Malformacja naczyniowa tętniczo-żylna mózgu
Epidemiologia
Malformacja naczyniowa tętniczo-żylna mózgu (AVM) to rzadka, wrodzona patologia naczyniowa charakteryzująca się bezpośrednim połączeniem tętnic i żył bez udziału naczyń włosowatych. Występowanie AVM szacuje się na 0,89-1,34 przypadków na 100 000 osobolat, z zapadalnością na objawowe zmiany około 0,94/100 000 osobolat (95% CI 0,57-1,30). Chorobowość punktowa wynosi około 18/100 000 dorosłych (95% CI 16-24). AVM najczęściej manifestują się klinicznie między 10. a 40. rokiem życia, z ryzykiem krwotoku rocznym 2-4%, które może wzrosnąć do 6-18% w pierwszym roku po incydencie. Czynniki ryzyka krwotoku obejmują mały rozmiar AVM, głębokie drenaże żylne, lokalizacje podnamiotowe, obecność tętniaków, nadciśnienie tętnicze oraz wysokie ciśnienie w tętnicy zasilającej. Śmiertelność po pęknięciu AVM wynosi 10-15%, a wskaźniki niepełnosprawności 30-50%. AVM odpowiadają za około 2% wszystkich i 4% krwotocznych udarów mózgu, szczególnie u młodych dorosłych.
Epidemiologia malformacji naczyniowej tętniczo-żylnej mózgu
Malformacja naczyniowa tętniczo-żylna mózgu (brain AVM) to rzadka choroba naczyniowa, charakteryzująca się nieprawidłowym połączeniem tętnic i żył w mózgu, z pominięciem sieci naczyń włosowatych. Epidemiologia tej jednostki chorobowej jest przedmiotem licznych badań, jednak ze względu na rzadkość występowania oraz obecność bezobjawowych przypadków, dokładne dane populacyjne są trudne do ustalenia.12
Wskaźniki zapadalności
Wskaźnik wykrywalności malformacji naczyniowej tętniczo-żylnej mózgu w populacji ogólnej wynosi od 0,89 do 1,34 przypadków na 100 000 osobolat.12 Dane te pochodzą z różnych badań populacyjnych prowadzonych w Stanach Zjednoczonych, Australii, Szwecji oraz Szkocji. W badaniu New York Islands AVM Study współczynnik wykrywalności określono na 1,34 na 100 000 osobolat.1 Z kolei w szkockim badaniu Scottish Intracranial Vascular Malformation Study (SIVMS) surowy współczynnik zapadalności na AVM mózgu wynosił 1,1 (95% CI 0,9 do 1,4) na 100 000 dorosłych rocznie.12
Najbardziej wiarygodne dane dotyczące występowania choroby wskazują na wskaźnik wykrywalności dla objawowych zmian na poziomie 0,94 na 100 000 osobolat (95% przedział ufności 0,57-1,30/100 000 osobolat). Dane te pochodzą z pojedynczego badania populacyjnego, ale są poparte ponowną analizą innych źródeł danych.12
W badaniu przeprowadzonym w Korei wskaźnik zachorowalności na pęknięte AVM mózgu był wyższy i wynosił 3,5 na 100 000 osobolat (standaryzowany wskaźnik 3,6 na 100 000 osobolat).1
Chorobowość
Chorobowość (rozpowszechnienie) malformacji naczyniowej tętniczo-żylnej mózgu jest trudniejsza do oszacowania ze względu na istnienie przypadków bezobjawowych. Wcześniejsze szacunki oparte na danych z autopsji sugerowały bardzo wysokie wskaźniki (500-600/100 000 populacji), jednak były one obarczone znacznym błędem i obecnie uważa się je za nieuzasadnione.12
Najnowsze dane wskazują, że punktowa chorobowość AVM mózgu wynosi około 18 (95% CI 16-24) na 100 000 dorosłych.12 Określenie to pochodzi z badania przeprowadzonego w regionie Lothian w Szkocji, wykorzystującego metodę capture-recapture z wieloma nakładającymi się źródłami identyfikacji przypadków.1
Chorobowość wykrytej, aktywnej (zagrażającej) choroby AVM jest nieznana, ale na podstawie danych dotyczących zapadalności można wnioskować, że jest niższa niż 10,3 na 100 000 populacji.12
Szacuje się, że w Stanach Zjednoczonych około 300 000 Amerykanów ma AVM, z czego tylko około 12% (około 36 000) będzie wykazywać objawy o różnym nasileniu.12
Czynniki demograficzne
Rozkład wiekowy
Mimo że malformacje naczyniowe tętniczo-żylne mózgu są uważane za wady wrodzone, rzadko są wykrywane przypadkowo u bardzo młodych osób. Opisano również wiele zmian de novo u dorosłych. Uważa się, że mogą one powiększać się z czasem.12
AVM mózgu mogą być diagnozowane w każdym wieku, ale objawy najczęściej pojawiają się po raz pierwszy między 10 a 40 rokiem życia.1 Pomimo przypuszczalnego wrodzonego pochodzenia AVM, prezentacja kliniczna najczęściej występuje u młodych dorosłych.1 Ogólnie są one diagnozowane w średnim wieku 31 lat.12
Jedna trzecia malformacji naczyniowych tętniczo-żylnych, które są diagnozowane z powodu krwotoku, jest identyfikowana przed 20 rokiem życia.1 Z kolei ryzyko objawów jest najwyższe między 30 a 50 rokiem życia.1
Rozkład płci
Większość badań wskazuje, że nie istnieje predylekcja płciowa w przypadku AVM mózgu, co oznacza, że występują one z podobną częstością u mężczyzn i kobiet.123 Jednakże niektóre dane sugerują, że krwotok z powodu pęknięcia AVM może występować częściej u kobiet.1 Inne badania wskazują, że AVM mózgu są częstsze u mężczyzn niż u kobiet.1
W badaniu dotyczącym dziedzicznego krwotocznego zapalenia naczyń krwionośnych (HHT) nie stwierdzono istotnej różnicy w częstości występowania AVM mózgu między mężczyznami (8,5%, 95% CI 4,9%-12,0%) a kobietami (11,0%, 95% CI 5,9%-16,1%).1
Czynniki ryzyka
Czynniki genetyczne
Chociaż większość przypadków AVM mózgu występuje sporadycznie, istnieją rzadkie przypadki rodzinnego występowania. Niektóre zespoły genetyczne są związane ze zwiększonym ryzykiem malformacji naczyniowych tętniczo-żylnych mózgu:12
- Dziedziczne krwotoczne zapalenie naczyń (choroba Oslera-Webera-Rendu) – jest najczęstszą genetyczną przyczyną AVM mózgu i wiąże się z 10-25% ryzykiem rozwoju AVM mózgu w ciągu życia1
- Zespół Wyburna-Masona (kranio-twarzowy tętniczo-żylny zespół metameru)12
- Choroba von Hippla-Lindaua1
- Zespół Sturge’a-Webera1
Pacjenci z dziedzicznym krwotocznym zapaleniem naczyń (HHT) mają znacznie wyższe wskaźniki występowania AVM mózgu niż populacja ogólna. W tej grupie częstość występowania AVM mózgu wynosi około 10,4% (95% CI 7,9%-13,0%). Co istotne, pacjenci z HHT typu 1 (HHT1) mają znacznie wyższy wskaźnik występowania AVM mózgu (13,4%, 95% CI 9,5%-17,4%) w porównaniu z pacjentami z HHT typu 2 (HHT2) (2,4%, 95% CI 1,0%-3,8%) (p < 0,0001).1
Czynniki anatomiczne
AVM mózgu mają tendencję do występowania pojedynczo w zdecydowanej większości przypadków (95%). Gdy są mnogie, należy rozważyć skojarzenia z zespołami, takimi jak dziedziczne krwotoczne zapalenie naczyń (zespół Oslera-Webera-Rendu) i zespół Wyburna-Masona.12
Zidentyfikowano różne czynniki anatomiczne związane z krwotoczną prezentacją AVM. Niezależne czynniki ryzyka prezentacji krwotocznej według analiz wieloczynnikowych obejmują:1
- Mały rozmiar AVM
- Głębokie odwadnianie żylne
- Głębokie, pozastrefowe (poza strefami granicznymi) i podnamiotowe lokalizacje
- Towarzyszące tętniaki
- Nadciśnienie tętnicze
- Mała liczba żył odwadniających
- Ektazje żylne
- Wysokie ciśnienie w tętnicy zasilającej
AVM podnamiotowe są częściej związane z tętniakami tętnic zasilających niż AVM nadnamiotowe (25% vs 5%).1
Ryzyko powikłań
Ryzyko krwotoku
Roczne ryzyko krwotoku u pacjentów z nieleczonymi AVM szacuje się na około 2-4% rocznie.12345 W badaniach populacyjnych 38-70% AVM mózgu początkowo prezentuje się z krwotokami.1
Mniejsze AVM mają większą skłonność do krwawienia, podczas gdy większe AVM częściej powodują napady padaczkowe.1 Ryzyko krwawienia może być wyższe w przypadku niektórych typów AVM. Ryzyko może być również większe u osób, które wcześniej doświadczyły krwotoku z AVM mózgu.1
Badania sugerują, że w pierwszym roku po spontanicznym krwotoku z AVM ryzyko ponownego krwawienia może wynosić nawet 6-18%.1 U dzieci roczne ryzyko ponownego krwawienia wynosi 24%, ale może wynosić nawet 65% w projekcji na całe życie dziecka.1
Śmiertelność i zachorowalność
Wskaźnik śmiertelności po pęknięciu AVM szacuje się na około 10-15%, a wskaźniki zachorowalności (niepełnosprawności) są znacznie wyższe, wynoszące od około 30-50%.123 Ogólne roczne wskaźniki śmiertelności wahały się od 0,7% do 2,9% w różnych populacjach badawczych.12
AVM mózgu odpowiadają za około 2% wszystkich i 4% krwotocznych udarów mózgu. Ofiary są często młodsze niż większość innych pacjentów z udarem, a AVM wyjaśniają jedną trzecią krwotocznych udarów mózgu u młodych dorosłych.1 AVM mózgu są przyczyną około 10% nieurazowych krwotoków podpajęczynówkowych.1
Ostre zdarzenia krwotoczne u dzieci wiążą się z wskaźnikiem śmiertelności dochodzącym do 25%.1
Inne manifestacje kliniczne
Oprócz krwotoku śródczaszkowego, AVM mózgu mogą powodować różne objawy neurologiczne:12
- Napady padaczkowe (18-35% przypadków)
- Bóle głowy (6-14% przypadków)
- Ogniskowe deficyty neurologiczne (3-10% przypadków)
Napady padaczkowe są drugą najczęstszą manifestacją kliniczną AVM, występującą w 11-35% przypadków.1 Czynniki zwiększające ryzyko napadów padaczkowych u pacjentów z AVM obejmują płeć męską, młodszy wiek, AVM płatów czołowych lub skroniowych, AVM zlokalizowane w korze mózgowej, powierzchowne odwadnianie żylne, powierzchowny nidus AVM płata skroniowego, AVM przetokowe oraz AVM ze stenozą żylną.1
U pacjentów z AVM nieprowokowane napady padaczkowe obserwowano w ciągu 5 lat po diagnozie w 26% przypadków, 11% po 10 latach i 18% po 20 latach obserwacji.1
Interesujące jest to, że historię subtelnych zaburzeń uczenia się stwierdza się u 66% dorosłych z AVM. Sugeruje to wczesne efekty, które są w dużej mierze subkliniczne i nie zwracają uwagi medycznej.1
Metody nadzoru i monitorowania malformacji naczyniowej tętniczo-żylnej mózgu
Nadzór obrazowy
Monitorowanie obrazowe AVM mózgu jest kluczowym elementem zarządzania chorobą, szczególnie w przypadkach, gdzie zdecydowano się na podejście zachowawcze. Najbardziej powszechne metody obrazowania wykorzystywane w nadzorze AVM obejmują:1
- Angiografia mózgowa – jest to najbardziej szczegółowe badanie w diagnostyce AVM mózgu. Angiografia mózgowa uwidacznia lokalizację tętnic zasilających i żył drenujących, co ma kluczowe znaczenie dla planowania leczenia.
- Obrazowanie metodą rezonansu magnetycznego (MRI) – jest bardziej czułe niż tomografia komputerowa i może pokazać subtelne zmiany w tkance mózgowej związane z AVM mózgu.
- Tomografia komputerowa (CT) – często stosowana jako badanie wstępne, szczególnie w sytuacjach nagłych.
Długoterminowe obrazowanie kontrolne AVM jest ogólnie odpowiednie, ale harmonogram zależy od rodzaju leczenia i charakterystyki pacjenta.1 Pacjenci będą mieli regularne badania obrazowe nadzorujące i wizyty kontrolne u swojego zespołu leczącego.1
Zarządzanie wielodyscyplinarne
Diagnostyka i zarządzanie AVM mózgu odbywa się z udziałem zespołu interdyscyplinarnego, składającego się z neurochirurga, neurologa, internisty i radiologa interwencyjnego.1 Ta wielodyscyplinarna współpraca jest niezbędna do optymalnego zarządzania tą złożoną chorobą.
Ścieżka zarządzania jest najlepiej określana przez dyskusję wielodyscyplinarną po kompleksowej charakterystyce obrazowej.1 Obserwacja pacjentów jest zwykle prowadzona przez pielęgniarkę praktykującą i lekarza podstawowej opieki zdrowotnej.1
Stratyfikacja ryzyka
Skala Spetzlera-Martina (SM) jest powszechnie stosowana jako system oceny do przewidywania ryzyka chorobowości i śmiertelności chirurgicznej przy AVM mózgu. Jest to złożony wynik rozmiaru nidusa, elokwencji przyległego mózgu i obecności głębokiego drenażu żylnego. Wyższe wyniki sugerują zwiększone ryzyko chorobowości i śmiertelności chirurgicznej.1
Badacze badają sposoby lepszego przewidywania ryzyka krwotoku u osób z AVM mózgu. Może to pomóc w lepszym kierowaniu decyzjami dotyczącymi leczenia.1
Podejścia do zarządzania
Strategie zarządzania AVM mózgu obejmują:12
- Konserwatywny nadzór – może być zalecany, jeśli masz niewiele objawów lub brak objawów, lub jeśli twoja AVM znajduje się w obszarze mózgu, który jest trudny do leczenia.1
- Radiochirurgia stereotaktyczna
- Otwarta resekcja chirurgiczna
- Embolizacja wewnątrznaczyniowa – stosowana jako uzupełniająca interwencja do chirurgii i radiochirurgii stereotaktycznej1
- Kombinacja powyższych metod
Odpowiednie leczenie zależy od wieku, stanu zdrowia oraz rozmiaru i lokalizacji AVM mózgu.1 Zarządzanie AVM mózgu zależy od rozmiaru, lokalizacji, wieku pacjenta i statusu AVM (wysokie ryzyko pęknięcia). Podczas gdy chirurgia jest głównym leczeniem, embolizacja jest inną opcją.1
Trwające postępy w technikach embolizacji, radiochirurgii i mikrochirurgii sprawiają również, że możliwe jest stosowanie chirurgii do leczenia AVM mózgu, które były trudno dostępne w przeszłości.1
Monitorowanie wyników
W badaniu ARUBA (A Randomized Trial of Unruptured Brain Arteriovenous Malformations) zaobserwowano znaczny krajowy spadek interwencji w nieprzerwanej AVM w latach po 2014 roku. W latach 2003-2017 zmniejszenie liczby interwencji w nieprzerwanej AVM było związane ze skorelowanym opóźnionym wzrostem częstości występowania pękniętych AVM. Wyniki te sugerują, że mniejsza liczba leczenia niepękniętych AVM może prowadzić do wzrostu częstości występowania pęknięć AVM.1
Wyniki tych pacjentów zależą od rozmiaru AVM, obecności objawów, lokalizacji, współchorobowości pacjenta i stanu psychicznego. Po operacji często występują powikłania, a rekonwalescencja u wielu pacjentów jest przedłużona, wymagająca intensywnej rehabilitacji. Najważniejszym czynnikiem ryzyka śmierci jest pęknięcie AVM.1
W badaniu dotyczącym bólu głowy u pacjentów z AVM stwierdzono, że mikrochirurgia, radiochirurgia stereotaktyczna i terapia multimodalna miały znaczące korzyści w łagodzeniu bólu głowy w porównaniu z leczeniem zachowawczym. Wyniki te dostarczają ważnych wskazówek dla klinicystów podczas opracowywania opcji leczenia, które mogą pomóc poprawić ogólne wyniki leczenia i jakość życia pacjentów.1
Przeprowadzono niewiele badań dotyczących długoterminowych wyników klinicznych AVM mózgu u pacjentów pediatrycznych. Wcześniej opublikowane badania wykorzystywały konwencjonalne metryki, które zostały zwalidowane w populacji dorosłych, takie jak zmodyfikowana skala Rankina. Chociaż te metryki mogą służyć jako rozsądne surogaty, dokładne zrozumienie ogólnej jakości życia związanej ze zdrowiem zależy od wykorzystania zwalidowanych zestawów narzędzi, takich jak pediatryczny inwentarz jakości życia PedsQL.1
Pęknięcia AVM u dzieci mogą powodować wyniszczające deficyty neurologiczne, takie jak dystonia, niedowład połowiczy, spastyczność, wodogłowie, padaczka i ataksja.1
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Materiały źródłowe
- #1 The epidemiology of brain arteriovenous malformations – PubMedhttps://pubmed.ncbi.nlm.nih.gov/10942012/
Objective: Common estimates of the prevalence rate for pial arteriovenous malformations (AVMs) of the brain vary widely, and their accuracy is questionable. Our objective was to critically review the original sources from which these rates were derived and to establish best estimates for both the incidence and prevalence of the disease. […] Many of the prevalence estimates (500-600/100,000 population) were based on autopsy data, a source that is inherently biased. Other estimates (140/100,000 population) originated from an inappropriate analysis of data from the Cooperative Study. The most reliable information comes from a population-based study of Olmsted County, MN, but prevalence data specific to AVMs was not found in that study. […] The estimates for AVM prevalence that are published in the medical literature are unfounded. Because of the rarity of the disease and the existence of asymptomatic patients, establishing a true prevalence rate is not feasible. Owing to variation in the detection rate of asymptomatic AVMs, the most reliable estimate for the occurrence of the disease is the detection rate for symptomatic lesions: 0.94 per 100,000 person-years (95% confidence interval, 0.57-1.30/100,000 person-years). This figure is derived from a single population-based study, but it is supported by a reanalysis of other data sources. The prevalence of detected, active (at risk) AVM disease is unknown, but it can be inferred from incidence data to be lower than 10.3 per 100,000 population.
- #1 Arteriovenous Malformations: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/1160167-overview
Arteriovenous malformations (AVMs) are congenital lesions composed of a complex tangle of arteries and veins connected by one or more fistulae. They most commonly occur in young adults, with morbidity and death occurring in 30-50% and 10-15% of patients, respectively. […] The detection rate in the general population based on prospective data from the New York Islands AVM Study is approximately 1.34 per 100,000 person-years. […] The prevalence of cerebral arteriovenous malformations (AVMs) in the United States is not known. Given the low threshold for MRI neuroimaging, many patients’ conditions are now discovered before they experience a brain hemorrhage. […] Reported detection rates range between 0.89 and 1.24 per 100,000 person-years according to reports from Australia, Sweden, and Scotland. The prevalence of cerebral AVMs in Scotland has been estimated to be 18 per 100,000 person-years.
- #1 The epidemiology of brain arteriovenous malformations in adultshttps://era.ed.ac.uk/handle/1842/793
The epidemiology of brain arteriovenous malformations in adults […] Arteriovenous malformations (AVMs) of the brain are part of the spectrum of intracranial vascular malformations (IVMs). They are the leading cause of intracerebral haemorrhage in young adults, they account for ~10% of non-traumatic subarachnoid haemorrhage, and they also cause epilepsy. […] I did not find a single prospective, truly population-based study, which is why I set up the Scottish Intracranial Vascular Malformation Study (SIVMS) with the multidisciplinary collaboration of the four clinical neuroscience centres in Scotland. […] During 1999-2000, 96 adults (of whom 92 were definite) were detected with a first-in-a-lifetime diagnosis of a brain AVM in Scotland. […] In SIVMS, the crude incidence of brain AVMs in Scotland in 1999 and 2000 was 1.1 (95%CI 0.9 to 1.4) per 100,000 adults per year. […] Having established brain AVM prevalence, incidence and the characteristics of presenting adults, the next stage for this work is to describe prognosis for this enlarging population-based cohort.
- #1 Epidemiology of ruptured brain arteriovenous malformation: a National Cohort Study in Korea in: Journal of Neurosurgery Volume 130 Issue 6 (2018) Journalshttps://thejns.org/view/journals/j-neurosurg/130/6/article-p1965.xml
Brain arteriovenous malformation (BAVM) is a rare cerebrovascular disease that causes intracranial hemorrhage. Although several reports have demonstrated the epidemiological features of BAVM in Western countries, no epidemiological investigations regarding BAVM have been reported in Korea. The authors aimed to investigate the national epidemiology of ruptured BAVM in a Korean population. […] A total of 8,802,696 person-years of observation were noted. During observation, 308 patients were diagnosed with a ruptured BAVM. The crude incidence of ruptured BAVM was 3.5 per 100,000 person-years. […] The standardized incidence of ruptured BAVMs was 3.6 per 100,000 person-years in Korea. Ruptured BAVMs resulted in high mortality and disability rates.
- #1 Epidemiology of Arteriovenous Malformations | East Bay Brain & Spinehttps://www.eastbaybrainandspine.com/conditions/vascular/arteriovenous-malformations/epidemiology-arteriovenous-malformations/
Arteriovenous malformations (AVMs) are relatively rare and occur in less than 1% of the general population. […] The incidence of detection is estimated to be around 1 in every 100,000 people per year. The overall prevalence is believed to be around 18 per 100,000 individuals. […] AVMs can be found at any age, but symptoms usually first appear between the ages of 10 and 40. […] Both sexes are equally affected by AVMs. However, hemorrhage due to AVM rupture is reportedly more common in females. […] The annual risk of hemorrhage in patients with untreated AVMs is estimated to be around 2-4% per year. […] The mortality rate after an AVM rupture is estimated to be around 10-15%, and morbidity (disability) rates are much higher. […] The epidemiology of AVMs underscores the importance of their early detection and appropriate management.
- #1 The epidemiology of brain arteriovenous malformations in adultshttps://era.ed.ac.uk/handle/1842/793?show=full
The epidemiology of brain arteriovenous malformations in adults. Arteriovenous malformations (AVMs) of the brain are part of the spectrum of intracranial vascular malformations (IVMs). They are the leading cause of intracerebral haemorrhage in young adults, they account for ~10% of non-traumatic subarachnoid haemorrhage, and they also cause epilepsy. […] I did not find a single prospective, truly population-based study, which is why I set up the Scottish Intracranial Vascular Malformation Study (SIVMS) with the multidisciplinary collaboration of the four clinical neuroscience centres in Scotland. […] During 1999-2000, 96 adults (of whom 92 were definite) were detected with a first-in-a-lifetime diagnosis of a brain AVM in Scotland. […] In a survey with multiple, overlapping sources of ascertainment confined to the Lothian healthboard region of Scotland, using capture-recapture analysis, I found the point prevalence of brain AVMs to be 18 (95%CI 16 to 24) per 100,000 adults. In SIVMS, the crude incidence of brain AVMs in Scotland in 1999 and 2000 was 1.1 (95%CI 0.9 to 1.4) per 100,000 adults per year. […] Having established brain AVM prevalence, incidence and the characteristics of presenting adults, the next stage for this work is to describe prognosis for this enlarging population-based cohort.
- #1 Arteriovenous malformation – Wikipediahttps://en.wikipedia.org/wiki/Arteriovenous_malformation
The estimated detection rate of AVM in the US general population is 1.4/100,000 per year. This is approximately one-fifth to one-seventh the incidence of intracranial aneurysms. An estimated 300,000 Americans have AVMs, of whom 12% (approximately 36,000) will exhibit symptoms of greatly varying severity. […] AVMs can occur in various parts of the body: brain (cerebral AV malformation), spleen, lung, kidney, spinal cord, liver, intercostal space, iris, spermatic cord, extremities arm, shoulder, etc. […] AVMs may occur in isolation or as a part of another disease (for example, Sturge-Weber syndrome or hereditary hemorrhagic telangiectasia).
- #1 Brain arteriovenous malformation | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/brain-arteriovenous-malformation?lang=us
Brain arteriovenous malformations are a type of intracranial high-flow vascular malformation composed of enlarged feeding arteries, a nidus of vessels closely associated with the brain parenchyma through which arteriovenous shunting occurs and draining veins. […] Although arteriovenous malformations are thought to represent a congenital abnormality, they are rarely found incidentally in the very young and many de novo lesions have been described amongst adults. They are thought to expand over time. Despite this, a third of arteriovenous malformations that are diagnosed due to hemorrhage are identified before the age of 20 years. Overall, they are diagnosed at a mean age of 31 years. […] Arteriovenous malformations are thought to occur in approximately 0.05% of the population. There is no gender predilection. […] AVMs tend to be solitary in the vast majority of cases (95%). When multiple, syndromic associations must be considered, including hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) and Wyburn-Mason syndrome (craniofacial arteriovenous metameric syndrome).
- #1 Arteriovenous Malformation of the Brain – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK430744/
The incidence in the United States is 1.34 per 100,000 person-years, although the actual prevalence rate is higher due to clinically silent disease, as only 12% of AVMs are estimated to become symptomatic. The mortality rate is 10-15% of patients who have a hemorrhage, and morbidity varies from approximately 30-50%. There is no sex predilection. Despite the considered congenital origin of AVMs, the clinical presentation most commonly occurs in young adults. […] The diagnosis and management of brain AVM are with an interprofessional team that consists of a neurosurgeon, neurologist, internist, and an invasive radiologist. The follow-up of these patients is usually by the nurse practitioner and primary care provider. The management of brain AVMs depends on the size, location, patient age, and status of the AVM (high risk of rupture). While surgery is the mainstay treatment, embolization is another option. The outcomes of these patients depend on the AVM size, presence of symptoms, location, patient comorbidity, and mental status. Complications following surgery are common and recovery in many patients is prolonged requiring extensive rehab. The most significant risk factor for death is the rupture of the AVM.
- #1 Arteriovenous Malformation (AVM): Causes & Symptomshttps://my.clevelandclinic.org/health/diseases/16755-arteriovenous-malformation-avm
Arteriovenous malformations are rare. Theyre present in about 1 in 100,000 people. […] Providers mainly discover them in people from ages 20 to 40. The risk of symptoms is highest between ages 30 and 50. […] If you have an arteriovenous malformation and dont get treatment, theres a 2-4% chance per year that itll bleed.
- #1 Cerebral Arteriovenous Malformation (AVM) | University of Michigan Healthhttps://www.uofmhealth.org/conditions-treatments/brain-neurological-conditions/cerebrovascular/cerebrovascular-disease-arteriovenous-malformation
The University of Michigans Cerebrovascular Program treats approximately 30 brain AVM patients each year. […] Brain AVMs occur in less than 1% of the population. They are more common in males than females. […] An increased prevalence has been seen in conditions such as hereditary hemorrhagic teleangiectasia. […] The studies also suggest that in the first year following a spontaneous AVM hemorrhage, the risk of bleeding again may be as high as 6% to 18%.
- #1 Prevalence and characteristics of brain arteriovenous malformations in hereditary hemorrhagic telangiectasia: a systematic review and meta-analysis in: Journal of Neurosurgery Volume 127 Issue 2 (2017) Journalshttps://thejns.org/view/journals/j-neurosurg/127/2/article-p302.xml
Patients with hereditary hemorrhagic telangiectasia (HHT) are known to suffer from high rates of cerebral arteriovenous malformations (AVMs). The authors performed a systematic review and meta-analysis of the literature examining prevalence rates, characteristics, and clinical presentation of cerebral AVMs in the HHT population. […] Thirty studies examined brain AVM prevalence rates in various HHT patient populations, and 18 studies examined AVM clinical and angiographic characteristics (9 studies examined both prevalence rates and AVM characteristics). The prevalence of brain AVMs in HHT patients was 10.4% (95% CI 7.9%13.0%) with no significant difference between males (8.5%, 95% CI 4.9%12.0%) and females (11.0%, 95% CI 5.9%16.1%). Patients with HHT Type 1 (HHT1) had a significantly higher brain AVM prevalence (13.4%, 95% CI 9.5%17.4%) compared with those with HHT Type 2 (HHT2) (2.4%, 95% CI 1.0%3.8%) (p 0.0001). […] The prevalence of brain AVMs in the HHT population is about 10%. HHT1 patients are significantly more likely to have brain AVMs than HHT2 patients. Most AVMs in the HHT population are symptomatic. The Spetzler-Martin grade for these lesions is 2 or less in nearly 90% of patients.
- #1 Vascular Malformations of the Brain and Spine in Children | PM&R KnowledgeNowhttps://now.aapmr.org/vascular-malformations-of-the-brain-and-spine-in-children/
In general, they occur sporadically with rare familial incidence, and a few reports mention an association with other abnormalities like hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease), Wyburn-Mason syndrome, von Hippel-Lindau disease, and Sturge-Weber syndrome. Hereditary hemorrhagic telangiectasia, an autosomal-dominant disease, is the most common genetic cause of cerebral AVM and carries a 10-25% lifetime risk of developing a cerebral AVM. […] AVM is the most unpredictable vascular malformation. It usually remains quiescent in childhood but tends to enlarge with time and cause local destruction. Cerebral AVMs may present with intracranial hemorrhage, seizures, headache, and focal neurologic deficits that may result in long-term disability. Neurologic symptoms are dependent on location within the brain or spinal cord, presenting either as a stroke or progressive myelopathy, respectively.
- #1 Arteriovenous Malformations: Epidemiology and Clinical Presentation | Neupsy Keyhttps://neupsykey.com/arteriovenous-malformations-epidemiology-and-clinical-presentation/
Various anatomic factors associated with hemorrhagic presentation have been identified. […] Independent risk factors for hemorrhagic presentation according to multivariate analyses include small size, deep venous drainage, deep, nonborder zone (watershed) and infratentorial locations, associated aneurysms, hypertension, small number of draining veins, venous ectasias, and high feeding artery pressure. […] Since hemorrhagic stroke is the most severe consequence of harboring an AVM, it is understandable that major efforts have been undertaken to identify risk factors predicting AVM rupture. […] The average annual AVM rupture rate is approximately 2% to 4% in most of these cohorts, but the rate varies highly depending on various risk factors.
- #1 Complications of Endovascular Treatments for Brain Arteriovenous Malformations: A Nationwide Surveillance | American Journal of Neuroradiologyhttp://www.ajnr.org/content/41/4/669
Complications arising after endovascular treatment of brain arteriovenous malformations are not negligible even though they may play a role in adjunctive therapy, especially in the management of infratentorial brain arteriovenous malformations. […] The annual hemorrhage rate for AVMs is between 2% and 4% per year. […] Despite the fairly large number of patients with bAVMs who are treated with embolization, published data on complications associated with embolization procedures are surprisingly scarce or only based on the experience of a single institution. […] Infratentorial AVMs are more frequently associated with feeding artery aneurysms than supratentorial AVMs (25% versus 5%). […] These findings support an aggressive management for infratentorial AVMs both before and after rupture. […] The present study shows that embolization of infratentorial AVMs could also involve procedure-related complications with a significantly higher rate. […] We observed that complications of endovascular treatment may result in worse outcomes in patients with bAVMs.
- #1 Arteriovenous Malformations: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/1160167-overview
Although 300,000 persons in the United States may harbor AVMs, only 12% of AVMs are estimated to become symptomatic. Death occurs in 10-15% of patients who have hemorrhage, and morbidity of various degrees occurs in approximately 30-50%. […] In population-based studies, 38-70% of brain AVMs present initially with hemorrhages. The overall risk of intracranial hemorrhage in patients with known AVM is 24% per year. […] Despite the presumed congenital origin of AVMs, the clinical presentation most commonly occurs in young adults. […] A history of subtle learning disorder is elicited in 66% of adults with AVMs. This suggests early effects that are largely subclinical and do not come to medical attention.
- #1 Cerebral arteriovenous malformation – Wikipediahttps://en.wikipedia.org/wiki/Cerebral_arteriovenous_malformation
The annual new detection rate incidence of AVMs is approximately 1 per 100,000 a year. The point prevalence in adults is approximately 18 per 100,000. AVMs are more common in males than females, although in females pregnancy may start or worsen symptoms due to the increase in blood flow and volume it usually brings. There is a significant preponderance (15-20%) of AVM in patients with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome). […] The overall annual incidence of haemorrhage from a ruptured AVM is 2-4%. Smaller AVMs have a greater propensity for haemorrhaging, whereas larger AVMs tend to more often cause seizures instead.
- #1 Brain AVM (arteriovenous malformation) – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/brain-avm/symptoms-causes/syc-20350260
Brain AVMs are seen in some people who have hereditary hemorrhagic telangiectasia (HHT). HHT also is known as Osler-Weber-Rendu syndrome. HHT affects the way blood vessels form in several areas of the body, including the brain. […] The risk of a brain AVM bleeding ranges from around 2% to 3% each year. The risk of bleeding may be higher for certain types of AVM. Risk also may be higher in people who have had a brain AVM bleed in the past.
- #1 Vascular Malformations of the Brain and Spine in Children | PM&R KnowledgeNowhttps://now.aapmr.org/vascular-malformations-of-the-brain-and-spine-in-children/
Acute hemorrhagic events in children have been associated with up to a 25% mortality rate. A hemorrhagic presentation is a significant independent predictor of future hemorrhage. The annual risk of rebleeding in children is 24% but may be up to 65% when projected over the child’s lifespan. […] AVMs are often an incidental finding versus a catastrophic presentation due to rupture/hemorrhage, making prediction of outcomes difficult. Once neurological deficit occurs, focus is on prevention of symptom worsening with secondary measures. Once rupture or hemorrhage occurs, there is an increased risk of recurrence, especially in the first year. […] The Spetzler-Martin (SM) grading scale is commonly used as a grading scale to predict the risk of surgical morbidity and mortality with brain AVMs. It is a composite score of nidus size, eloquence of adjacent brain and presence of deep venous drainage. Higher scores suggest increased surgical morbidity and mortality risk.
- #1 Arteriovenous Malformations: Epidemiology and Clinical Presentation | Neupsy Keyhttps://neupsykey.com/arteriovenous-malformations-epidemiology-and-clinical-presentation/
Arteriovenous malformations (AVMs) of the brain are relatively rare congenital developmental vascular lesions. […] The reported incidence rates of newly diagnosed AVMs have varied in different population-based studies from 0.89 to 1.34 cases per 100,000 personyears. […] The total prevalence of AVMs, including all the asymptomatic lesions, is nevertheless extremely difficult to estimate reliably due to the rarity of the disease. […] Overall annual mortality rates have varied from 0.7% to 2.9% in different study populations, but the mortality rates have usually not been compared with the background population. […] The authors study population of 623 AVM patients from Finland also included a subset of 155 untreated patients. […] AVMs cause only 2% of all and 4% of hemorrhagic strokes, the victims are often younger than most other stroke patients, and AVMs explain one-third of hemorrhagic strokes in young adults.
- #1 Current advances in epilepsy among patients with arteriovenous malformationshttps://www.explorationpub.com/Journals/en/Article/100643
Epileptic seizures are prevalent in people with brain vascular abnormalities like arteriovenous malformations (AVMs) and cavernous malformations, greatly affecting their quality of life. […] The association between intracranial vascular malformations and epilepsy remains a subject of ongoing discussion. Thus, the study of epilepsy in patients with AVMs represents a current and essential research topic. […] The initial sections cover fundamental concepts, definitions, natural history, and the epidemiology of epilepsy in AVM patients. […] Seizures represent the second most common clinical manifestation of AVMs, occurring in 11-35% of cases. […] The diagnostic rate for AVMs is estimated at 11.42 cases per 100,000 people. […] The estimated average annual bleeding risk for untreated AVMs is 2-4%.
- #1 Current advances in epilepsy among patients with arteriovenous malformationshttps://www.explorationpub.com/Journals/en/Article/100643
The estimated mortality rate in AVM patients is 0.7-2.9%. […] Among patients with AVMs, unprovoked epileptic seizures have been observed within 5 years after diagnosis in 26% of cases, 11% at 10 years, and 18% at 20 years of follow-up. […] Factors that increase the risk of seizures in patients with AVMs include male gender, younger age, frontal or temporal lobe AVMs, brain cortex-located AVMs, a superficial venous drainage, a superficial temporal lobe AVM nidus, fistulous AVMs, and AVMs with venous stenosis. […] The connection between cerebral vascular malformations and epilepsy is still a topic of discussion. […] In many cases, it is commonly observed that the specific area associated with the vascular malformation is closely linked to the occurrence of focal epileptogenic activity, and the removal of the malformation often results in better seizure control.
- #1 Brain AVM (arteriovenous malformation) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/brain-avm/diagnosis-treatment/drc-20350265
To diagnose a brain arteriovenous malformation (AVM), your healthcare professional reviews your symptoms and conducts a physical exam. […] One or more tests may be used to diagnose brain AVM. Imaging tests are usually done by radiologists trained in brain and nervous system imaging, known as neuroradiologists. […] Tests used to diagnose brain AVMs include: Cerebral angiography. This is the most detailed test to diagnose a brain AVM. Cerebral angiography reveals the location of the feeding arteries and draining veins, which is critical to planning treatment. […] MRI is more sensitive than CT and can show subtle changes in brain tissue related to a brain AVM. […] The proper treatment depends on your age, health, and the size and location of the brain AVM. […] Sometimes healthcare professionals decide to monitor a brain AVM rather than treating it. This may be recommended if you have few or no symptoms or if your AVM is in an area of your brain that’s hard to treat. Monitoring includes regular medical checkups with your healthcare team.
- #1 Brain Arteriovenous Malformations | Radiology Keyhttps://radiologykey.com/brain-arteriovenous-malformations-2/
Brain arteriovenous malformations (AVMs) are rare lesions, with an estimated detection rate of 1 to 1.3 per 100,000 person-years over several population-based studies. […] According to the literature, untreated AVMs carry a 2% to 4% annual risk of hemorrhage; however, in a single prospective randomized trial, treatment of unruptured AVMs was associated with an even higher rate of periprocedural complications. […] Management strategies for AVMs include conservative surveillance, radiosurgery, open surgical resection, endovascular embolization, or a combination of the aforementioned. The management pathway is determined best by multidisciplinary discussion after comprehensive imaging characterization. […] Long-term follow-up imaging from AVMs generally is appropriate, but the schedule depends on the type of management and patient characteristics.
- #1 Cerebral Arteriovenous Malformation AVMhttps://www.rch.org.au/kidsinfo/fact_sheets/Cerebral_Arteriovenous_Malformation_AVM/
A Cerebral Arteriovenous Malformation (AVM) is a collection of tightly tangled, abnormal looking, and enlarged blood vessels. AVMs are abnormal and complex connections between arteries and veins which interfere with the circulation of blood. […] AVMs in the brain occur in approximately 1 in 3,300 people and bleeding from an AVM most often occurs between 10 and 30 years of age. […] Your child will have regular surveillance imaging and follow-up appointments with their treating team.
- #1 Brain AVM (arteriovenous malformation) – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/brain-avm/diagnosis-treatment/drc-20350265
Researchers are studying ways to better predict the risk of hemorrhage in people with brain AVM. This can help better guide treatment decisions. […] Ongoing advances in embolization, radiosurgery and microsurgery techniques also are making it possible to use surgery to treat brain AVMs that were hard to access in the past.
- #1 Ruptured bilateral brain arteriovenous malformations in a young woman with early pregnancy: a case report | Journal of Medical Case Reports | Full Texthttps://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-023-03946-6
The risk of pregnancy to the rupture of brain AVMs remains controversial, with conflicting results. For instance, a study by Xi et al. indicated increased risk by 10.1% of AVMs bleeding during pregnancy, while other studies have previously indicated the contrary. […] The major goal of interventional treatment include the reduction of the risk of AVM-related hemorrhage, seizures, and other neurologic impairments. However, the efficacy of interventional methods in improving outcomes remains debatable. […] The main options for brain AVMs treatment are conservative medical management versus microsurgical excision or stereotactic radiosurgery; endovascular embolization is used as an adjunct intervention to surgery and stereotactic radiosurgery. […] AVMs are rare vascular malformations, of ill-elucidated congenital developmental origin. However, the increasing advancement in brain imaging is likely to improve the diagnosis and help to shed light on the actual burden of brain AVMs in Africa.
- #1 National reduction in cerebral arteriovenous malformation treatment correlated with increased rupture incidence | Journal of NeuroInterventional Surgeryhttps://jnis.bmj.com/content/15/8/735
A significant national decrease in unruptured AVM interventions was observed in the years following 2014. […] From 2003 to 2017, decreases in unruptured AVM interventions have been followed by a correlated lagged increase in the incidence of ruptured AVMs. […] These findings suggest that fewer unruptured AVM treatments may lead to increases in AVM rupture incidence. […] We observed that annual AVM rupture incidence was inversely correlated with annual AVM interventions, with rupture incidence increasing as interventions decreased. […] Our data suggest that this relationship may be profound, as we observed that rupture incidence surpassed interventions for the first time in 2017. […] The segmented average annual percent change for annual interventions was 0.63% before 2014 and 3.71% after 2014. […] Annual intervention and rupture rates had a strong inverse correlation (Pearson coefficient 0.82, p=0.0002; Spearman coefficient 0.77, p=0.0008). […] Further research must be done before we conclude that no unruptured AVMs should receive treatment.
- #1 A comprehensive analysis of patients with cerebral arteriovenous malformation with headache: assessment of risk factors and treatment effectiveness | The Journal of Headache and Pain | Full Texthttps://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-024-01774-7
This study identified potential risk factors for headaches in AVMs and found that microsurgery, stereotactic radiosurgery, and multimodal therapy had significant benefits in headache relief compared to conservative treatment. […] These findings provide important guidance for clinicians when developing treatment options that can help improve overall treatment outcomes and quality of life for patients.
- #1 Vascular Malformations of the Brain and Spine in Children | PM&R KnowledgeNowhttps://now.aapmr.org/vascular-malformations-of-the-brain-and-spine-in-children/
There have been few studies of long-term clinical outcomes of brain AVMs in pediatric patients, and previously published studies have used conventional metrics that have been validated in the adult population, such as the modified Rankin Scale. Although these metrics can serve as reasonable surrogates, an accurate understanding of overall health-related quality of life is contingent on utilizing validated toolsets, such as the pediatric quality of life inventory PedsQL. […] AVM ruptures in children can cause debilitating neurological deficits such as dystonia, hemiparesis, spasticity, hydrocephalus, epilepsy, and ataxia.
- #2 Arteriovenous Malformation of the Brain – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK430744/
The incidence in the United States is 1.34 per 100,000 person-years, although the actual prevalence rate is higher due to clinically silent disease, as only 12% of AVMs are estimated to become symptomatic. The mortality rate is 10-15% of patients who have a hemorrhage, and morbidity varies from approximately 30-50%. There is no sex predilection. Despite the considered congenital origin of AVMs, the clinical presentation most commonly occurs in young adults. […] The diagnosis and management of brain AVM are with an interprofessional team that consists of a neurosurgeon, neurologist, internist, and an invasive radiologist. The follow-up of these patients is usually by the nurse practitioner and primary care provider. The management of brain AVMs depends on the size, location, patient age, and status of the AVM (high risk of rupture). While surgery is the mainstay treatment, embolization is another option. The outcomes of these patients depend on the AVM size, presence of symptoms, location, patient comorbidity, and mental status. Complications following surgery are common and recovery in many patients is prolonged requiring extensive rehab. The most significant risk factor for death is the rupture of the AVM.
- #2 Arteriovenous Malformations: Epidemiology and Clinical Presentation | Neupsy Keyhttps://neupsykey.com/arteriovenous-malformations-epidemiology-and-clinical-presentation/
Arteriovenous malformations (AVMs) of the brain are relatively rare congenital developmental vascular lesions. […] The reported incidence rates of newly diagnosed AVMs have varied in different population-based studies from 0.89 to 1.34 cases per 100,000 personyears. […] The total prevalence of AVMs, including all the asymptomatic lesions, is nevertheless extremely difficult to estimate reliably due to the rarity of the disease. […] Overall annual mortality rates have varied from 0.7% to 2.9% in different study populations, but the mortality rates have usually not been compared with the background population. […] The authors study population of 623 AVM patients from Finland also included a subset of 155 untreated patients. […] AVMs cause only 2% of all and 4% of hemorrhagic strokes, the victims are often younger than most other stroke patients, and AVMs explain one-third of hemorrhagic strokes in young adults.
- #2 The epidemiology of brain arteriovenous malformations in adultshttps://era.ed.ac.uk/handle/1842/793?show=full
The epidemiology of brain arteriovenous malformations in adults. Arteriovenous malformations (AVMs) of the brain are part of the spectrum of intracranial vascular malformations (IVMs). They are the leading cause of intracerebral haemorrhage in young adults, they account for ~10% of non-traumatic subarachnoid haemorrhage, and they also cause epilepsy. […] I did not find a single prospective, truly population-based study, which is why I set up the Scottish Intracranial Vascular Malformation Study (SIVMS) with the multidisciplinary collaboration of the four clinical neuroscience centres in Scotland. […] During 1999-2000, 96 adults (of whom 92 were definite) were detected with a first-in-a-lifetime diagnosis of a brain AVM in Scotland. […] In a survey with multiple, overlapping sources of ascertainment confined to the Lothian healthboard region of Scotland, using capture-recapture analysis, I found the point prevalence of brain AVMs to be 18 (95%CI 16 to 24) per 100,000 adults. In SIVMS, the crude incidence of brain AVMs in Scotland in 1999 and 2000 was 1.1 (95%CI 0.9 to 1.4) per 100,000 adults per year. […] Having established brain AVM prevalence, incidence and the characteristics of presenting adults, the next stage for this work is to describe prognosis for this enlarging population-based cohort.
- #2https://journals.lww.com/neurosurgery/fulltext/2000/08000/the_epidemiology_of_brain_arteriovenous.23.aspx
Common estimates of the prevalence rate for pial arteriovenous malformations (AVMs) of the brain vary widely, and their accuracy is questionable. Our objective was to critically review the original sources from which these rates were derived and to establish best estimates for both the incidence and prevalence of the disease. […] Many of the prevalence estimates (500600/100,000 population) were based on autopsy data, a source that is inherently biased. Other estimates (140/100,000 population) originated from an inappropriate analysis of data from the Cooperative Study. The most reliable information comes from a population-based study of Olmsted County, MN, but prevalence data specific to AVMs was not found in that study. […] The estimates for AVM prevalence that are published in the medical literature are unfounded. Because of the rarity of the disease and the existence of asymptomatic patients, establishing a true prevalence rate is not feasible. Owing to variation in the detection rate of asymptomatic AVMs, the most reliable estimate for the occurrence of the disease is the detection rate for symptomatic lesions: 0.94 per 100,000 person-years (95% confidence interval, 0.571.30/100,000 person-years). This figure is derived from a single population-based study, but it is supported by a reanalysis of other data sources. The prevalence of detected, active (at risk) AVM disease is unknown, but it can be inferred from incidence data to be lower than 10.3 per 100,000 population.
- #2 Brain Arteriovenous Malformation (AVM) – Diagnosis and TreatmentSecond Opinion IconGroup 49Group 49https://www.barrowneuro.org/condition/arteriovenous-malformation-avm/
An AVM is very rare and affects less than one percent of the population, or about 10 per 100,000 people. […] An estimated 300,000 Americans are affected by arteriovenous malformations of the brain and spinal cord (neurological AVMs), but only about 12 percent of the affected population will have symptoms. […] Arteriovenous malformations are equally common among men and women of all races and ethnicities. They are believed to be congenital (existing at birth), but they can enlarge over time and cause symptoms at any age. […] Most arteriovenous malformations are detected through diagnostic imaging, such as a CT or MRI scan. Angiography, an imaging technique that involves the injection of a special dye, may be used to get a better look at the AVM. […] Because most people with AVMs experience few symptoms, they are often discovered during treatment for an unrelated disorder.
- #2 Brain arteriovenous malformation | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/brain-arteriovenous-malformation?embed_domain=hackmd.io%2525252525252525252525252F%25252525252525252525252540yIPUAFeCSL2JsU8smR5nJQ%2525252525252525252525252Fbnjhjgjghjghjgh&lang=gb
Brain arteriovenous malformations are a type of intracranial high-flow vascular malformation composed of enlarged feeding arteries, a nidus of vessels closely associated with the brain parenchyma through which arteriovenous shunting occurs and draining veins. […] Although arteriovenous malformations are thought to represent a congenital abnormality, they are rarely found incidentally in the very young and many de novo lesions have been described amongst adults. They are thought to expand over time. Despite this, a third of arteriovenous malformations that are diagnosed due to haemorrhage are identified before the age of 20 years. Overall, they are diagnosed at a mean age of 31 years. […] Arteriovenous malformations are thought to occur in approximately 0.05% of the population. There is no gender predilection. […] AVMs tend to be solitary in the vast majority of cases (95%). When multiple, syndromic associations must be considered, including hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) and Wyburn-Mason syndrome (craniofacial arteriovenous metameric syndrome).
- #2 Brain AVM (arteriovenous malformation) – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/brain-avm/symptoms-causes/syc-20350260
A brain arteriovenous malformation (AVM) is a tangle of blood vessels that creates irregular connections between arteries and veins in the brain. […] Overall, brain AVMs are rare. […] The cause of brain AVMs isn’t clear. Most people who have them are born with them, but they can form later in life. Rarely, AVM can be a trait passed down in families. […] Symptoms of a brain AVM may begin at any age but usually appear between ages 10 and 40. Brain AVMs can damage brain tissue over time. The effects slowly build and often cause symptoms in early adulthood. […] Brain AVMs account for about 2% of all hemorrhagic strokes each year. In children and young adults who experience brain hemorrhage, brain AVMs are often the cause. […] The cause of brain arteriovenous malformations (AVMs) is not known. Researchers believe most brain AVMs are present at birth and form during a baby’s growth in the womb. But brain AVMs can happen later in life as well.
- #2 Arteriovenous Malformations: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/1160167-overview
Although 300,000 persons in the United States may harbor AVMs, only 12% of AVMs are estimated to become symptomatic. Death occurs in 10-15% of patients who have hemorrhage, and morbidity of various degrees occurs in approximately 30-50%. […] In population-based studies, 38-70% of brain AVMs present initially with hemorrhages. The overall risk of intracranial hemorrhage in patients with known AVM is 24% per year. […] Despite the presumed congenital origin of AVMs, the clinical presentation most commonly occurs in young adults. […] A history of subtle learning disorder is elicited in 66% of adults with AVMs. This suggests early effects that are largely subclinical and do not come to medical attention.
- #2 Current advances in epilepsy among patients with arteriovenous malformationshttps://www.explorationpub.com/Journals/en/Article/100643
The estimated mortality rate in AVM patients is 0.7-2.9%. […] Among patients with AVMs, unprovoked epileptic seizures have been observed within 5 years after diagnosis in 26% of cases, 11% at 10 years, and 18% at 20 years of follow-up. […] Factors that increase the risk of seizures in patients with AVMs include male gender, younger age, frontal or temporal lobe AVMs, brain cortex-located AVMs, a superficial venous drainage, a superficial temporal lobe AVM nidus, fistulous AVMs, and AVMs with venous stenosis. […] The connection between cerebral vascular malformations and epilepsy is still a topic of discussion. […] In many cases, it is commonly observed that the specific area associated with the vascular malformation is closely linked to the occurrence of focal epileptogenic activity, and the removal of the malformation often results in better seizure control.
- #2https://link.springer.com/article/10.1007/s12028-019-00674-y
Brain arteriovenous malformations (bAVMs) are a known cause of intracranial hemorrhage. BAVMs are found either incidentally (2-10%) or due to symptomatic presentation. BAVM may present with headache (6-14%), symptomatic epilepsy (18-35%) or a focal neurological deficit (3-10%). However, hemorrhagic stroke is the most common presentation for a bAVM (45-72%). The incidence of DPH ranges from 2 to 21% depending on the study. The Spetzler-Martin grading (SMG) system is used to evaluate the risk of surgical treatment of bAVMs. Grading is based on the size of the nidus, the eloquence of adjacent brain and the venous drainage of the nidus, with higher grade indicating greater risk for complications, such as DPH. DPH was associated with high SMG, which supports its use in deciding postoperative treatment, complex venous drainage pattern, male gender and high intravenous fluid intake. Helsinki protocol for postoperative treatment of bAVMs seems to perform equally well when compared to a more complicated protocol. The benefits of simpler protocols are multifarious such as making the implementation of the protocol easier, decreasing the chance for treatment personnel error and reducing the risk for treatment related adverse event.
- #2 Ruptured bilateral brain arteriovenous malformations in a young woman with early pregnancy: a case report | Journal of Medical Case Reports | Full Texthttps://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-023-03946-6
The risk of pregnancy to the rupture of brain AVMs remains controversial, with conflicting results. For instance, a study by Xi et al. indicated increased risk by 10.1% of AVMs bleeding during pregnancy, while other studies have previously indicated the contrary. […] The major goal of interventional treatment include the reduction of the risk of AVM-related hemorrhage, seizures, and other neurologic impairments. However, the efficacy of interventional methods in improving outcomes remains debatable. […] The main options for brain AVMs treatment are conservative medical management versus microsurgical excision or stereotactic radiosurgery; endovascular embolization is used as an adjunct intervention to surgery and stereotactic radiosurgery. […] AVMs are rare vascular malformations, of ill-elucidated congenital developmental origin. However, the increasing advancement in brain imaging is likely to improve the diagnosis and help to shed light on the actual burden of brain AVMs in Africa.
- #3 Arteriovenous Malformation of the Brain – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK430744/
The incidence in the United States is 1.34 per 100,000 person-years, although the actual prevalence rate is higher due to clinically silent disease, as only 12% of AVMs are estimated to become symptomatic. The mortality rate is 10-15% of patients who have a hemorrhage, and morbidity varies from approximately 30-50%. There is no sex predilection. Despite the considered congenital origin of AVMs, the clinical presentation most commonly occurs in young adults. […] The diagnosis and management of brain AVM are with an interprofessional team that consists of a neurosurgeon, neurologist, internist, and an invasive radiologist. The follow-up of these patients is usually by the nurse practitioner and primary care provider. The management of brain AVMs depends on the size, location, patient age, and status of the AVM (high risk of rupture). While surgery is the mainstay treatment, embolization is another option. The outcomes of these patients depend on the AVM size, presence of symptoms, location, patient comorbidity, and mental status. Complications following surgery are common and recovery in many patients is prolonged requiring extensive rehab. The most significant risk factor for death is the rupture of the AVM.
- #3 Arteriovenous Malformations: Epidemiology and Clinical Presentation | Neupsy Keyhttps://neupsykey.com/arteriovenous-malformations-epidemiology-and-clinical-presentation/
Various anatomic factors associated with hemorrhagic presentation have been identified. […] Independent risk factors for hemorrhagic presentation according to multivariate analyses include small size, deep venous drainage, deep, nonborder zone (watershed) and infratentorial locations, associated aneurysms, hypertension, small number of draining veins, venous ectasias, and high feeding artery pressure. […] Since hemorrhagic stroke is the most severe consequence of harboring an AVM, it is understandable that major efforts have been undertaken to identify risk factors predicting AVM rupture. […] The average annual AVM rupture rate is approximately 2% to 4% in most of these cohorts, but the rate varies highly depending on various risk factors.
- #3 Arteriovenous Malformations: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/1160167-overview
Although 300,000 persons in the United States may harbor AVMs, only 12% of AVMs are estimated to become symptomatic. Death occurs in 10-15% of patients who have hemorrhage, and morbidity of various degrees occurs in approximately 30-50%. […] In population-based studies, 38-70% of brain AVMs present initially with hemorrhages. The overall risk of intracranial hemorrhage in patients with known AVM is 24% per year. […] Despite the presumed congenital origin of AVMs, the clinical presentation most commonly occurs in young adults. […] A history of subtle learning disorder is elicited in 66% of adults with AVMs. This suggests early effects that are largely subclinical and do not come to medical attention.
- #4 Complications of Endovascular Treatments for Brain Arteriovenous Malformations: A Nationwide Surveillance | American Journal of Neuroradiologyhttp://www.ajnr.org/content/41/4/669
Complications arising after endovascular treatment of brain arteriovenous malformations are not negligible even though they may play a role in adjunctive therapy, especially in the management of infratentorial brain arteriovenous malformations. […] The annual hemorrhage rate for AVMs is between 2% and 4% per year. […] Despite the fairly large number of patients with bAVMs who are treated with embolization, published data on complications associated with embolization procedures are surprisingly scarce or only based on the experience of a single institution. […] Infratentorial AVMs are more frequently associated with feeding artery aneurysms than supratentorial AVMs (25% versus 5%). […] These findings support an aggressive management for infratentorial AVMs both before and after rupture. […] The present study shows that embolization of infratentorial AVMs could also involve procedure-related complications with a significantly higher rate. […] We observed that complications of endovascular treatment may result in worse outcomes in patients with bAVMs.
- #5 Brain AVM (arteriovenous malformation) – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/brain-avm/symptoms-causes/syc-20350260
Brain AVMs are seen in some people who have hereditary hemorrhagic telangiectasia (HHT). HHT also is known as Osler-Weber-Rendu syndrome. HHT affects the way blood vessels form in several areas of the body, including the brain. […] The risk of a brain AVM bleeding ranges from around 2% to 3% each year. The risk of bleeding may be higher for certain types of AVM. Risk also may be higher in people who have had a brain AVM bleed in the past.