stanowią heterogenną grupę patologii, których roczne ryzyko krwawienia wynosi około 2-4%, a w przypadku malformacji tętniczo-żylnych mózgu (bAVM) około 12%. Najsilniejszym predyktorem krwawienia jest wcześniejszy epizod krwotoku, z ryzykiem ponownego krwawienia sięgającym 6-25% rocznie w ciągu pierwszych pięciu lat, szczególnie wysokim w pierwszym roku. Skala Spetzlera-Martina (SM) pozostaje standardem w klasyfikacji bAVM i przewidywaniu wyników leczenia, gdzie chirurgia jest preferowana dla zmian stopnia I i II, a radiochirurgia dla małych zmian, natomiast zmiany stopnia IV i V zwykle leczone są zachowawczo. W embolizacji wewnątrznaczyniowej skuteczność prognostyczną wykazują skale Toronto (AUROC 0,905) i AVMEPRS (AUROC 0,751). Czynniki prognostyczne dla obliteracji po radiochirurgii stereotaktycznej obejmują wiek pacjenta, rozmiar gniazda (1,4-1,8 cm związany z 76,5% prawdopodobieństwem obliteracji), objętość, dawkę promieniowania oraz stopień w skali SM.
- Prognostyka malformacji naczyniowych ośrodkowego układu nerwowego (OUN)
- Czynniki prognostyczne w malformacjach tętniczo-żylnych
- Skale prognostyczne w leczeniu malformacji tętniczo-żylnych
- Czynniki wpływające na prognozę w radiochirurgii
- Wyniki leczenia malformacji naczyniowych pnia mózgu
- Wyniki badania ARUBA i konsekwencje dla prognozy
- Wyniki leczenia malformacji jamistych u dzieci
- Przyszłość prognozowania w malformacjach naczyniowych OUN
Prognostyka malformacji naczyniowych ośrodkowego układu nerwowego (OUN)
Malformacje naczyniowe ośrodkowego układu nerwowego (OUN) stanowią zróżnicowaną grupę patologii naczyniowych, obejmującą szereg jednostek chorobowych o różnym znaczeniu diagnostycznym i terapeutycznym. Postępy w obrazowaniu neuronaczyniowym doprowadziły do częstszego wykrywania tych zmian. Zrozumienie patofizjologii, naturalnego przebiegu oraz czynników predykcyjnych ryzyka pęknięcia jest kluczowe dla klinicystów podejmujących decyzje terapeutyczne.1
Czynniki prognostyczne w malformacjach tętniczo-żylnych
Roczne ryzyko krwawienia w malformacjach naczyniowych OUN wynosi według aktualnej literatury około 24%. Najsilniejszym predyktorem krwawienia jest historia wcześniejszego krwotoku, ze szacowanym 625% rocznym ryzykiem ponownego krwawienia w ciągu pierwszych pięciu lat, przy czym pierwszy rok niesie najwyższe ryzyko. Jeśli nie występują żadne z tych czynników wysokiego ryzyka, ryzyko krwotoku szacuje się na mniej niż 1%.2
W przypadku malformacji tętniczo-żylnych mózgu (bAVM) roczne ryzyko krwotoku śródczaszkowego szacuje się na około 12%. Głównym czynnikiem predykcyjnym powrotu do zdrowia po interwencji jest status kliniczny pacjenta przed interwencją. Niemniej jednak, korzystne wyniki są często obserwowane nawet u pacjentów z długotrwałymi i ciężkimi objawami.3
Skale prognostyczne w leczeniu malformacji tętniczo-żylnych
Skala Spetzlera-Martina (SM) pozostaje złotym standardem w klasyfikacji malformacji tętniczo-żylnych i przewidywaniu wyników leczenia. Uwzględnia ona rozmiar zmiany, lokalizację i drenaż żylny. Według tej skali, chirurgia jest metodą z wyboru dla zmian stopnia I i II, natomiast radiochirurgia stanowi alternatywę dla małych zmian w zależności od lokalizacji lub innych cech anatomicznych. Zmiany stopnia IV i V są zwykle leczone zachowawczo.4
Uzupełniająca skala S-M (S-M Supp) wprowadza punktację graniczną 6 jako kryterium operacyjności malformacji tętniczo-żylnych i jest lepszym predyktorem pooperacyjnych wyników neurologicznych.5
W kontekście embolizacji wewnątrznaczyniowej, kilka skal prognostycznych wykazuje dobrą skuteczność w przewidywaniu wyników:
- Skala Toronto wykazuje najlepszą skuteczność w przewidywaniu wyleczenia radiologicznego (AUROC 0,905)
- Skala AVM Embolization Prognostic Risk Score (AVMEPRS) najlepiej przewiduje powikłania neurologiczne (AUROC 0,751)
- Skale Toronto, AVMEPRS i AVMES wykazują ogólnie najlepszą skuteczność prognostyczną
Czynniki wpływające na prognozę w radiochirurgii
W ocenie czynników prognostycznych związanych z obliteracją gniazda naczyniowego po radiochirurgii stereotaktycznej (SRS) dla mózgowych malformacji tętniczo-żylnych, najważniejszymi czynnikami od najbardziej do najmniej istotnych są: wiek pacjenta, rozmiar gniazda, objętość, całkowita dawka promieniowania i stopień w skali SM.7
Analiza wykazała, że rozmiar gniazda, objętość i całkowita dawka, wraz z innymi czynnikami klinicznymi, były istotnymi predyktorami obliteracji. Dla pacjentów z rozmiarem gniazda od 1,4 do 1,8 cm, model predykcyjny wskazywał na wysokie prawdopodobieństwo obliteracji wynoszące 76,5%.8
Wyniki leczenia malformacji naczyniowych pnia mózgu
W prospektywnym badaniu obserwacyjnym dotyczącym nieleczonych operacyjnie malformacji jamistych pnia mózgu wyniki neurologiczne poprawiły się lub pozostały bez zmian u większości pacjentów (89,8%), a śmiertelność wyniosła 1,7%, co wydaje się korzystne.9
Cechy radiologiczne istotnie przewidywały pogorszenie wyników. Odsetek pogorszonych wyników znacząco wzrastał wraz z liczbą prospektywnych krwotoków (od 1,5% przy braku krwawienia do 37,5% przy jednym incydencie i 38,5% przy powtarzających się incydentach).10
W analizie jednowymiarowej, istotnymi czynnikami ryzyka pogorszenia wyników były:
- Rozmiar zmiany (na każdy 1 mm) (ryzyko względne (RR) 2,343, p<0,001)
- Przekraczanie punktu osiowego (RR 2,993, p<0,001)
- Obecność żylaków rozwojowych (DVA) (RR 1,801, p=0,020)
- Głębokość zmiany (RR 1,595, p=0,021)
W analizie wielowymiarowej z uwzględnieniem czasu obserwacji, przekraczanie punktu osiowego (RR 2,325, 95% CI 1,332-4,060, p=0,003) oraz obecność żylaków rozwojowych (RR 1,776, 95% CI 1,037-3,041, p=0,036) były istotnie związane z pogorszeniem wyników neurologicznych.12
Wyniki badania ARUBA i konsekwencje dla prognozy
Badanie ARUBA (Randomized trial on cerebral Arteriovenous Malformation) z 2014 roku wykazało, że pacjenci leczeni przy użyciu powszechnych strategii interwencyjnych mają trzy razy większe prawdopodobieństwo wystąpienia udaru lub zgonu w porównaniu z osobami leczonymi zachowawczo (redukcja ciśnienia tętniczego).13
Z badania wynika, że istnieje luka lub słabość modalności interwencyjnych w leczeniu malformacji tętniczo-żylnych o wysokim stopniu w skali SM. Wielu pacjentów z mózgowymi AVM czerpie mniejsze korzyści z obecnie dominujących metod leczenia, które wiążą się z wysokim ryzykiem, kosztami i intensywnymi procedurami, a nawet jeśli ostatecznie są skuteczne, to ich ukończenie zajmuje lata.14
Pojawia się wyraźny konsensus – konieczne są dalsze badania w celu określenia optymalnego postępowania w przypadku niepękniętych AVM, szczególnie tych o stopniu 3 w skali SM. Dwie wiodące opcje interwencyjne dla zmian średnich do dużych to obecnie leczenie wielomodalne i etapowe.15
Wyniki leczenia malformacji jamistych u dzieci
Mózgowe malformacje jamiste (CM) to zmiany naczyniowe o wolnym przepływie, które dotykają do 0,5% populacji pediatrycznej. Zmiany te są narażone na krwawienie, powodowanie napadów padaczkowych i prowadzenie do deficytów neurologicznych.16
Analiza opublikowanej literatury pokazuje, że interwencja chirurgiczna powinna być rozważana jako terapia pierwszego wyboru dla pacjentów, którzy mają objawy związane z CM, prezentują napady padaczkowe i mają zmiany dostępne chirurgicznie.17
Pacjenci pediatryczni charakteryzują się zwiększoną plastycznością mózgu w porównaniu do pacjentów dorosłych i mogą mieć udaną rekonwalescencję, nawet jeśli wystąpi natychmiastowa zachorowalność pooperacyjna. U pacjentów leczonych radiochirurgią stereotaktyczną przeżycie bez krwawienia jest znacznie lepsze u dzieci w porównaniu do dorosłych.18
Analiza zbiorcza wykazała, że 85,2% pacjentów z padaczką było w klasie 1 według Engela po operacji. W ciągu średniego okresu obserwacji wynoszącego 4,1 roku, tylko 3,4% pacjentów miało dodatkowe deficyty neurologiczne, w tym niedowłady i zaburzenia mowy.19
Dla pacjentów, którzy nie przeszli operacji po pierwszej prezentacji, ryzyko przyszłego krwawienia i następstw neurologicznych pozostaje. Badanie dotyczące prospektywnego ryzyka krwawienia wykazało, że roczne wskaźniki krwawienia dla: pacjentów początkowo prezentujących krwawienie, pacjentów z objawami niezwiązanymi z krwawieniem i pacjentów z CM jako znaleziskiem przypadkowym wynosiły odpowiednio 6%, 2% i 0,3%.20
Przyszłość prognozowania w malformacjach naczyniowych OUN
Zrozumienie podstawowej patofizjologii i odróżnienie malformacji naczyniowych od ich imitacji są kluczowymi czynnikami dla sukcesu we wczesnym rozpoznaniu, terminowym leczeniu i systematycznym nadzorze u takich pacjentów.21
Przyszłe badania są potrzebne, aby pogłębić nasze zrozumienie patobiologii malformacji naczyniowych OUN w celu opracowania solidnych modeli prognostycznych dla stratyfikacji osób wysokiego ryzyka w kierunku stosowania spersonalizowanych interwencji.22
Kilka badań analizowało różne czynniki prognostyczne do przewidywania wyników związanych z udaną okluzją po radiochirurgii mózgowych AVM przy użyciu konwencjonalnego podejścia uczenia maszynowego.23 Modele predykcyjne identyfikują główne czynniki przyczyniające się do prognozy obliteracji gniazda po radiochirurgii bazującej na akceleratorze liniowym w przypadku mózgowych AVM.24
Brak jednoznacznego wyboru leczenia dla patologii o częstości występowania około 18/100 000 u dorosłych, odpowiedzialnej za 4% wszystkich pierwotnych krwotoków śródmózgowych, stanowi motywację do dalszych badań.25
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Materiały źródłowe
- #1 Central nervous system vascular malformations: A clinical reviewhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7886037/
CNS vascular malformation is an umbrella term that encompasses a wide variety of pathologies, with a wide range of therapeutic and diagnostic importance. […] Advances in neurovascular imaging along with increased utilization of these advances, have resulted in more frequent identification of these lesions. […] This review covers intracranial and spinal cord vascular malformations and discusses syndromes associated with CNS vascular malformations. […] Patients with CNS vascular lesions can present with a variety of presentations from headache and seizure to isolated cranial nerve deficits and progressive motor and sensory alterations. This highlights the importance of high clinical suspicion and early detection to reduce future risk of complications. […] Understanding the pathophysiology, natural history, and characteristics predictive of the risk of rupture are all essential for clinicians when making appropriate therapeutic decisions.
- #2 Central nervous system vascular malformations: A clinical reviewhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7886037/
The current literature reports a 24% annual risk of rupture. […] The strongest predictor for hemorrhage is a history of bleeding in the past, with the estimated 625% annual rebleeding risk within the first five years, with the first year carrying the highest risk. […] If none of these high-risk characteristics are present, the risk of hemorrhage is estimated at less than 1%. […] The annual risk of intracranial hemorrhage is predicted ~12% many studies have assessed variables to predict risk with mixed results. […] The main predictor for postintervention recovery is the preintervention clinical status. Nonetheless, favorable outcomes are commonly seen even in patients with longstanding and severe symptoms. […] Understanding the underlying pathophysiology and differentiating vascular malformations from mimics are the key factors for success in early recognition, timely treatment, and systematic surveillance in such patients. […] Future studies are needed to advance our understanding on pathobiology of CNS vascular malformations in order to develop robust prognostic models for the stratification of high-risk individuals towards applying personalized interventions.
- #3 Central nervous system vascular malformations: A clinical reviewhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7886037/
The current literature reports a 24% annual risk of rupture. […] The strongest predictor for hemorrhage is a history of bleeding in the past, with the estimated 625% annual rebleeding risk within the first five years, with the first year carrying the highest risk. […] If none of these high-risk characteristics are present, the risk of hemorrhage is estimated at less than 1%. […] The annual risk of intracranial hemorrhage is predicted ~12% many studies have assessed variables to predict risk with mixed results. […] The main predictor for postintervention recovery is the preintervention clinical status. Nonetheless, favorable outcomes are commonly seen even in patients with longstanding and severe symptoms. […] Understanding the underlying pathophysiology and differentiating vascular malformations from mimics are the key factors for success in early recognition, timely treatment, and systematic surveillance in such patients. […] Future studies are needed to advance our understanding on pathobiology of CNS vascular malformations in order to develop robust prognostic models for the stratification of high-risk individuals towards applying personalized interventions.
- #4 Cerebral Arteriovenous Malformation from Classification to the Management | IntechOpenhttps://www.intechopen.com/chapters/67582
The surgical excision of cAVM gives definitive therapy in cAVM patients, radiosurgery is curative in selected cAVM patients and cerebral angiographic embolization is rarely a sole therapy and usually in combination with surgical therapy. […] Spetzler-Martin grading scale classifies surgical risk of removing cAVM according to its size, location and the presence of deep venous drainage. According to S-M grading, surgery is the modality of choice for grade I and II lesions, with radiosurgery as an alternative for small lesions based on location or other vascular anatomic features, while grade IV and V lesions are generally managed conservatively. S-M Supp grading gives a cut-off scale of 6 for operability of cAVM, and it is also a better predictor for post-cAVM excision neurological outcome.
- #5 Cerebral Arteriovenous Malformation from Classification to the Management | IntechOpenhttps://www.intechopen.com/chapters/67582
The surgical excision of cAVM gives definitive therapy in cAVM patients, radiosurgery is curative in selected cAVM patients and cerebral angiographic embolization is rarely a sole therapy and usually in combination with surgical therapy. […] Spetzler-Martin grading scale classifies surgical risk of removing cAVM according to its size, location and the presence of deep venous drainage. According to S-M grading, surgery is the modality of choice for grade I and II lesions, with radiosurgery as an alternative for small lesions based on location or other vascular anatomic features, while grade IV and V lesions are generally managed conservatively. S-M Supp grading gives a cut-off scale of 6 for operability of cAVM, and it is also a better predictor for post-cAVM excision neurological outcome.
- #6 Outcome prediction for treatment of brain arteriovenous malformations: performance of endovascular predictive scores in a single-center population – PubMedhttps://pubmed.ncbi.nlm.nih.gov/40139784/
Endovascular embolization is an accepted treatment modality for brain arteriovenous malformations (bAVM); however, treatment outcomes are highly variable, warranting accurate prediction for adequate patient selection. […] The rates of radiological cure and neurological complications were 18.2% and 14.1%, respectively. […] In the overall analysis, the Toronto score showed the best performance for radiological cure (AUROC 0.905). […] The sensitivity analysis showed improved performance of most scores. […] The Toronto score exhibited the highest performance for radiological cure (AUROC 0.857). […] The AVM Embolization Prognostic Risk Score (AVMEPRS) showed the highest performance for neurological complications (AUROC 0.751). […] Among the selected scores, the Toronto, AVMEPRS, and AVMES scores showed the best performances.
- #7 Feature Analysis of Predictors Affecting the Nidus Obliteration of Linear Accelerator-Based Radiosurgery for Arteriovenous Malformations Using Explainable Predictive Modelinghttps://www.mdpi.com/2076-3417/13/7/4267
This study aimed to evaluate prognostic factors associated with nidus obliteration following stereotactic radiosurgery (SRS) for cerebral arteriovenous malformations. […] The prognostic factors affecting nidus obliteration from most to least important were age, nidus size, volume, total prescription dose, and SM grade, using a predictive model. […] The predictive model identified the main contributing factors associated with a prognostic of nidus obliteration from linear accelerator-based SRS for cerebral AVM. It was confirmed that the results, including the prognostic factors, are potentially useful for outcome prediction for patient and treatment. […] Several studies have analyzed various prognostic factors for outcome prediction related to successful occlusion after radiosurgery of cerebral AVM with a conventional machine learning approach.
- #8 Feature Analysis of Predictors Affecting the Nidus Obliteration of Linear Accelerator-Based Radiosurgery for Arteriovenous Malformations Using Explainable Predictive Modelinghttps://www.mdpi.com/2076-3417/13/7/4267
The major prognostic factors including the size, anatomical location, and SM grade of the lesion used by the authors are important to considered when planning radiosurgery in this study. […] The analysis revealed that nidus size, volume, and total dose, along with other clinical factors, were significant predictors of obliteration. […] For patients with a nidus size ranging from 1.4 to 1.8 cm, the predictive model yielded a high obliteration probability of 76.5%.
- #9 Neurological outcomes of untreated brainstem cavernous malformations in a prospective observational cohort and literature review | Stroke and Vascular Neurologyhttps://svn.bmj.com/content/6/4/501
The 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. […] 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)). […] The univariately significant risk factors for worsened outcome are lesion size (per 1mm) (relative risk (RR) 2.343, p0.001), crossing the axial point (RR 2.993, p0.001), DVA (RR 1.801, p=0.020) and depth (RR 1.595, p=0.021). […] In the multivariate analysis (Enter method) with adjustment for duration of follow-up, crossing the axial point (RR 2.325, 95% CI 1.332 to 4.060, p=0.003) and DVA (RR 1.776, 95%CI 1.037 to 3.041, p=0.036) were significantly related to worsened neurological outcomes. […] Overall, we performed a pooled analysis of nine studies, and the proportion of improved/unchanged and worsened outcomes was 85.2% (465 of 546) and 14.3% (78 of 546), respectively, which was analogous to our series and appeared more benign than previously thought.
- #10 Neurological outcomes of untreated brainstem cavernous malformations in a prospective observational cohort and literature review | Stroke and Vascular Neurologyhttps://svn.bmj.com/content/6/4/501
The 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. […] 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)). […] The univariately significant risk factors for worsened outcome are lesion size (per 1mm) (relative risk (RR) 2.343, p0.001), crossing the axial point (RR 2.993, p0.001), DVA (RR 1.801, p=0.020) and depth (RR 1.595, p=0.021). […] In the multivariate analysis (Enter method) with adjustment for duration of follow-up, crossing the axial point (RR 2.325, 95% CI 1.332 to 4.060, p=0.003) and DVA (RR 1.776, 95%CI 1.037 to 3.041, p=0.036) were significantly related to worsened neurological outcomes. […] Overall, we performed a pooled analysis of nine studies, and the proportion of improved/unchanged and worsened outcomes was 85.2% (465 of 546) and 14.3% (78 of 546), respectively, which was analogous to our series and appeared more benign than previously thought.
- #11 Neurological outcomes of untreated brainstem cavernous malformations in a prospective observational cohort and literature review | Stroke and Vascular Neurologyhttps://svn.bmj.com/content/6/4/501
The 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. […] 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)). […] The univariately significant risk factors for worsened outcome are lesion size (per 1mm) (relative risk (RR) 2.343, p0.001), crossing the axial point (RR 2.993, p0.001), DVA (RR 1.801, p=0.020) and depth (RR 1.595, p=0.021). […] In the multivariate analysis (Enter method) with adjustment for duration of follow-up, crossing the axial point (RR 2.325, 95% CI 1.332 to 4.060, p=0.003) and DVA (RR 1.776, 95%CI 1.037 to 3.041, p=0.036) were significantly related to worsened neurological outcomes. […] Overall, we performed a pooled analysis of nine studies, and the proportion of improved/unchanged and worsened outcomes was 85.2% (465 of 546) and 14.3% (78 of 546), respectively, which was analogous to our series and appeared more benign than previously thought.
- #12 Neurological outcomes of untreated brainstem cavernous malformations in a prospective observational cohort and literature review | Stroke and Vascular Neurologyhttps://svn.bmj.com/content/6/4/501
The 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. […] 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)). […] The univariately significant risk factors for worsened outcome are lesion size (per 1mm) (relative risk (RR) 2.343, p0.001), crossing the axial point (RR 2.993, p0.001), DVA (RR 1.801, p=0.020) and depth (RR 1.595, p=0.021). […] In the multivariate analysis (Enter method) with adjustment for duration of follow-up, crossing the axial point (RR 2.325, 95% CI 1.332 to 4.060, p=0.003) and DVA (RR 1.776, 95%CI 1.037 to 3.041, p=0.036) were significantly related to worsened neurological outcomes. […] Overall, we performed a pooled analysis of nine studies, and the proportion of improved/unchanged and worsened outcomes was 85.2% (465 of 546) and 14.3% (78 of 546), respectively, which was analogous to our series and appeared more benign than previously thought.
- #13 Advocating Intraluminal Radiation Therapy in Cerebral Arteriovenous Malformation Treatment | IntechOpenhttps://www.intechopen.com/chapters/69610
In 2014, ARUBA (a randomized trial on cerebral Arteriovenous Malformation AVM) found patients treated using prevalent interventional strategies are three times more likely to suffer a stroke/die compared with those treated conservatively (blood pressure reduction). […] A clear accord emerges. There is, indeed, a lacuna or at least weakness of interventional modalities when addressing high SM grade AVMs. […] To conclude, many patients with cerebral AVM benefit less from current prevalent treatment modalities that carry high risks, costs, and intensive procedures and, even if eventually effective, take years to complete. […] The lack of clear treatment choice for a pathology with a point prevalence of ~18/100,000 in adults responsible for 4% of all primary intracerebral hemorrhages is the motivation for this review.
- #14 Advocating Intraluminal Radiation Therapy in Cerebral Arteriovenous Malformation Treatment | IntechOpenhttps://www.intechopen.com/chapters/69610
In 2014, ARUBA (a randomized trial on cerebral Arteriovenous Malformation AVM) found patients treated using prevalent interventional strategies are three times more likely to suffer a stroke/die compared with those treated conservatively (blood pressure reduction). […] A clear accord emerges. There is, indeed, a lacuna or at least weakness of interventional modalities when addressing high SM grade AVMs. […] To conclude, many patients with cerebral AVM benefit less from current prevalent treatment modalities that carry high risks, costs, and intensive procedures and, even if eventually effective, take years to complete. […] The lack of clear treatment choice for a pathology with a point prevalence of ~18/100,000 in adults responsible for 4% of all primary intracerebral hemorrhages is the motivation for this review.
- #15 Advocating Intraluminal Radiation Therapy in Cerebral Arteriovenous Malformation Treatment | IntechOpenhttps://www.intechopen.com/chapters/69610
The SM grading system for predicting patient outcome is microsurgery-based and remains the gold standard in the field. […] The main advantage of AVM microsurgery is its straightforward approach, which allows relatively definite lesion resection and rapid clear follow-up. […] The ARUBA trial, published in 2014, compared interventional therapy with medical management of unruptured brain AVMs. […] One clear consensus emergesfurther research is advocated to delineate the optimal management of unruptured AVMs, particularly those with SM grade3. […] We feel that one possible cause is the limitations inherent in present approaches that lead to diminishing returns with every new improvement (necessitating ever-increasing technological and financial investments). […] The two leading avenues of interventional choice for medium-to-large lesions are currently multi-modal and staged treatment. […] If proven feasible, low-radiation implants could add several unique benefits to AVM treatment and we advocate studying their use.
- #16https://journals.lww.com/brci/fulltext/2022/08030/presentation_and_management_of_nervous_system.2.aspx
Cerebral cavernous malformations (CMs) are slow-flow vascular lesions that affect up to 0.5% of the pediatric population. These lesions are at risk for hemorrhage, causing seizures, and leading to neurological deficits. […] Our analysis of published literature shows surgical intervention should be considered first-line therapy for patients who are symptomatic from CM, present with seizure, and have surgically accessible lesions. […] Pediatric patients are known to have increased brain plasticity compared to adult patients, and can have a successful recovery even if immediate surgical morbidity occurs. Indeed, in patients treated with stereotactic radiosurgery, hemorrhage-free survival is markedly better in children compared to adults. […] Our pooled analysis revealed that 85.2% of patients who had epilepsy were Engel Class 1 after surgery. Over a mean follow-up of 4.1 years, only 3.4% of patients had additional neurological deficits, including paresis and speech deficits.
- #17https://journals.lww.com/brci/fulltext/2022/08030/presentation_and_management_of_nervous_system.2.aspx
Cerebral cavernous malformations (CMs) are slow-flow vascular lesions that affect up to 0.5% of the pediatric population. These lesions are at risk for hemorrhage, causing seizures, and leading to neurological deficits. […] Our analysis of published literature shows surgical intervention should be considered first-line therapy for patients who are symptomatic from CM, present with seizure, and have surgically accessible lesions. […] Pediatric patients are known to have increased brain plasticity compared to adult patients, and can have a successful recovery even if immediate surgical morbidity occurs. Indeed, in patients treated with stereotactic radiosurgery, hemorrhage-free survival is markedly better in children compared to adults. […] Our pooled analysis revealed that 85.2% of patients who had epilepsy were Engel Class 1 after surgery. Over a mean follow-up of 4.1 years, only 3.4% of patients had additional neurological deficits, including paresis and speech deficits.
- #18https://journals.lww.com/brci/fulltext/2022/08030/presentation_and_management_of_nervous_system.2.aspx
Cerebral cavernous malformations (CMs) are slow-flow vascular lesions that affect up to 0.5% of the pediatric population. These lesions are at risk for hemorrhage, causing seizures, and leading to neurological deficits. […] Our analysis of published literature shows surgical intervention should be considered first-line therapy for patients who are symptomatic from CM, present with seizure, and have surgically accessible lesions. […] Pediatric patients are known to have increased brain plasticity compared to adult patients, and can have a successful recovery even if immediate surgical morbidity occurs. Indeed, in patients treated with stereotactic radiosurgery, hemorrhage-free survival is markedly better in children compared to adults. […] Our pooled analysis revealed that 85.2% of patients who had epilepsy were Engel Class 1 after surgery. Over a mean follow-up of 4.1 years, only 3.4% of patients had additional neurological deficits, including paresis and speech deficits.
- #19https://journals.lww.com/brci/fulltext/2022/08030/presentation_and_management_of_nervous_system.2.aspx
Cerebral cavernous malformations (CMs) are slow-flow vascular lesions that affect up to 0.5% of the pediatric population. These lesions are at risk for hemorrhage, causing seizures, and leading to neurological deficits. […] Our analysis of published literature shows surgical intervention should be considered first-line therapy for patients who are symptomatic from CM, present with seizure, and have surgically accessible lesions. […] Pediatric patients are known to have increased brain plasticity compared to adult patients, and can have a successful recovery even if immediate surgical morbidity occurs. Indeed, in patients treated with stereotactic radiosurgery, hemorrhage-free survival is markedly better in children compared to adults. […] Our pooled analysis revealed that 85.2% of patients who had epilepsy were Engel Class 1 after surgery. Over a mean follow-up of 4.1 years, only 3.4% of patients had additional neurological deficits, including paresis and speech deficits.
- #20https://journals.lww.com/brci/fulltext/2022/08030/presentation_and_management_of_nervous_system.2.aspx
For patients who do not undergo surgery after the first presentation, the risk for future hemorrhage and neurological sequela remains. A study on prospective hemorrhage risk found that the annual rates of hemorrhage for: patients initially presenting with hemorrhage, patients with symptoms not related to hemorrhage, and patients with CM as an incidental finding were 6%, 2%, and 0.3%, respectively.
- #21 Central nervous system vascular malformations: A clinical reviewhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7886037/
The current literature reports a 24% annual risk of rupture. […] The strongest predictor for hemorrhage is a history of bleeding in the past, with the estimated 625% annual rebleeding risk within the first five years, with the first year carrying the highest risk. […] If none of these high-risk characteristics are present, the risk of hemorrhage is estimated at less than 1%. […] The annual risk of intracranial hemorrhage is predicted ~12% many studies have assessed variables to predict risk with mixed results. […] The main predictor for postintervention recovery is the preintervention clinical status. Nonetheless, favorable outcomes are commonly seen even in patients with longstanding and severe symptoms. […] Understanding the underlying pathophysiology and differentiating vascular malformations from mimics are the key factors for success in early recognition, timely treatment, and systematic surveillance in such patients. […] Future studies are needed to advance our understanding on pathobiology of CNS vascular malformations in order to develop robust prognostic models for the stratification of high-risk individuals towards applying personalized interventions.
- #22 Central nervous system vascular malformations: A clinical reviewhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7886037/
The current literature reports a 24% annual risk of rupture. […] The strongest predictor for hemorrhage is a history of bleeding in the past, with the estimated 625% annual rebleeding risk within the first five years, with the first year carrying the highest risk. […] If none of these high-risk characteristics are present, the risk of hemorrhage is estimated at less than 1%. […] The annual risk of intracranial hemorrhage is predicted ~12% many studies have assessed variables to predict risk with mixed results. […] The main predictor for postintervention recovery is the preintervention clinical status. Nonetheless, favorable outcomes are commonly seen even in patients with longstanding and severe symptoms. […] Understanding the underlying pathophysiology and differentiating vascular malformations from mimics are the key factors for success in early recognition, timely treatment, and systematic surveillance in such patients. […] Future studies are needed to advance our understanding on pathobiology of CNS vascular malformations in order to develop robust prognostic models for the stratification of high-risk individuals towards applying personalized interventions.
- #23 Feature Analysis of Predictors Affecting the Nidus Obliteration of Linear Accelerator-Based Radiosurgery for Arteriovenous Malformations Using Explainable Predictive Modelinghttps://www.mdpi.com/2076-3417/13/7/4267
This study aimed to evaluate prognostic factors associated with nidus obliteration following stereotactic radiosurgery (SRS) for cerebral arteriovenous malformations. […] The prognostic factors affecting nidus obliteration from most to least important were age, nidus size, volume, total prescription dose, and SM grade, using a predictive model. […] The predictive model identified the main contributing factors associated with a prognostic of nidus obliteration from linear accelerator-based SRS for cerebral AVM. It was confirmed that the results, including the prognostic factors, are potentially useful for outcome prediction for patient and treatment. […] Several studies have analyzed various prognostic factors for outcome prediction related to successful occlusion after radiosurgery of cerebral AVM with a conventional machine learning approach.
- #24 Feature Analysis of Predictors Affecting the Nidus Obliteration of Linear Accelerator-Based Radiosurgery for Arteriovenous Malformations Using Explainable Predictive Modelinghttps://www.mdpi.com/2076-3417/13/7/4267
This study aimed to evaluate prognostic factors associated with nidus obliteration following stereotactic radiosurgery (SRS) for cerebral arteriovenous malformations. […] The prognostic factors affecting nidus obliteration from most to least important were age, nidus size, volume, total prescription dose, and SM grade, using a predictive model. […] The predictive model identified the main contributing factors associated with a prognostic of nidus obliteration from linear accelerator-based SRS for cerebral AVM. It was confirmed that the results, including the prognostic factors, are potentially useful for outcome prediction for patient and treatment. […] Several studies have analyzed various prognostic factors for outcome prediction related to successful occlusion after radiosurgery of cerebral AVM with a conventional machine learning approach.
- #25 Advocating Intraluminal Radiation Therapy in Cerebral Arteriovenous Malformation Treatment | IntechOpenhttps://www.intechopen.com/chapters/69610
In 2014, ARUBA (a randomized trial on cerebral Arteriovenous Malformation AVM) found patients treated using prevalent interventional strategies are three times more likely to suffer a stroke/die compared with those treated conservatively (blood pressure reduction). […] A clear accord emerges. There is, indeed, a lacuna or at least weakness of interventional modalities when addressing high SM grade AVMs. […] To conclude, many patients with cerebral AVM benefit less from current prevalent treatment modalities that carry high risks, costs, and intensive procedures and, even if eventually effective, take years to complete. […] The lack of clear treatment choice for a pathology with a point prevalence of ~18/100,000 in adults responsible for 4% of all primary intracerebral hemorrhages is the motivation for this review.