Malformacja tętniczo-żylna rdzenia kręgowego
Objawy
Malformacja tętniczo-żylna rdzenia kręgowego (spinal AVM) to patologiczne bezpośrednie połączenia tętniczo-żylne w obrębie lub w pobliżu rdzenia kręgowego, pomijające naczynia włosowate, co prowadzi do zaburzeń hemodynamicznych, takich jak zastój żylny, nadciśnienie żylne i efekt „podkradania” krwi z segmentów rdzenia. Objawy kliniczne są zróżnicowane i zależą od lokalizacji oraz typu AVM, obejmując zaburzenia motoryczne (osłabienie mięśni, paraliż), czuciowe (parestezje, ból), zaburzenia zwieraczy oraz ból pleców i korzeniowy. Występują dwie główne formy prezentacji: stopniowa progresja objawów (80-85% przypadków) oraz ostra manifestacja związana z krwawieniem (10-20%). Ryzyko krwawienia szacuje się na 2-4% rocznie, a u dzieci może sięgać 24% rocznie, z poważnymi konsekwencjami neurologicznymi, w tym paraliżem i śmiercią. Diagnostyka opiera się na obrazowaniu MRI, gdzie widoczny jest obrzęk rdzenia jako hiperintensywny sygnał w sekwencji T2, oraz angiografii. Typy AVM (I-IV) różnią się lokalizacją i obrazem klinicznym, co wpływa na rokowanie i strategię leczenia.
- Objawy malformacji tętniczo-żylnej rdzenia kręgowego
- Wczesne objawy malformacji tętniczo-żylnej rdzenia kręgowego
- Zaawansowane objawy i progresja choroby
- Patomechanizmy powstawania objawów
- Czynniki ryzyka i nasilenie objawów
- Następstwa nieleczonej malformacji tętniczo-żylnej rdzenia kręgowego
- Znaczenie wczesnego rozpoznania i leczenia
- Podsumowanie objawów malformacji tętniczo-żylnej rdzenia kręgowego
Objawy malformacji tętniczo-żylnej rdzenia kręgowego
Malformacja tętniczo-żylna rdzenia kręgowego (ang. spinal arteriovenous malformation, spinal AVM) to nieprawidłowe splecenie naczyń krwionośnych, tworzące bezpośrednie połączenia między tętnicami a żyłami z pominięciem naczyń włosowatych w obrębie lub w pobliżu rdzenia kręgowego. Ta rzadka patologia może prowadzić do poważnych konsekwencji neurologicznych i uszkodzenia rdzenia kręgowego, jeśli nie zostanie leczona.12
Występowanie i pojawianie się objawów
Objawy malformacji tętniczo-żylnej rdzenia kręgowego mogą być bardzo zróżnicowane i zależą od lokalizacji oraz stopnia nasilenia zmiany. U wielu pacjentów AVM rdzenia może przez długi czas nie dawać żadnych objawów.3 Szacuje się, że nawet do 15% osób z AVM może nie doświadczać żadnych symptomów.4
Objawy najczęściej pojawiają się u osób w wieku 20-40 lat, choć mogą wystąpić w każdym wieku. Około 20% diagnozowanych pacjentów to osoby poniżej 16 roku życia.56 Większość malformacji tętniczo-żylnych rdzenia kręgowego jest wrodzona, ale mogą również rozwinąć się w wieku dorosłym (nabyte).7
Sposoby manifestacji klinicznej
Objawy AVM rdzenia kręgowego mogą pojawić się na dwa główne sposoby:89
- Stopniowe narastanie objawów neurologicznych (powolna progresja) – dotyczy około 80-85% przypadków
- Nagły początek objawów (ostra manifestacja) – występuje w około 10-20% przypadków, zwykle z powodu krwawienia
Czas trwania objawów przed postawieniem diagnozy często wynosi od 1 do 3 lat (40-63% pacjentów), jednak u 10-34% osób choroba może trwać dłużej niż 3 lata bez rozpoznania.11 Obserwuje się również stopniowo postępujący przebieg z pogorszeniami skokowymi u około 11-32% pacjentów.11
Wczesne objawy malformacji tętniczo-żylnej rdzenia kręgowego
Wczesne objawy AVM rdzenia kręgowego mogą być subtelne i niespecyficzne, co często prowadzi do opóźnienia w diagnozie. Najczęstsze początkowe objawy to:112
- Problemy z chodzeniem lub wchodzeniem po schodach
- Drętwienie, mrowienie lub nagły ból w kończynach dolnych
- Osłabienie mięśniowe po jednej lub obu stronach ciała
- Zaburzenia równowagi lub koordynacji (ataksja)
- Sztywność w nogach utrudniająca chodzenie
Początkowe objawy są często niespecyficzne i mogą obejmować symetryczne lub asymetryczne zaburzenia czuciowe, takie jak parestezje w jednej lub obu stopach oraz rozlane lub ogniskowe zaburzenia czucia (17-72%), trudności w chodzeniu i zaburzenia motoryczne (50-81%), ból, w tym ból pleców lub ból korzeniowy (13-64%) oraz zaburzenia oddawania moczu (4-75%).11
Ból jako objaw wczesny
Ból jest częstym wczesnym symptomem malformacji tętniczo-żylnej rdzenia kręgowego. Może się on manifestować jako:1310
- Ból pleców, często zlokalizowany w okolicy piersiowej
- Ból korzeniowy promieniujący wzdłuż rozkładu nerwów
- Nagły, silny ból pleców (szczególnie w przypadku krwawienia)
Zaawansowane objawy i progresja choroby
W miarę postępu choroby, objawy mogą się nasilać i pojawiać się mogą dodatkowe symptomy:1
- Nagły, silny ból pleców (często związany z krwawieniem)
- Utrata czucia w nogach
- Problemy z oddawaniem moczu lub wypróżnianiem
- Ból głowy
- Sztywność karku
- Nadwrażliwość na światło
Zaawansowana malformacja tętniczo-żylna rdzenia kręgowego może prowadzić do poważnych deficytów neurologicznych, w tym:3
- Osłabienia mięśni lub paraliżu
- Zaburzeń funkcji zwieraczy (pęcherza moczowego i odbytu)
- Zaburzeń funkcji seksualnych (w tym zaburzeń erekcji u mężczyzn)
- Napadów drgawkowych
Objawy związane z krwawieniem
Krwawienie z AVM rdzenia kręgowego jest najpoważniejszym powikłaniem i może objawiać się:1613
- Nagłym, intensywnym bólem pleców („coup de poignard” – ból jak pchnięcie sztyletem)
- Nagłym osłabieniem motorycznym kończyn
- Nagłą utratą czucia lub parestezjami
- Ostrym zatrzymaniem moczu
- Nagłą niewydolnością zwieraczy
- Porażeniem lub niedowładem
W przypadku wysokiej lokalizacji zmian, krwawienie z AVM może rozszerzyć się do przestrzeni podpajęczynówkowej powodując objawy krwotoku podpajęczynówkowego, w tym silny ból głowy, sztywność karku i zaburzenia świadomości.14
Patomechanizmy powstawania objawów
Malformacja tętniczo-żylna rdzenia kręgowego może powodować objawy neurologiczne na kilka sposobów:818
Zaburzenia hemodynamiczne
AVM rdzenia kręgowego zakłóca prawidłowy przepływ krwi powodując:19
- Zastój żylny i nadciśnienie żylne – krew przepływa bezpośrednio z tętnic do żył, powodując wysokie ciśnienie w układzie żylnym
- Pogorszenie perfuzji rdzenia kręgowego – prowadzi do niedotlenienia tkanek
- Efekt „podkradania” – odpływ krwi z przylegających segmentów rdzenia kręgowego
Zastój żylny może powodować obrzęk rdzenia kręgowego, który jest widoczny jako hiperintensywny sygnał w obrazowaniu MRI w sekwencji T2.21
Efekt masy i ucisk na rdzeń kręgowy
Duże AVM mogą wywierać ucisk na:18
- Rdzeń kręgowy – powodując mielopatię
- Korzenie nerwowe – powodując radikulopatię
Krwawienie i jego konsekwencje
Krwawienie z AVM rdzenia kręgowego może prowadzić do:16
- Krwawienia do tkanki rdzenia kręgowego
- Krwawienia do przestrzeni podpajęczynówkowej
- Krwiaków zewnątrzoponowych
Krwawienie powoduje uszkodzenie rdzenia kręgowego poprzez bezpośrednie zniszczenie tkanki nerwowej oraz poprzez wtórne mechanizmy, takie jak ucisk i reakcja zapalna.16
Czynniki ryzyka i nasilenie objawów
Czynniki wpływające na ryzyko krwawienia
Ryzyko krwawienia z AVM rdzenia kręgowego szacuje się na 2-4% rocznie.1625 Czynniki zwiększające to ryzyko to:26
- Wcześniejsze krwawienie (szczególnie w ciągu ostatnich 5 lat)
- Głęboka lokalizacja zmiany
- Wyłączny głęboki drenaż żylny
- Współistnienie tętniaka
- Rozlana morfologia zmiany
- Lokalizacja w odcinku szyjnym lub górnym piersiowym
Ryzyko ponownego krwawienia u dzieci wynosi 24% rocznie, ale może sięgać nawet 65% w perspektywie całego życia dziecka.26
Typy AVM rdzenia kręgowego a obraz kliniczny
Obraz kliniczny zależy od typu malformacji tętniczo-żylnej:27
- Typ I (przetoki tętniczo-żylne opony twardej) – typowa prezentacja to radikulomielopatia z postępującym pogorszeniem neurologicznym
- Typ II (śródrdzeniowe malformacje typu glomus) – postępująca i fluktuująca mielopatia, paraplegia i ból, z okresami ostrego pogorszenia neurologicznego z powodu krwawienia w obrębie AVM
- Typ III (malformacje młodzieńcze) – obraz kliniczny podobny do typu II
- Typ IV (przetoki okołordzeniowe) – objawy pojawiają się między trzecią a szóstą dekadą życia
Następstwa nieleczonej malformacji tętniczo-żylnej rdzenia kręgowego
Bez leczenia, malformacja tętniczo-żylna rdzenia kręgowego może prowadzić do postępującej niepełnosprawności z powodu uszkodzenia rdzenia kręgowego i otaczających tkanek.15 Mogą wystąpić następujące powikłania:29
- Problemy z poruszaniem się (deficyty motoryczne)
- Ból, mrowienie i drętwienie (deficyty czuciowe)
- Deformacja kręgosłupa
- Tętniak naczyń krwionośnych
- Nadciśnienie żylne, które może powodować gromadzenie się płynu (obrzęk) i śmierć tkanek z powodu braku tlenu (zawał rdzenia kręgowego)
- Krwotok, który może przyspieszyć uszkodzenie rdzenia kręgowego
Ostre krwawienie z AVM rdzenia kręgowego wiąże się ze znaczącą śmiertelnością i ryzykiem trwałej niepełnosprawności.31
W ciągu 3 lat od wystąpienia objawów, około 50% nieleczonych pacjentów będzie przykutych do łóżka lub wózka inwalidzkiego, a około 15% umrze z powodu powikłań przewlekłej paraplegii.32
Znaczenie wczesnego rozpoznania i leczenia
Wczesne rozpoznanie i leczenie malformacji tętniczo-żylnej rdzenia kręgowego jest kluczowe dla poprawy rokowania.7 Czas między wystąpieniem objawów a interwencją chirurgiczną jest krytyczny.33 Korzystne wyniki można osiągnąć, gdy interwencja chirurgiczna jest wykonywana w ciągu 36 lub 48 godzin po wystąpieniu objawów, odpowiednio u pacjentów z całkowitymi i niecałkowitymi deficytami.33
Leczenie ma na celu:34
- Zmniejszenie ryzyka krwawienia
- Zatrzymanie lub zapobieganie progresji niepełnosprawności
- Złagodzenie objawów
Wyniki leczenia są lepsze u pacjentów z krótszym czasem trwania objawów. Zmiany pozostawione bez leczenia przez ponad 18 miesięcy wykazują mniejszą odpowiedź na leczenie.15 Chód poprawia się częściej niż funkcje pęcherza moczowego i jelit.15
Obserwacje dotyczące poprawy po leczeniu
Po zastosowaniu odpowiedniego leczenia, u pacjentów może wystąpić stopniowa poprawa funkcji neurologicznych. Przykładowo, w przypadku dzieci udokumentowano stopniową poprawę w ciągu roku po embolizacji wewnątrznaczyniowej, włącznie z odzyskaniem zdolności chodzenia z pomocą i pełnej kontroli pęcherza moczowego.21
U pacjentów leczonych z powodu malformacji tętniczo-żylnej rdzenia kręgowego, nawet po skutecznym leczeniu, mogą utrzymywać się epizody sztywności karku i skurczów mięśniowych.36
Podsumowanie objawów malformacji tętniczo-żylnej rdzenia kręgowego
Objawy malformacji tętniczo-żylnej rdzenia kręgowego mogą być bardzo zróżnicowane i obejmują:132
- Zaburzenia motoryczne: osłabienie mięśni, paraliż, problemy z chodzeniem, trudności z koordynacją i równowagą
- Zaburzenia czuciowe: drętwienie, mrowienie, nagły ból w kończynach, utrata czucia
- Ból: ból pleców (nagły i silny w przypadku krwawienia), ból korzeniowy
- Zaburzenia zwieraczy: problemy z oddawaniem moczu i stolca, inkontynencja
- Zaburzenia funkcji seksualnych: zaburzenia erekcji u mężczyzn
- Objawy ogólne: ból głowy, sztywność karku, nadwrażliwość na światło (w przypadku krwawienia)
Należy pamiętać, że malformacja tętniczo-żylna rdzenia kręgowego wymaga specjalistycznej diagnostyki i leczenia. Pacjenci z objawami sugerującymi AVM rdzenia kręgowego powinni zostać skierowani do ośrodków o dużym doświadczeniu w leczeniu tych rzadkich patologii naczyniowych.1538
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Materiały źródłowe
- #1 Spinal arteriovenous malformation (AVM) – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/spinal-arteriovenous-malformation/symptoms-causes/syc-20355613
A spinal arteriovenous malformation (AVM) is a tangle of blood vessels that forms on, in or near the spinal cord. This creates irregular connections between arteries and veins. Without treatment, this rare condition can cause lasting damage to the spinal cord. […] Symptoms of a spinal arteriovenous malformation (AVM) can differ from person to person. Symptoms depend on where the AVM is found and how serious it is. Some people may not notice symptoms for many years, if at all. Others may experience symptoms that are life-threatening. […] Symptoms may start suddenly or slowly and may include: Trouble walking or climbing stairs. Numbness, tingling or sudden pain in the legs. Weakness on one or both sides of the body. […] As the condition gets worse, you may have more symptoms including: Sudden, serious back pain. Lack of feeling in the legs. Trouble urinating or having bowel movements. Headache. Stiff neck. […] Without treatment, a spinal arteriovenous malformation (AVM) can cause disability that gets worse over time. This is from damage to the spinal cord and surrounding tissues.
- #2 Spinal AVM: What It Is, Symptoms, Treatment & Prognosishttps://my.clevelandclinic.org/health/diseases/spinal-arteriovenous-malformation-avm
A spinal arteriovenous malformation (AVM) is a tangle of blood vessels near your spinal cord. Blood bypasses your capillaries on its way from an artery to a vein. This causes symptoms like back pain and muscle weakness. Severe complications can happen if the blood vessel malformation breaks open. […] Symptoms of a spinal AVM happen because a tangle of blood vessels presses on your nerve roots and spinal cord. This type of compression is known as myelopathy. Common symptoms include: Back pain. Muscle weakness or stiffness (neck, arms and legs). Tingling, numbness or loss of feeling in your legs. Headache. Difficulty with balance and coordination. Trouble controlling your bladder and bowels. […] When symptoms do start, you should contact a healthcare provider right away. Sudden, severe symptoms like back pain may be a sign that the AVM ruptured (burst). This is a medical emergency and needs treatment immediately.
- #3 Spinal Arteriovenous Malformations | UMass Memorial Healthhttps://www.harringtonhospital.org/health-library/spinal-arteriovenous-malformations
Spinal AVMs often dont cause any symptoms. When they do, they’re usually minor and hard to notice. In a few people, though, the symptoms can be bad enough to affect their ability to function. […] These are the most common symptoms of a spinal AVM: Muscles that feel weak or become paralyzed, Stiffness in the legs, causing trouble walking, Abnormal control of bowel and bladder, Problems with balance and coordination (ataxia), Pain or unusual feelings throughout your body, such as tingling or numbness. […] Even though a spinal AVM may not always cause symptoms, it can still be dangerous, particularly if it starts to cause symptoms. Your healthcare provider should check any suspicious symptoms you have. These may include: Muscles that feel weak, Muscle paralysis, Difficulty with balance and coordination (ataxia), Unusual sensations, such as numbness or tingling, or pain. […] Pay attention to the following symptoms of spinal AVMs and seek care for: Muscles that suddenly feel weak or become paralyzed, Any problems you may be having with balance and coordination or bowel or bladder control, Pain or unusual sensations, such as numbness or tingling in your body.
- #4 Arteriovenous Malformation (AVM): Causes & Symptomshttps://my.clevelandclinic.org/health/diseases/16755-arteriovenous-malformation-avm
Arteriovenous malformation symptoms may include: Seizures with or without loss of consciousness. Headache. Muscle weakness or complete paralysis. Nausea and vomiting. Numbness or tingling sensation. Dizziness. Problems with movement, speech, memory, thinking, balance or vision. Mental confusion, hallucinations or dementia. Back pain (can be sudden and severe) or weakness in your lower body (hips) and legs to your toes. Shortness of breath during exertion. Coughing up blood (if the AVM is in your lungs). Abdominal pain. Lumps on arms or legs or your trunk. Pain and swelling. […] You may or may not have symptoms if you have an arteriovenous malformation. Up to 15% of people with AVMs dont have symptoms. Often, the first sign you have an AVM is after it bleeds. If you have a brain AVM and it leads to a blood vessel bleed (rupture), it can cause a stroke and brain damage. About 50% of people with a brain AVM have a brain bleed (hemorrhage) as their initial symptom.
- #5 Spinal arteriovenous malformation (AVM) – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutubehttps://www.augustahealth.com/disease/spinal-arteriovenous-malformation-avm/
Spinal AVM can go undiagnosed unless you begin experiencing signs and symptoms. […] Symptoms of spinal AVM vary greatly from person to person depending on the severity and location of the AVM. Some people may not develop noticeable symptoms for many years, if at all. Others may experience symptoms that are debilitating or life-threatening. […] Symptoms usually develop when people are in their 20s, although almost 20% of people diagnosed with spinal AVM are under the age of 16. […] The emergence of symptoms may be sudden or gradual. Symptoms typically include: Problems with walking or climbing stairs, Numbness, tingling or sudden pain in your legs, Weakness on one or both sides of your body. […] As the condition progresses, additional symptoms may include: Sudden, severe back pain, Lack of feeling in the legs, Difficulty urinating or moving your bowels, Headache, Stiff neck, Sensitivity to light. […] The goal of spinal AVM treatment is to reduce the risk of hemorrhage and stop or prevent the progression of disability and other symptoms.
- #6 Spinal Vascular Malformations Symptoms & Treatments | Barrow Neurological InstituteSecond Opinion Iconhttps://www.barrowneuro.org/condition/vascular-malformations-of-the-spinal-cord/
Vascular malformations of the spinal cord are abnormally formed blood vessels in the spinal cord. Without treatment, vascular malformations may permanently damage the spinal cord by compressing it, depriving it of oxygen-rich blood, or by rupturing and causing bleeding into the spinal cord. […] A vascular malformation can occur at any location along the spinal cord, causing different problems. Symptoms may appear either suddenly or gradually. Early symptoms may include: Numbness, tingling, or sudden pain in legs, Problems with walking or climbing stairs, Weakness on one or both sides of the body. […] As the condition progresses, symptoms may include: Headache, Numbness in the legs, Loss of bowel or bladder control, Neck stiffness, Sudden severe back pain. […] Of the two most common types of vascular formations of the spinal cord, spinal AVMs most often cause symptoms between the ages of 10 and 40, and are more common in men. Small children who are afflicted with spinal AVMs may show no symptoms. However, symptoms often develop as these children grow.
- #7 Spinal AVM: What It Is, Symptoms, Treatment & Prognosishttps://my.clevelandclinic.org/health/diseases/spinal-arteriovenous-malformation-avm
AVM symptoms can happen at any age. Most people experience symptoms between ages 15 and 60. It can vary by type. Most spinal AVMs are present at birth (congenital). But you can develop an AVM as an adult (acquired). […] Spinal AVM complications can be life-threatening and could include: Hemorrhage: The spinal AVM can rupture (break open) and cause internal bleeding. […] Surgery can correct a spinal AVM. Your outlook is best when a healthcare provider diagnoses and treats this condition before complications begin. This isnt always an easy task, as you might have a spinal AVM and not have symptoms right away. Not treating a spinal AVM can cause damaging and life-threatening complications like paralysis and bleeding. […] Many factors contribute to the surgery outcome, like your general health and the size and location of the AVM. Your provider can give you the best information about your situation.
- #8 Spinal Arteriovenous Malformation – Dr. Paul C. McCormickhttps://www.paulcmccormick.com/condition/spinal-arteriovenous-malformation/
A spinal arteriovenous malformation (spinal AVM) is an abnormal tangle of blood vessels in the spinal cord. […] The majority of spinal AVMs will cause neurological symptoms that worsen gradually over months or years. Such symptoms may include back pain, sensory loss, and leg weakness. […] But about 10 to 20 percent of spinal AVMs will cause symptoms suddenly. These symptoms may include weakness, numbness, difficulty urinating, loss or reduction of bowel or bladder control, or paralysis. In these cases, a vessel may have burst, causing a hemorrhage (bleeding). […] Any of these problems with blood flow, pressure, and nutrient delivery can injure the spinal cord tissues around the AVM. Tissue death in these areas can cause neurological dysfunction.
- #9 Spinal Arteriovenous Malformation (AVM) – Neurosurgery | UCLA Healthhttps://www.uclahealth.org/medical-services/neurosurgery/conditions-treated/spinal-arteriovenous-malformation-avm
Eighty-five percent of spinal AVMs involve progressive neurological symptoms over months to years, especially back pain associated with progressive sensory loss and lower extremity weakness. […] Ten percent to 20 percent involve a sudden onset of weakness, numbness, difficulty urinating, urinary incontinence, fecal incontinence, or paralysis (usually in patients younger than 30) as a result of hemorrhage.
- #10 Spinal AVM (Arteriovenous Malformation) » Lillian S. Wells Department of Neurosurgery at the University of Florida » College of Medicine » University of Floridahttps://neurosurgery.ufl.edu/patient-care/diseases-conditions/spinal-avm/
Spinal cord arteriovenous malformations are a group of blood vessel disorders that affect the spinal cord blood supply. […] The clinical presentation of spinal AVMs is specific to the type of AVM. The majority of these AVMs present with progressive neurological symptoms over several months to years. The symptoms are usually back pain associated with progressive sensory loss and lower extremity weakness. A small number of spinal cord AVMs present as a sudden onset of weakness, numbness, difficulty urinating, urinary incontinence, fecal incontinence, or paralysis due to acute hemorrhage.
- #11 Spinal arteriovenous malformationshttp://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S1665-50442021000200077
sAVM most commonly affects the upper thoracic and cervical spine. […] Initial symptoms are often non-specific and include symmetrical or asymmetrical sensory symptoms such as paresthesia in one or both feet, and diffuse or patchy sensory loss (17-72%), gait difficulties and motor disturbances (50-81%), pain including back or radicular pain (13-64%), and micturition difficulties (4-75%). […] In most patients (40-63%), progression lasts for 1-3 years before the diagnosis is made, but a protracted course with a duration of 3 years occurs in 10-34%. […] A gradually progressive course with stepwise deterioration is recorded in approximately 11-32% of patients. […] An acute onset is reported in 5-18% of patients. […] At the time of diagnosis, there are often considerable neurological deficits and much of the clinical presentation depends on the type of the sAVM.
- #12https://www.aurorahealthcare.org/services/aurora-spine-services/spinal-vascular-malformation
Symptoms of spinal vascular malformations vary. Spinal arteriovenous malformations (AVMs) interfere with normal blood flow. This can cause your arteries and veins to rupture and spill blood inside your spinal cord. AVMs can also push on your spinal cord and cause pain. […] Some people dont experience any problems. Other people have symptoms that appear suddenly and become worse over time. Early symptoms of these malformations may include: Balance or coordination problems, Difficulty walking or climbing stairs, Dizziness, Headache, Odd sensations, such as a feeling of burning or heat, Numbness, tingling or sudden pain in your legs, Weakness on one or both sides of your body. […] As the condition progresses, you may also experience: Erectile dysfunction (in men), Lack of sensation in your legs, Loss of bowel or bladder control, Lower back pain, Sensitivity to light, Seizures, Stiff neck.
- #13 Arteriovenous Malformations of the Central Nervous System – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK531479/
Spinal AVMs most often present with progressive back and leg pain accompanied by neurologic deficits secondary to mass effect or ischemic injury; an acutely hemorrhagic presentation is also possible. […] Spinal AVMs often present with chronic progressive neurologic deficits or hemorrhage leading to acute back pain with lower extremity pain, paresthesias, motor weakness, or bowel or bladder dysfunction. […] Two different symptomologies are typically seen in patients with spinal AVMs. An acute hemorrhage presenting with the sudden onset of severe back pain may be accompanied by lower extremity motor weakness, sensory deficits such as paresthesias and numbness, and bladder or bowel dysfunction. Alternatively, an unruptured spinal AVM may present with chronic progressive low back pain or lower extremity neurologic deficits secondary to progressive mass effect on the spinal cord. […] Complications of spinal AVMs include rupture with subsequent hemorrhage and neurologic deficits secondary to progressive mass effect.
- #14 Spinal Cord Arteriovenous Malformations (AVMs) – Neurologic Disorders – Merck Manual Professional Editionhttps://www.merckmanuals.com/professional/neurologic-disorders/spinal-cord-disorders/spinal-cord-arteriovenous-malformations-avms
Arteriovenous malformations (AVMs) in or around the spinal cord can cause cord compression, ischemia, parenchymal hemorrhage, subarachnoid hemorrhage, or a combination. Symptoms may include gradually progressive, ascending, or waxing and waning segmental neurologic deficits; radicular pain; and sudden back pain with sudden segmental neurologic deficits. […] AVMs may rupture into the spinal cord parenchyma, causing sudden, severe back pain and sudden segmental neurologic deficits. Rarely, high cervical AVMs rupture into the subarachnoid space, causing subarachnoid hemorrhage with sudden and severe headache, nuchal rigidity, and impaired consciousness. […] AVMs may compress nerve roots (causing pain down the distribution of a nerve root) or the spinal cord (causing segmental neurologic deficits that gradually progress or that wax and wane); they may rupture (causing sudden, severe back pain and sudden segmental neurologic deficits).
- #15 Spinal Vascular Malformation – Interventional Neuroradiology | UCLA Healthhttps://www.uclahealth.org/medical-services/radiology/interventional-neuroradiology/diseases-treated/spinal-vascular-malformation
Larger malformations tend to cause symptoms in a younger age group than smaller AVMs. […] Symptoms from spinal AVMs can occur in a gradual fashion or acutely and dramatically. The symptoms include abnormal sensation in the body, back pain, weakness in the extremities, sexual dysfunction, and bowel/bladder disturbances. […] The symptoms include progressive weakness and numbness in the lower extremities, bowel bladder disturbance, back pain, and sexual dysfunction. […] In our institutional experience, gait improves more often than bladder and bowel dysfunctions. Also, the lesions left untreated for more than 18 months resist to our treatment. A spinal vascular malformation should be referred to a highly experienced center and appropriate management plan has to be made as soon as possible to maximize the chance of recovery.
- #16 Arteriovenous Malformation (AVM): Causes & Symptomshttps://my.clevelandclinic.org/health/diseases/16755-arteriovenous-malformation-avm
AVMs cause harm in the following ways: Bleeding. The force of the blood flow from your arteries brings a lot of pressure to the AVM. Veins have weak walls and cant always adjust to the pressure of blood flow. If your veins cant handle the blood pressure, they might burst and bleed. Bleeding into surrounding tissue can cause permanent damage. Significant bleeding can result in death. […] Complications of brain AVMs include: Brain bleed/stroke. This is the biggest risk of having an AVM. A bleed from an AVM in your brain causes a stroke, brain damage or seizures. AVMs in these enclosed areas can also press on and displace parts of your brain and spinal cord. […] If you have an arteriovenous malformation and dont get treatment, theres a 2-4% chance per year that itll bleed.
- #17 Spinal arteriovenous malformations | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/spinal-arteriovenous-malformations?lang=us
Clinical presentation is variable, ranging from progressive myelopathy (Foix-Alajouanine syndrome), often with delayed diagnosis, to catastrophic spinal subarachnoid hemorrhage (see: coup de poignard of Michon 3). […] Both surgery and angioembolisation have a role in the treatment of spinal AVMs 6. […] myelopathy from venous congestion/hypertension […] hemorrhage: within the cord parenchyma or subarachnoid space 6 […] high-flow AVMs may cause arterial steal from adjacent spinal cord segments 6 […] myelopathy from large AVMs (rare) 6.
- #18 Arteriovenous Malformations (AVMs) | National Institute of Neurological Disorders and Strokehttps://www.ninds.nih.gov/health-information/disorders/arteriovenous-malformations-avms
Symptoms of AVMs can be mild or severe. They may include: […] Symptoms caused by AVMs can appear at any age. Because the malformations tend to result from a slow buildup of neurological damage over time, they are most often noticed when people are in their 20s or older. […] The greatest potential danger posed by AVMs is hemorrhage. […] AVMs can damage the brain or spinal cord by: […] AVMs affect oxygen delivery to the brain or spinal cord by altering normal patterns of blood flow through the arteries, veins, and capillaries. […] Bleeding into the brain, called intracranial hemorrhage, can result from the combination of high blood vessel internal pressure and vessel wall weakness. […] Massive hemorrhages, however, can occur if the physical stresses caused by extremely high blood pressure, rapid blood flow rates, and vessel wall weakness are great enough. […] Large AVMs can press on surrounding brain or spinal cord structures and cause damage.
- #19 Spinal AVM – UF Healthhttps://ufhealth.org/conditions-and-treatments/spinal-avm
Symptoms aeries because the AVF causes venous congestion and hypertension, resulting in poor perfusion of the spinal cord. […] The majority of these AVMs present with progressive neurological symptoms over several months to years. The symptoms are usually back pain associated with progressive sensory loss and lower extremity weakness. A small number of spinal cord AVMs present as a sudden onset of weakness, numbness, difficulty urinating, urinary incontinence, fecal incontinence, or paralysis due to acute hemorrhage.
- #20 Neurosurgical Consultants: Spine Disorders: Spinal Arteriovenous Malformationshttps://www.neurosurgerydallas.com/2_2_2_11.php
Spinal Arteriovenous Malformations (AVM’s) are rare disorders. These defects of the circulatory system consist of an abnormal connection between the normally higher intravascular pressure arterial system and the lower pressure venous pathways. An AVM can occur when the intervening capillary system is absent. Haemorrhage into surrounding tissue is a real threat since the thin walled vein portion of this malformation may be unable to sustain the higher intravascular blood pressure that is directly shunted from the thicker muscular walled arteries. Ischemia (lack of blood born oxygen) is a frequent problem in the absence of the capillary system which is designed to release oxygen and nutrients to these tissues. As pressure within the venous system increases, the vein dilates resulting in slowing of blood flow. Slow blood flow leads to ischemia. Additionally, the surrounding tissue (the Neurons and Fibre Tracts) is subjected to increased localized pressure from these dilated veins. This local pressure produces ischemia as well. As a result, the tissue suffers from several sources of decreased blood perfusion and is then also predisposed to hemorrhage.
- #21 A rare case report of endovascular management of pediatric spinal arteriovenous malformation | Egyptian Journal of Radiology and Nuclear Medicine | Full Texthttps://ejrnm.springeropen.com/articles/10.1186/s43055-022-00940-8
Spinal arteriovenous malformations (AVM) consist of a heterogeneous group of pathological vascular entities that affect the spinal cord parenchyma either directly or indirectly. […] She presented with weakness of both lower limbs and urinary incontinence. […] The onset of weakness was acute in nature and non-progressive. […] The spinal cord may be edematous on MRI, which appears as a hyperintense signal on T2WI MRI. […] Spinal AVM patients generally present with symptoms of backache and progressive myelopathy, gait abnormalities, sensory involvement, and bladder/bowel incontinence. […] Spinal cord AVMs are associated with a poor prognosis if left untreated. […] On follow-up, the child gradually improved over the next 1 year and could walk with assistance and regain complete bladder control. […] If presented early, patient can be taken up for endovascular embolization, which is a relatively safe procedure with better neurological outcome.
- #22 Neurosurgical Consultants: Spine Disorders: Spinal Arteriovenous Malformationshttps://www.neurosurgerydallas.com/2_2_2_11.php
„Mass effect” caused by growth and progressive enlargement of the intradural AVM can cause compression of the surrounding neural tissue which results in impairment in neurologic function. Patients with intradural AVMs may present with upper or lower extremity difficulties, whereas patients with Type 1 dural AVFs typically have lower limb extremity involvement only.
- #23 Spinal arteriovenous malformation with a calcified nodule: illustrative case in: Journal of Neurosurgery: Case Lessons Volume 6 Issue 13 (2023) Journalshttps://thejns.org/caselessons/view/journals/j-neurosurg-case-lessons/6/13/article-CASE23260.xml
A 22-year-old female presenting with a C67 AVM with a calcification nodule experienced new-onset acute right upper radiculopathy associated with extradural compression of the spinal cord. […] The clinical manifestations of spinal AVMs include hypoesthesia (sudden or gradual) as well as paresthesia, progressive paraparesis, back pain, and sphincter disturbances. […] If left untreated, these lesions can cause epidural compression-induced myelopathy or radiculopathy.
- #24 Exclusively Epidural Spinal Arteriovenous Malformation: A Short Reviewhttps://www.jneuropsychiatry.org/peer-review/exclusively-epidural-spinal-arteriovenous-malformation-a-short-review-12239.html
The incidence of exclusively epidural spinal arteriovenous malformation (EESAVM) is extremely low and there are only a few case-reports in literature. […] Early, correct recognition of the pathology is mandatory to halt the progression of the disease and minimize permanent spinal cord injury. […] Generally, the clinical presentation of EESAVM is slighter than intradural/intramedullary AVMs. Unruptured EESAVM may symptomless, or present as protracted, progressive neurological decline. Ruptured EESAVM may present acute back or thoracic pain subsequently with paraplegia. […] Common to Unruptured EESAVM are symptoms of myelopathy and pain, such as lower or upper extremity weakness, abnormal sensory, disturbance of gait, back pain, and bladder and/or bowel incontinence. […] Acute symptoms caused by spinal epidural hematoma are typical presentation of ruptured EESAVM, patients may be without any previous syndromes or secondary to unrecognized previous neurologic symptoms caused by EESAVM.
- #25https://www.singhealth.com.sg/patient-care/conditions-treatments/arteriovenous-malformation
Arteriovenous Malformation (AVM) presenting symptoms include: […] The risk of hemorrhage from an Arteriovenous Malformation (AVM) is estimated to be about 2 to 4 percent per year.
- #26 Vascular Malformations of the Brain and Spine in Children | PM&R KnowledgeNowhttps://now.aapmr.org/vascular-malformations-of-the-brain-and-spine-in-children/
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. […] 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. […] In the spinal cord, lesions with persistent perimedullary veins and those located in the cervical and upper thoracic level appear to have a higher risk of rehemorrhage.
- #27 Spinal arteriovenous malformationshttp://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S1665-50442021000200077
Type I (dural arteriovenous fistulas [DAVFs]): the typical presentation is radiculomyelopathy followed by progressive neurological deterioration. […] Type II (intramedullary glomus AVMs): the clinical course of these lesions is marked by progressive and fluctuating myelopathy, paraplegia and pain, overlaid by periods of acute neurological deterioration secondary to hemorrhage within the AVM. […] Type III (Juvenile AVMs): their clinical presentation is similar to that seen with Type II sAVMs. […] Type IV (perimedullary AVFs): presentation occurs in the third-to-sixth decade.
- #28 Neurosurgical Consultants: Spine Disorders: Spinal Arteriovenous Malformationshttps://www.neurosurgerydallas.com/2_2_2_11.php
Type 1 (AVF) patients with are usually over 40 years of age and occur more frequently in males than in females. Symptoms tend to increase over an extended period of months and years manifested by progressive weakness in the lower extremities as well as bowel or bladder control difficulties. Pain, located in the distal posterior thoracic spine with or without a significant radicular component, can be a prominent feature. Symptoms may be worsened by strenuous activity or a change in body position. […] Types 2-4 patients are usually younger than 30 years of age. They are often identified as a result of a hemorrhage (either subarachnoid or intra-parenchymal), vascular steal phenomenon (a form of ischemia), or, more rarely, pressure effect by their „mass” upon the Spinal Cord. Patients with Subarachnoid Hemorrhage usually experience sudden onset of a severe headache, stiff neck (meningismus), and photophobia (pain associated light). A Spinal AVM should be considered in the differential diagnosis of any patient with a Subarachnoid Hemorrhage who has negative results from a cerebral angiogram. Haemorrhage within the Spinal Cord substance (intraparenchymal) is usually associated with sudden neurologic deterioration, sudden onset of pain, and a distinct spinal level of neurologic dysfunction.
- #29 Spinal arteriovenous malformation (AVM) | UM Health-Sparrowhttps://www.uofmhealthsparrow.org/departments-conditions/conditions/spinal-arteriovenous-malformation-avm
Symptoms of a spinal arteriovenous malformation (AVM) can differ from person to person. Symptoms depend on where the AVM is found and how serious it is. Some people may not notice symptoms for many years, if at all. Others may experience symptoms that are life-threatening. […] Symptoms may start suddenly or slowly and may include: Trouble walking or climbing stairs. Numbness, tingling or sudden pain in the legs. Weakness on one or both sides of the body. […] As the condition gets worse, you may have more symptoms including: Sudden, serious back pain. Lack of feeling in the legs. Trouble urinating or having bowel movements. Headache. Stiff neck. […] Without treatment, a spinal arteriovenous malformation (AVM) can cause disability that gets worse over time. This is from damage to the spinal cord and surrounding tissues. This can cause: Trouble moving. Pain, tingling and numbness. Damage to the spine. Bulging blood vessel, known as an aneurysm. High blood pressure in the veins, known as venous hypertension. This can cause fluid to build up, called edema. It also can cause tissues to die due to lack of oxygen, known as spinal cord infarction. Hemorrhage, which can speed up spinal cord damage.
- #30 Mayo Clinic Health Library – Spinal arteriovenous malformation (AVM) | Swiss Medical Networkhttps://www.swissmedical.net/en/healtcare-library/con-20251921
Symptoms of a spinal arteriovenous malformation (AVM) can differ from person to person. Symptoms depend on where the AVM is found and how serious it is. Some people may not notice symptoms for many years, if at all. Others may experience symptoms that are life-threatening. […] Symptoms often begin when people are in their 20s but can occur at earlier or later ages. Some people are diagnosed under the age of 16. […] Symptoms may start suddenly or slowly and may include: Trouble walking or climbing stairs. Numbness, tingling or sudden pain in the legs. Weakness on one or both sides of the body. […] As the condition gets worse, you may have more symptoms including: Sudden, serious back pain. Lack of feeling in the legs. Trouble urinating or having bowel movements. Headache. Stiff neck. […] Without treatment, a spinal arteriovenous malformation (AVM) can cause disability that gets worse over time. This is from damage to the spinal cord and surrounding tissues. This can cause: Trouble moving. Pain, tingling and numbness. Damage to the spine. Bulging blood vessel, known as an aneurysm. High blood pressure in the veins, known as venous hypertension. This can cause fluid to build up, called edema. It also can cause tissues to die due to lack of oxygen, known as spinal cord infarction. Hemorrhage, which can speed up spinal cord damage.
- #31 Arteriovenous Malformation: Symptoms, Causes, and Treatmenthttps://www.healthline.com/health/arteriovenous-malformation
AVMs that form in your brain or spinal cord can be very serious. The risk of death is very high if the AVM ruptures, although the majority of people never develop problems. […] A ruptured AVM comes with a high risk of death if it occurs in your brain. If your doctor thinks your AVM is at a high risk of rupturing, they may recommend surgery to keep this from happening.
- #32https://journals.lww.com/joss/fulltext/2023/10030/exclusive_spinal_epidural_arteriovenous.8.aspx
Most spinal arteriovenous malformations (AVMs) are dural arteriovenous fistulas in which a singularly intradural venous drainage emanates from an extradural nidus. […] Adult patient presented with 7-month progressive spastic quadriparesis, hypertonia in lower limbs and graded sensory loss below c6 level. […] To conclude, E-AVMs may present with compressive myelopathy like symptoms. […] In 3 years of symptom onset, about 50% of patients would be confined to bed or wheelchair, and about 15% would suffer complications of chronic paraplegia leading to death. […] Unruptured EESAVM commonly presents with symptoms of myelopathy and pain in the dorsal and lumbar regions. However, ours is the first case in literature to present with symptoms mimicking myelopathy in the cervical region. […] High suspicion for this diagnosis is of prime importance, especially in acute condition such as spontaneous spinal epidural hematoma.
- #33https://journals.lww.com/joss/fulltext/2023/10030/exclusive_spinal_epidural_arteriovenous.8.aspx
Time lapse between symptom onset and surgical intervention is critical. Favorable outcomes can be achieved when surgical intervention is performed within 36 or 48 h after onset in patients with complete and incomplete deficits, respectively. […] Diagnosis for unruptured EESAVMs is a challenge and for those presenting with neurological deficits should be prudent to limit the neurological deficit and morbidity.
- #34 Spinal arteriovenous malformation (AVM) – Hancock Healthhttps://www.hancockhealth.org/mayo-health-library/spinal-arteriovenous-malformation-avm/
Without treatment, a spinal arteriovenous malformation (AVM) can cause disability that gets worse over time. This is from damage to the spinal cord and surrounding tissues. […] Spinal arteriovenous malformations (AVMs) can be hard to diagnose. The symptoms are similar to those of other spinal conditions. […] The goal of spinal AVM treatment is to lower the risk of the AVM bleeding. Treatment also can stop or prevent disability and other symptoms from getting worse.
- #35 Spinal arteriovenous malformation – Institute for Neurology and Neurosurgery | Northwell Healthhttps://www.northwell.edu/neurosciences/conditions/spinal-arteriovenous-malformation
Spinal AVM symptoms can vary greatly, depending on the location and size of the AVM. Symptoms can be mild, moderate or severe. Some people will develop noticeable symptoms, while others show no symptoms and may only have a spinal AVM discovered during treatment or diagnosis for another condition. Symptoms can be sudden or gradual and include: Numbness, stiffness or tingling in the legs […] Pain […] Headache […] Weakness […] Problems with walking, balance or coordination […] Difficulty controlling the bowels and bladder. The main goal of spinal AVM treatment is to reduce the risk of hemorrhage (bleeding) and to stop or prevent the progression of the spinal AVM.
- #36 Spinal Avm – Spinal AVM – AVM Survivors Networkhttps://avmsurvivors.org/t/spinal-avm/28017
I have spinal vascular malformation. I first developed a sudden neck pain and stiffness. This was sufficiently severe for him to present to hospital and describes what may be opisthotonic posturing at the time as well as severed headache. I developed urinary retention about that time and later began to notice I also notice bladder inconsistency and weak erections. began to notice his right leg dragging such that he could no longer walk quickly or run. Between 2016 and 2017 his gait continued to deteriorate and he had recurrent episodes of similar headaches and neck pain. […] I underwent an embolisation of this spinal vascular malformation under Professor at the Artemis Hospital in New Delhi. It was recommended that I undergo a follow-up angiogram after a 6-month interval but was unable to get in contact with the treating team and as a result, never had this imaging and has continued have episodes of neck stiffness since that time although neurologically I remained functionally. […] I had similar symptoms like you: weakness, numbness, pin needles on both legs starting late 2014. At this time, I can still walk 3 Km. There after I also have bladder control issues and lost feelings from waist down and balance problem. […] I still have spasm, but is stable.
- #37 Arteriovenous Malformations (AVM) | Living With Paralysishttps://www.christopherreeve.org/todays-care/living-with-paralysis/health/causes-of-paralysis/arteriovenous-malformations/
Sudden and severe pain in the back, usually at the level of the AVM […] Loss of movement and/or sensation, numbness, tingling, stiffness […] Loss of coordination, balance […] Incontinence or difficulty with urine and/or bowel elimination […] If the bleeding from an sAVM is extensive, the blood will extend into the area of least pressure which is into the brain. Therefore, it is important to know the symptoms of a brain AVM as well. Symptoms of a sAVM may first appear in the brain. […] Localized headache […] Seizures […] Loss of movement and or sensation to all or parts of the body, numbness, tingling […] Loss of coordination […] Changes in vision […] Loss of speech or slurring of speech […] Difficulty planning tasks […] Dizziness […] Mental confusion […] Hallucinations or dementia.
- #38 Arteriovenous Malformations and Fistulas (AVM/AVF) of the Spinal Cord: Practice Essentials, Background, History of the Procedurehttps://emedicine.medscape.com/article/248456-overview
Spinal arteriovenous malformations (AVMs) are a rare form of spinal blood vessel defect in which blood is received from the spinal feeding arteries, resulting in vessel engorgement that leads to clinical signs secondary to mass effect and ischemia. They account for about 10-15% of all spinal vascular shunts. Because of the rarity of spinal AVMs, studies regarding their diagnosis and treatment are limited. In addition, the various classifications that have been proposed historically make it difficult for young neurosurgeons to understand this disease. Because delayed initial diagnosis leads to irreversible damage to the spinal cord, neurosurgeons should always consider spinal AVM as part of the differential diagnosis. […] For many patients with spinal AVM, the symptoms are nonspecific. Therefore, we consider it critical to detect signal flow voids in enlarged spinal veins by using magnetic resonance imaging (MRI). An accurate understanding of the vascular structures is indispensable for deciding appropriate treatment strategies.