Malformacje żylnego układu naczyniowego wewnątrzczaszkowego
Epidemiologia

Malformacje żylnego układu naczyniowego wewnątrzczaszkowego (icVM) stanowią rzadkie, ale istotne wady naczyniowe mózgu, charakteryzujące się nieprawidłowo powiększonymi żyłami. Występują z częstością 2,1% w badaniu kohortowym 898 pacjentów, przy czym rozwojowe anomalie żylne (DVA), podtyp icVM, wykazują częstość 3-9% w badaniach MRI z kontrastem. Malformacje te często współistnieją z dużymi, złożonymi malformacjami żylnymi pozaczaszkowymi (78,9% przypadków) oraz sinus pericranii (47,3%). Epidemiologicznie icVM różnią się od malformacji tętniczo-żylnych (AVMs), które występują u około 0,05% populacji i cechują się wyższym ryzykiem krwotoku śródczaszkowego (2-4% rocznie). W icVM ryzyko powikłań, takich jak samoistna zakrzepica żyły zbiorczej czy krwotok, jest niskie, około 0,15% rocznie, a większość pacjentów pozostaje bezobjawowa.

Epidemiologia Malformacji żylnego układu naczyniowego wewnątrzczaszkowego

Malformacje żylnego układu naczyniowego wewnątrzczaszkowego (intracranial venous malformations, icVM) to stosunkowo rzadkie wady naczyniowe, charakteryzujące się nieprawidłowo powiększonymi żyłami w mózgu. Stanowią one istotną część spektrum wewnątrzczaszkowych malformacji naczyniowych (intracranial vascular malformations, IVMs), które łącznie występują u 0,1-4,0% populacji ogólnej.1

Częstotliwość występowania

Dokładne dane epidemiologiczne dotyczące izolowanych malformacji żylnych wewnątrzczaszkowych są ograniczone, jednak badania wskazują, że rozwojowe anomalie żylne (developmental venous anomalies, DVAs), będące podtypem icVM, są dość powszechnym znaleziskiem przypadkowym, z szacowaną częstością występowania wynoszącą 3-9% w badaniach MRI z kontrastem.2 W przeciwieństwie do innych rodzajów malformacji naczyniowych, takich jak malformacje tętniczo-żylne (arteriovenous malformations, AVMs), które mają częstość występowania około 0,05% populacji, malformacje żylne są znacznie częstsze.3

W badaniu przeprowadzonym na grupie 898 pacjentów, malformacje żylne wewnątrzczaszkowe wykryto u 19 osób (2,1%), z czego u 9 (47,3%) stwierdzono sinus pericranii, a u 15 (78,9%) współistniały duże, złożone malformacje żylne pozaczaszkowe.4 Prawdziwe anomalie rozwojowe żył (DVA) stwierdzono u 45% pacjentów w tej kohorcie, sinus pericranii również u 45%, natomiast przetrwały zatoki sierpu (persistent falcine sinuses) nie wykryto u żadnego z badanych pacjentów.5

Spektrum choroby

Malformacje żylne wewnątrzczaszkowe należą do szerszej kategorii wad naczyniowych, obejmującej cztery główne podtypy wad wrodzonych:6

  • Malformacje tętniczo-żylne (AVMs)
  • Malformacje jamiste (Cavernous malformations, CMs)
  • Rozwojowe anomalie żylne (Developmental venous anomalies, DVAs)
  • Teleangiektazje włośniczkowe (Capillary telangiectasias)

7

Wiele osób z malformacjami żylnymi wewnątrzczaszkowymi może nigdy nie mieć objawów i nigdy nie otrzymać diagnozy.8 Ze względu na niski próg stosowania badań neuroobrazowych MRI, coraz więcej przypadków jest wykrywanych przed wystąpieniem jakichkolwiek objawów klinicznych.9

Czynniki ryzyka i predyspozycje genetyczne

Chociaż dokładna przyczyna malformacji żylnych wewnątrzczaszkowych pozostaje nieznana, zidentyfikowano pewne czynniki ryzyka:10

Mutacje genetyczne leżące u podstaw rozwoju malformacji żylnych wewnątrzczaszkowych, czy to liniowe czy somatyczne, pozostają niewyjaśnione, ale mogą obejmować wspólne mechanizmy i szlaki z malformacjami żylnymi pozaczaszkowymi.13 W przypadku malformacji tętniczo-żylnych mózgu wykryto somatyczne aktywujące mutacje w genie KRAS jako przyczynę tych wad naczyniowych.14 Ponadto polimorfizm promotora w genie IL6 jest związany z podatnością na krwotok śródczaszkowy w malformacjach tętniczo-żylnych mózgu.15

Nadzór i monitorowanie

Wykrywanie i diagnostyka

Malformacje żylne wewnątrzczaszkowe często są wykrywane przypadkowo podczas badań neuroobrazowych przeprowadzanych z innych powodów, ponieważ większość pacjentów nie doświadcza objawów.16 Ze względu na rosnące wykorzystanie metod obrazowania i ich coraz większą czułość, przewiduje się, że częstość wykrywania tych malformacji będzie wzrastać.17

Chociaż rezonans magnetyczny (MRI) może zdiagnozować większość malformacji żylnych wewnątrzczaszkowych, cyfrowa angiografia subtrakcyjna (DSA) pozostaje najlepszą metodą obrazowania do badania hemodynamicznego zachowania tych malformacji.18

Ryzyko powikłań

W przeciwieństwie do innych malformacji naczyniowych, izolowane malformacje żylne wewnątrzczaszkowe mają bardzo niski wskaźnik powikłań, wynoszący około 0,15% rocznie. Powikłania mogą obejmować samoistną zakrzepicę żyły zbiorczej prowadzącą do zawału żylnego i krwotoku.19

Co ciekawe, w badaniu nad icVM nie zaobserwowano żadnych dowodów na krwotok wewnątrzczaszkowy ani nadciśnienie żylne w całej kohorcie.20 U żadnego pacjenta nie stwierdzono obrazowych dowodów na poszerzenie krążenia obocznego żylnego ani rozlanych skupisk rzekomozapalnych żył, które sugerowałyby wewnątrzczaszkowe nadciśnienie żylne.21 U żadnego pacjenta malformacje żylne wewnątrzczaszkowe nie uległy samoistnej zakrzepicy ani nie zniknęły.22

Bezobjawową zakrzepicę zatoki strzałkowej górnej obserwowano u trzech pacjentów z malformacjami żylnymi wewnątrzczaszkowymi.23 Pacjenci mogą rzadko doświadczać napadów padaczkowych lub krwawienia do mózgu (krwotok mózgowy), które zwykle są spowodowane innymi malformacjami naczyniowymi, które mogą współwystępować z malformacją żylną.24

Znaczenie kliniczne i monitorowanie

Większość pacjentów z malformacjami żylnymi wewnątrzczaszkowymi nie wykazuje objawów klinicznych, a rokowanie jest zazwyczaj korzystne.25 Ważne jest jednak regularne monitorowanie w przypadku wykrycia malformacji, aby zarządzać potencjalnymi powikłaniami.26

U pacjentów, którzy doświadczają objawów, takich jak bóle głowy, lekarz może przepisać leki. W rzadkich przypadkach krwawienia do mózgu niektóre krwotoki mogą wymagać interwencji chirurgicznej, ale wiele z nich można leczyć poprzez postępowanie zachowawcze i obserwację w szpitalu.27

W przypadku dzieci, jeśli występują niewyjaśnione bóle głowy, napady drgawkowe lub objawy neurologiczne, takie jak zawroty głowy, zmiany widzenia lub osłabienie, konieczna jest konsultacja z lekarzem. Objawy te mogą uzasadniać dalsze badania, szczególnie jeśli są trwałe lub nasilają się.28

Różnice w porównaniu z malformacjami tętniczo-żylnymi

Warto zauważyć, że malformacje żylne wewnątrzczaszkowe znacznie różnią się od malformacji tętniczo-żylnych (AVMs) pod względem epidemiologii i ryzyka klinicznego. Malformacje tętniczo-żylne mają wskaźnik wykrywalności w populacji ogólnej wynoszący około 1,34 na 100 000 osobolat29 i roczne ryzyko krwotoku śródczaszkowego wynoszące 2-4%.30

Szacuje się, że malformacje tętniczo-żylne mózgu występują u około 0,05% populacji i nie wykazują predylekcji płciowej.31 Stanowią one wiodącą przyczynę krwotoku śródmózgowego u młodych dorosłych, odpowiadają za ~10% nieurazowych krwotoków podpajęczynówkowych i również powodują padaczkę.32

Ryzyko krwotoku w nieleczonych, niepękniętych malformacjach tętniczo-żylnych wynosi 1-3% rocznie.33 Morfologiczne czynniki ryzyka związane ze zwiększonym ryzykiem krwotoku w malformacjach tętniczo-żylnych obejmują wzorzec drenażu żylnego, mniejszą liczbę żył drenujących, lokalizację i wielkość zmiany, obecność związanych tętniaków tętniczych lub żylaków żylnych.34

W przeciwieństwie do tego, malformacje żylne wewnątrzczaszkowe mają znacznie niższe ryzyko powikłań i generalnie nie wymagają leczenia.35

Podsumowanie danych epidemiologicznych

Podsumowując dane epidemiologiczne dotyczące malformacji żylnych wewnątrzczaszkowych:

  • Rozwojowe anomalie żylne (DVAs), będące podtypem icVM, występują z częstością 3-9% w badaniach MRI z kontrastem36
  • W badaniu na 898 pacjentach, malformacje żylne wewnątrzczaszkowe wykryto u 2,1% osób37
  • Malformacje żylne wewnątrzczaszkowe często współistnieją z dużymi, złożonymi malformacjami żylnymi pozaczaszkowymi (78,9% przypadków)38
  • Opóźnienie neurokognitywne stwierdzono u 15,7% pacjentów z malformacjami żylnymi wewnątrzczaszkowymi39
  • Ryzyko powikłań dla izolowanych malformacji żylnych jest niskie, wynoszące około 0,15% rocznie40
  • Bezobjawowa zakrzepica zatoki strzałkowej górnej obserwowana jest u niektórych pacjentów41

W przeciwieństwie do malformacji tętniczo-żylnych, które mają wyższe ryzyko krwotoku i częściej wymagają leczenia, malformacje żylne wewnątrzczaszkowe mają generalnie łagodny przebieg kliniczny i korzystne rokowanie. Właściwe nadzorowanie i monitorowanie pacjentów z tymi malformacjami jest jednak istotne dla wczesnego wykrywania potencjalnych powikłań.

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Vascular malformations of the central nervous system – UpToDate
    https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system
    Cerebral vascular malformations refer to a group of conditions characterized by abnormal vascular configurations occurring within the brain (and spinal cord). As a group, they occur in 0.1 to 4.0 percent of the general population […] Four general subtypes of congenital malformations include: Arteriovenous malformations (AVMs), Cavernous malformations (CMs), Developmental venous anomalies (DVAs), Capillary telangiectasias.
  • #2 Developmental venous anomaly | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/developmental-venous-anomaly?lang=us
    Developmental venous anomalies are very common as an incidental finding, with an estimated prevalence of 3-9% on contrast-enhanced MRI scans. […] Patients can present with intracranial hemorrhage (1-5%). An association has also been described with ischemic stroke and epilepsy. […] When isolated, developmental venous anomalies have a very low complication rate (0.15% per annum) mainly from spontaneous thrombosis of the collecting vein leading to venous infarction and hemorrhage.
  • #3 Brain arteriovenous malformation | Radiology Reference Article | Radiopaedia.org
    https://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).
  • #4
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #5
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #6 Vascular malformations of the central nervous system – UpToDate
    https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system
    Cerebral vascular malformations refer to a group of conditions characterized by abnormal vascular configurations occurring within the brain (and spinal cord). As a group, they occur in 0.1 to 4.0 percent of the general population […] Four general subtypes of congenital malformations include: Arteriovenous malformations (AVMs), Cavernous malformations (CMs), Developmental venous anomalies (DVAs), Capillary telangiectasias.
  • #7 Vascular malformations of the central nervous system – UpToDate
    https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system
    Cerebral vascular malformations refer to a group of conditions characterized by abnormal vascular configurations occurring within the brain (and spinal cord). As a group, they occur in 0.1 to 4.0 percent of the general population […] Four general subtypes of congenital malformations include: Arteriovenous malformations (AVMs), Cavernous malformations (CMs), Developmental venous anomalies (DVAs), Capillary telangiectasias.
  • #8 Intracranial venous malformations – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/intracranial-venous-malformations/symptoms-causes/syc-20353336
    Intracranial venous malformations are veins in the brain that are unusually large. […] Some people may have an intracranial venous malformation that is never diagnosed and never causes symptoms. […] Intracranial venous malformations usually don’t require treatment. […] Intracranial venous malformations may never cause symptoms. […] Experts don’t understand what causes intracranial venous malformations. […] Having a family history of intracranial venous malformations can increase your risk. […] Certain hereditary conditions can increase your risk of intracranial venous malformations.
  • #9 Arteriovenous Malformations: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1160167-overview
    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. […] 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%.
  • #10 Intracranial venous malformations – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/intracranial-venous-malformations/symptoms-causes/syc-20353336
    Intracranial venous malformations are veins in the brain that are unusually large. […] Some people may have an intracranial venous malformation that is never diagnosed and never causes symptoms. […] Intracranial venous malformations usually don’t require treatment. […] Intracranial venous malformations may never cause symptoms. […] Experts don’t understand what causes intracranial venous malformations. […] Having a family history of intracranial venous malformations can increase your risk. […] Certain hereditary conditions can increase your risk of intracranial venous malformations.
  • #11 Intracranial venous malformations | Beacon Health System
    https://www.beaconhealthsystem.org/library/diseases-and-conditions/intracranial-venous-malformations?content_id=CON-20313605
    Intracranial venous malformations are veins in the brain that are unusually large. These larger veins aren’t likely to cause symptoms or affect how the veins work. […] Some people may have an intracranial venous malformation that is never diagnosed and never causes symptoms. […] Intracranial venous malformations usually don’t require treatment. […] Because you may not have symptoms with intracranial venous malformations, your healthcare team might find that you have the condition during testing for other neurological problems. […] Having a family history of intracranial venous malformations can increase your risk. But most types aren’t inherited. […] Certain hereditary conditions can increase your risk of intracranial venous malformations. These include hereditary hemorrhagic telangiectasia, Sturge-Weber syndrome and Klippel-Trenaunay syndrome.
  • #12 Intracranial venous malformations | Beacon Health System
    https://www.beaconhealthsystem.org/library/diseases-and-conditions/intracranial-venous-malformations?content_id=CON-20313605
    Intracranial venous malformations are veins in the brain that are unusually large. These larger veins aren’t likely to cause symptoms or affect how the veins work. […] Some people may have an intracranial venous malformation that is never diagnosed and never causes symptoms. […] Intracranial venous malformations usually don’t require treatment. […] Because you may not have symptoms with intracranial venous malformations, your healthcare team might find that you have the condition during testing for other neurological problems. […] Having a family history of intracranial venous malformations can increase your risk. But most types aren’t inherited. […] Certain hereditary conditions can increase your risk of intracranial venous malformations. These include hereditary hemorrhagic telangiectasia, Sturge-Weber syndrome and Klippel-Trenaunay syndrome.
  • #13
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #14
    https://omim.org/entry/108010
    A number sign (#) is used with this entry because of the occurrence of cerebral arteriovenous malformations in several genetic disorders including hereditary hemorrhagic telangiectasia (HHT; 187300) and hereditary neurocutaneous angioma (106070). A promoter polymorphism in the IL6 gene (147620) is associated with susceptibility to intracranial hemorrhage in brain arteriovenous malformations. Somatic activating mutations in the KRAS gene (190070) have been identified as a cause of arteriovenous malformations of the brain. […] These vascular malformations occur in approximately 15 per 100,000 persons and are a leading cause of hemorrhagic stroke in young adults and children (summary by Nikolaev et al., 2018). […] Among 180 patients with brain arteriovenous malformations (BAVM), Pawlikowska et al. (2004) found an association between a promoter polymorphism in the IL6 gene (-174G/C; 147620.0001) and intracranial hemorrhage. Patients who were homozygous for the G allele had an increased risk intracranial bleed (odds ratio of 2.62) compared to carriers of the C allele.
  • #15
    https://omim.org/entry/108010
    A number sign (#) is used with this entry because of the occurrence of cerebral arteriovenous malformations in several genetic disorders including hereditary hemorrhagic telangiectasia (HHT; 187300) and hereditary neurocutaneous angioma (106070). A promoter polymorphism in the IL6 gene (147620) is associated with susceptibility to intracranial hemorrhage in brain arteriovenous malformations. Somatic activating mutations in the KRAS gene (190070) have been identified as a cause of arteriovenous malformations of the brain. […] These vascular malformations occur in approximately 15 per 100,000 persons and are a leading cause of hemorrhagic stroke in young adults and children (summary by Nikolaev et al., 2018). […] Among 180 patients with brain arteriovenous malformations (BAVM), Pawlikowska et al. (2004) found an association between a promoter polymorphism in the IL6 gene (-174G/C; 147620.0001) and intracranial hemorrhage. Patients who were homozygous for the G allele had an increased risk intracranial bleed (odds ratio of 2.62) compared to carriers of the C allele.
  • #16 Intracranial venous malformations – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/intracranial-venous-malformations/diagnosis-treatment/drc-20353337
    Because you may not have symptoms with intracranial venous malformations, your healthcare team might find that you have the condition during testing for other neurological problems. […] Intracranial venous malformations aren’t usually treated because they rarely cause symptoms. If you experience symptoms, such as headaches, your healthcare professional might prescribe medicines. […] Rarely, people who have intracranial venous malformations have seizures or bleeding in the brain, called a brain hemorrhage. These are usually caused by other vascular malformations that can be found with a venous malformation. Seizures are typically treated with medicines. […] Some hemorrhages require surgery, but many hemorrhages can be treated with medical management and observation in a hospital. […] If your healthcare professional thinks that you might have an intracranial venous malformation, you may be referred to a doctor who specializes in diseases that affect the brain and nervous system. This type of doctor is called a neurologist or neurosurgeon.
  • #17
    https://www.rgare.com/knowledge-center/article/cerebral-vascular-lesions
    According to recent studies, the estimated incidence and prevalence of cerebral AVMs is 1 per 100,000 and 18 per 100,000, respectively. […] When pricing for these lesions, it can be reasonably predicted that overall incidence figures are likely to increase over time due to both increasing utilization of radiographic studies and greater sensitivity of imaging techniques. […] Surprisingly, few risk factors for AVMs have been established, but they do exist. AVMs are slightly more common in males. […] While there is significant variation, AVMs also tend to be a disorder of younger adults. In a large retrospective study, the average age of symptomatic presentation was 36 years. […] Only 4% of cases were asymptomatic or incidental findings. […] Unfortunately, large-scale prospective population-based mortality data for cerebral AVMs is not currently available.
  • #18 Cerebral venous malformation with meningioma: A case report
    https://www.spandidos-publications.com/10.3892/etm.2016.3007
    Cerebral venous malformations (CVMs) are the most frequently encountered type of cerebral vascular malformation, with an incidence of 0.26 per year worldwide. […] The majority of CVM patients exhibit no clinical symptoms, and the disease prognosis is typically favorable. […] A previous study reported that the incidence of intracranial hemorrhage is increasing, primarily due to the coexistence of mixed venous-cavernous hemangiomas and arteriovenous malformations. […] Furthermore, previous autopsy reports indicate that CVMs account for 2.5-2.6% of cerebral vascular malformations, which is 3-4 times more common than arteriovenous malformations. […] Although MRI is able to diagnose the majority of CVMs, the DSA remains the best imaging module for investigating the hemodynamic behavior of CVMs. […] The majority of patients with CVMs have no clinical symptoms, and prognosis is typically favorable.
  • #19 Developmental venous anomaly | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/developmental-venous-anomaly?lang=us
    Developmental venous anomalies are very common as an incidental finding, with an estimated prevalence of 3-9% on contrast-enhanced MRI scans. […] Patients can present with intracranial hemorrhage (1-5%). An association has also been described with ischemic stroke and epilepsy. […] When isolated, developmental venous anomalies have a very low complication rate (0.15% per annum) mainly from spontaneous thrombosis of the collecting vein leading to venous infarction and hemorrhage.
  • #20
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #21
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Interestingly, none of the patients exhibited angiographic or other imaging evidence of intracranial venous hypertension or hemorrhage. […] No patient had imaging evidence of dilated venous collaterals or diffuse pseudophlebitic venous clustering to suggest intracranial venous hypertension. […] In no patient did the icVM spontaneously thrombose or disappear.
  • #22
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Interestingly, none of the patients exhibited angiographic or other imaging evidence of intracranial venous hypertension or hemorrhage. […] No patient had imaging evidence of dilated venous collaterals or diffuse pseudophlebitic venous clustering to suggest intracranial venous hypertension. […] In no patient did the icVM spontaneously thrombose or disappear.
  • #23
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #24 Intracranial venous malformations – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/intracranial-venous-malformations/diagnosis-treatment/drc-20353337
    Because you may not have symptoms with intracranial venous malformations, your healthcare team might find that you have the condition during testing for other neurological problems. […] Intracranial venous malformations aren’t usually treated because they rarely cause symptoms. If you experience symptoms, such as headaches, your healthcare professional might prescribe medicines. […] Rarely, people who have intracranial venous malformations have seizures or bleeding in the brain, called a brain hemorrhage. These are usually caused by other vascular malformations that can be found with a venous malformation. Seizures are typically treated with medicines. […] Some hemorrhages require surgery, but many hemorrhages can be treated with medical management and observation in a hospital. […] If your healthcare professional thinks that you might have an intracranial venous malformation, you may be referred to a doctor who specializes in diseases that affect the brain and nervous system. This type of doctor is called a neurologist or neurosurgeon.
  • #25 Cerebral venous malformation with meningioma: A case report
    https://www.spandidos-publications.com/10.3892/etm.2016.3007
    Cerebral venous malformations (CVMs) are the most frequently encountered type of cerebral vascular malformation, with an incidence of 0.26 per year worldwide. […] The majority of CVM patients exhibit no clinical symptoms, and the disease prognosis is typically favorable. […] A previous study reported that the incidence of intracranial hemorrhage is increasing, primarily due to the coexistence of mixed venous-cavernous hemangiomas and arteriovenous malformations. […] Furthermore, previous autopsy reports indicate that CVMs account for 2.5-2.6% of cerebral vascular malformations, which is 3-4 times more common than arteriovenous malformations. […] Although MRI is able to diagnose the majority of CVMs, the DSA remains the best imaging module for investigating the hemodynamic behavior of CVMs. […] The majority of patients with CVMs have no clinical symptoms, and prognosis is typically favorable.
  • #26 Intracranial Venous Malformations in Pediatric Conditions
    https://www.tenderpalm.com/intracranial-venous-malformations-in-pediatric-conditions-treatment-lucknow-india
    Intracranial venous malformations are relatively rare but are the most common type of vascular malformation found in the brain of some children who despite bearing most of its symptoms do not need any medical care or treatment. […] If a child experiences unexplained headaches, seizures, or neurological symptoms such as dizziness, vision changes, or weakness, it is essential to consult a healthcare provider. These symptoms may warrant further investigation, especially if they are persistent or worsening. […] There are currently no known ways to prevent intracranial venous malformations, as they are typically congenital and present from birth. Regular monitoring can help manage any potential complications if a malformation is detected.
  • #27 Intracranial venous malformations | Beacon Health System
    https://www.beaconhealthsystem.org/library/diseases-and-conditions/intracranial-venous-malformations?content_id=CON-20313605
    Intracranial venous malformations aren’t usually treated because they rarely cause symptoms. If you experience symptoms, such as headaches, your healthcare professional might prescribe medicines. […] Rarely, people who have intracranial venous malformations have seizures or bleeding in the brain, called a brain hemorrhage. […] Some hemorrhages require surgery, but many hemorrhages can be treated with medical management and observation in a hospital.
  • #28 Intracranial Venous Malformations in Pediatric Conditions
    https://www.tenderpalm.com/intracranial-venous-malformations-in-pediatric-conditions-treatment-lucknow-india
    Intracranial venous malformations are relatively rare but are the most common type of vascular malformation found in the brain of some children who despite bearing most of its symptoms do not need any medical care or treatment. […] If a child experiences unexplained headaches, seizures, or neurological symptoms such as dizziness, vision changes, or weakness, it is essential to consult a healthcare provider. These symptoms may warrant further investigation, especially if they are persistent or worsening. […] There are currently no known ways to prevent intracranial venous malformations, as they are typically congenital and present from birth. Regular monitoring can help manage any potential complications if a malformation is detected.
  • #29 Arteriovenous Malformations: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1160167-overview
    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. […] 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%.
  • #30 Current advances in epilepsy among patients with arteriovenous malformations
    https://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%.
  • #31 Brain arteriovenous malformation | Radiology Reference Article | Radiopaedia.org
    https://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).
  • #32 The epidemiology of brain arteriovenous malformations in adults
    https://era.ed.ac.uk/handle/1842/793?show=full
    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. […] 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.
  • #33 Arteriovenous malformations of the brain | MedLink Neurology
    https://www.medlink.com/articles/arteriovenous-malformations-of-the-brain
    Cerebral arteriovenous malformations occur rarely, in about 0.1% of the population, but account for at least 1% to 2% of strokes and up to 9% of all nontraumatic subarachnoid hemorrhages. […] The overall incidence of brain arteriovenous malformations is about 1.10 to 1.42 cases per 100,000. […] Brain arteriovenous malformations account for 1% to 2% of all strokes, 4% of strokes in young adults, 9% of subarachnoid hemorrhages, and 4% of intracerebral hemorrhages. […] The estimated risk of hemorrhage in an untreated, unruptured arteriovenous malformation is 1% to 3% per year. […] Morphologic risk factors associated with an increased risk of hemorrhage in arteriovenous malformations include venous drainage pattern, fewer draining veins, nidus location and size, presence of associated arterial aneurysms, or venous varices. […] About 10% to 25% of patients with hereditary hemorrhagic telangiectasia will develop at least one cerebral arteriovenous malformation.
  • #34 Arteriovenous malformations of the brain | MedLink Neurology
    https://www.medlink.com/articles/arteriovenous-malformations-of-the-brain
    Cerebral arteriovenous malformations occur rarely, in about 0.1% of the population, but account for at least 1% to 2% of strokes and up to 9% of all nontraumatic subarachnoid hemorrhages. […] The overall incidence of brain arteriovenous malformations is about 1.10 to 1.42 cases per 100,000. […] Brain arteriovenous malformations account for 1% to 2% of all strokes, 4% of strokes in young adults, 9% of subarachnoid hemorrhages, and 4% of intracerebral hemorrhages. […] The estimated risk of hemorrhage in an untreated, unruptured arteriovenous malformation is 1% to 3% per year. […] Morphologic risk factors associated with an increased risk of hemorrhage in arteriovenous malformations include venous drainage pattern, fewer draining veins, nidus location and size, presence of associated arterial aneurysms, or venous varices. […] About 10% to 25% of patients with hereditary hemorrhagic telangiectasia will develop at least one cerebral arteriovenous malformation.
  • #35 Intracranial venous malformations – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/intracranial-venous-malformations/symptoms-causes/syc-20353336
    Intracranial venous malformations are veins in the brain that are unusually large. […] Some people may have an intracranial venous malformation that is never diagnosed and never causes symptoms. […] Intracranial venous malformations usually don’t require treatment. […] Intracranial venous malformations may never cause symptoms. […] Experts don’t understand what causes intracranial venous malformations. […] Having a family history of intracranial venous malformations can increase your risk. […] Certain hereditary conditions can increase your risk of intracranial venous malformations.
  • #36 Developmental venous anomaly | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/developmental-venous-anomaly?lang=us
    Developmental venous anomalies are very common as an incidental finding, with an estimated prevalence of 3-9% on contrast-enhanced MRI scans. […] Patients can present with intracranial hemorrhage (1-5%). An association has also been described with ischemic stroke and epilepsy. […] When isolated, developmental venous anomalies have a very low complication rate (0.15% per annum) mainly from spontaneous thrombosis of the collecting vein leading to venous infarction and hemorrhage.
  • #37
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #38
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #39
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.
  • #40 Developmental venous anomaly | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/developmental-venous-anomaly?lang=us
    Developmental venous anomalies are very common as an incidental finding, with an estimated prevalence of 3-9% on contrast-enhanced MRI scans. […] Patients can present with intracranial hemorrhage (1-5%). An association has also been described with ischemic stroke and epilepsy. […] When isolated, developmental venous anomalies have a very low complication rate (0.15% per annum) mainly from spontaneous thrombosis of the collecting vein leading to venous infarction and hemorrhage.
  • #41
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7903553/
    Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. […] There was no intracranial hemorrhage or venous hypertension seen in the cohort. […] Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. […] icVM were not associated with venous hypertension. […] The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. […] Within the icVM cohort, true DVA, sinus pericranii, and persistent falcine sinuses were found in 45%, 45% and 0% of the cohort, respectively.