Choroba moyamoya
Zapobieganie i profilaktyka

Choroba moyamoya (MMD) to postępujące zwężenie tętnic szyjnych wewnętrznych i ich proksymalnych odgałęzień, prowadzące do powstania kruchej sieci naczyń obocznych u podstawy mózgu. Wczesna diagnostyka i leczenie są kluczowe dla zapobiegania udarom mózgu i poważnym powikłaniom neurologicznym. Najskuteczniejszą metodą leczenia jest chirurgiczna rewaskularyzacja, która znacząco zmniejsza ryzyko udaru (iloraz szans 0,17, 95% CI 0,12-0,26, P<0,01) poprzez poprawę przepływu krwi i stabilizację hemodynamiki. Techniki rewaskularyzacji obejmują bypass bezpośredni (zespolenie tętnicy skroniowej powierzchownej z tętnicą środkową mózgu), bypass pośredni (neowaskularyzacja z wykorzystaniem angiogenezy) oraz metody łączone. Leczenie farmakologiczne, w tym kwas acetylosalicylowy (50-100 mg/dobę) oraz cilostazol, może wspomagać terapię chirurgiczną, choć skuteczność leków przeciwpłytkowych wymaga dalszej walidacji. Kontrola ciśnienia tętniczego (zalecane wartości: skurczowe <180 mmHg, rozkurczowe <105 mmHg) oraz leczenie nadciśnienia z użyciem inhibitorów ACE, ARB, diuretyków i blokerów kanału wapniowego są istotne, zwłaszcza w fazie ostrej krwotoku śródmózgowego.

Zapobieganie chorobie moyamoya

Choroba moyamoya (MMD) jest rzadkim schorzeniem naczyniowym charakteryzującym się postępującym zwężeniem tętnic szyjnych wewnętrznych i ich proksymalnych odgałęzień, co prowadzi do rozwoju sieci kruchych naczyń obocznych u podstawy mózgu. Należy podkreślić, że obecnie nie istnieją metody, które mogłyby całkowicie zapobiec wystąpieniu choroby moyamoya, zwłaszcza w przypadkach o podłożu genetycznym.12 Jednak odpowiednie postępowanie profilaktyczne może znacząco zmniejszyć ryzyko powikłań, w szczególności udarów mózgu.3

Wczesna diagnoza i leczenie

Wczesne wykrycie i odpowiednie leczenie choroby moyamoya ma kluczowe znaczenie w zapobieganiu udarom mózgu i innym poważnym powikłaniom neurologicznym.4 Ponieważ choroba ma charakter postępujący, opóźnienie w diagnozie i leczeniu może prowadzić do pogorszenia stanu neurologicznego i potencjalnie nieodwracalnych deficytów.5 U pacjentów z jednostronną chorobą moyamoya, szczególnie u dzieci, zaleca się regularne monitorowanie za pomocą badań diagnostycznych, aby śledzić ewentualną progresję choroby.6

Leczenie chirurgiczne jako podstawowa metoda profilaktyki

Leczenie chirurgiczne jest uznawane za najskuteczniejszą metodę zapobiegania udarom u pacjentów z objawową chorobą moyamoya.57 Badania wykazały, że interwencja chirurgiczna znacząco zmniejsza ryzyko udaru (iloraz szans 0,17, 95% przedział ufności 0,12-0,26, P<0,01).7 Rewaskularyzacja chirurgiczna ma na celu poprawę przepływu krwi do mózgu oraz stabilizację hemodynamiki naczyń mózgowych.8

Istnieją różne techniki rewaskularyzacji:

  • Bezpośredni bypass – najczęściej wykonywana procedura, polegająca na zespoleniu tętnicy skroniowej powierzchownej z tętnicą środkową mózgu, co zapewnia natychmiastową poprawę przepływu krwi.89
  • Pośredni bypass – polega na neowaskularyzacji powierzchni kory mózgowej przy użyciu mechanizmów angiogennych z przeszczepów opartych na szypule, takich jak synangioza opony miękkiej i przeszczepy mięśnia skroniowego. Efekty hemodynamiczne mogą pojawić się dopiero po kilku miesiącach.10
  • Połączone techniki rewaskularyzacji – łączą bezpośredni i pośredni bypass, aby osiągnąć zarówno natychmiastową, jak i późniejszą poprawę hemodynamiczną.10

Wczesne leczenie chirurgiczne może pomóc spowolnić postęp choroby moyamoya.11 Neurolog może zalecić zabieg rewaskularyzacji, jeśli u pacjenta wystąpią objawy lub udary, a także gdy badania wykazują dowody na niski przepływ krwi do mózgu.11

Leczenie farmakologiczne w profilaktyce

Mimo że leczenie chirurgiczne jest preferowaną metodą w chorobie moyamoya, terapia farmakologiczna może być stosowana jako uzupełnienie leczenia chirurgicznego lub jako alternatywa, gdy zabieg operacyjny nie jest możliwy.12 Leki przeciwpłytkowe są często stosowane w celu zapobiegania udarom niedokrwiennym, chociaż ich skuteczność w chorobie moyamoya jest zróżnicowana.13

Leki przeciwpłytkowe

Kwas acetylosalicylowy (aspiryna) jest konwencjonalnie stosowany u pacjentów z chorobą moyamoya w celu zapobiegania dalszym udarom.14 Mimo braku jednoznacznych dowodów na potencjalne korzyści stosowania leków przeciwpłytkowych w zapobieganiu udarom, wielu neurologów na całym świecie stosuje aspirynę w celu zmniejszenia ryzyka dalszych udarów oraz jako terapię podtrzymującą zapobiegającą zakrzepicy i zatorowości po chirurgicznej rewaskularyzacji. Zalecana dawka wynosi zwykle 50-100 mg.14 Dzieci z chorobą moyamoya często przyjmują codziennie aspirynę przez całe życie jako metodę zapobiegania udarom.1516

Cilostazol, selektywny inhibitor fosfodiesterazy III o działaniu przeciwpłytkowym, przeciwzakrzepowym i naczyniorozszerzającym, może być obiecującą opcją w leczeniu choroby moyamoya.17 Badania sugerują potencjalne korzyści z terapii cilostazolu, w tym zmniejszenie śmiertelności, poprawę perfuzji mózgowej i funkcji poznawczych.18 Wytyczne AHA/ASA z 2021 r. wskazują, że cilostazol w porównaniu z innymi lekami przeciwpłytkowymi może poprawiać przeżywalność, przepływ krwi w mózgu i funkcje poznawcze, choć wymaga to dalszej walidacji.19

Leczenie przeciwnadciśnieniowe

Zaleca się leczenie przeciwnadciśnieniowe u pacjentów z chorobą moyamoya, szczególnie w ostrej fazie krwotoku śródmózgowego, aby zapobiec poszerzeniu krwiaka.13 Docelowe wartości ciśnienia tętniczego nie są jednoznacznie określone, ale sugeruje się utrzymywanie ciśnienia skurczowego poniżej 180 mmHg i ciśnienia rozkurczowego poniżej 105 mmHg.13

Długotrwałe leczenie przeciwnadciśnieniowe zaleca się dla pacjentów z już rozpoznanym nadciśnieniem tętniczym, ponieważ jest ono czynnikiem ryzyka złych wyników u pacjentów z chorobą moyamoya.13 Lekami pierwszego rzutu są inhibitory konwertazy angiotensyny lub blokery receptora angiotensyny, diuretyki i blokery kanału wapniowego.13

Jednak rutynowe stosowanie leków przeciwnadciśnieniowych u pacjentów z chorobą moyamoya bez nadciśnienia tętniczego w celu pierwotnej profilaktyki krwotocznej lub niedokrwiennej nie jest zalecane.20

Leczenie obniżające poziom lipidów

Skuteczność leczenia obniżającego poziom lipidów w chorobie moyamoya nie jest dobrze określona. Jednak statyny wykazały korzyści w zapobieganiu udarom niedokrwiennym u pacjentów z chorobą miażdżycową i mogą również spowalniać progresję choroby moyamoya.13 Konieczne są dalsze badania, aby określić rolę terapii obniżającej poziom lipidów w chorobie moyamoya.

Indywidualizacja podejścia terapeutycznego

Ze względu na znaczną zmienność w prezentacji pacjenta, takiej jak wiek wystąpienia, objawy i choroby współistniejące, kluczowe jest przyjęcie dostosowanych podejść terapeutycznych, które uwzględniają unikalne cechy każdego pacjenta.21 Zindywidualizowane strategie mogą skutecznie łagodzić ryzyko zdarzeń niedokrwiennych i poprawiać ogólne wyniki leczenia pacjentów.21

Leczenie zachowawcze może być priorytetem w następujących sytuacjach:21

  • Hemodynamicznie stabilni, bezobjawowi lub łagodnie objawowi pacjenci
  • Pacjenci o wysokim ryzyku powikłań okołooperacyjnych
  • Progresja po chirurgicznej rewaskularyzacji
  • Pacjenci z waskulopatią systemową związaną z wariantami białka palca pierścieniowego 213 (RNF213)

U pacjentów dorosłych istotne jest potwierdzenie, że istniejące objawy nie są związane z upośledzeniem hemodynamicznym. W przypadkach bezobjawowej choroby moyamoya należy stosować okresowe oceny naczyniowe lub nowsze techniki, takie jak HR-MRI, aby zweryfikować, czy stan jest stabilny, co wskazuje, że interwencja chirurgiczna może nie być konieczna.22

Standaryzowane protokoły opieki okołooperacyjnej

Wprowadzenie standaryzowanego protokołu okołooperacyjnego przeciw nudnościom i wymiotom było związane ze zmniejszeniem okołooperacyjnych udarów u dzieci z chorobą moyamoya leczonych pośrednim bypassem.23 Badania pokazują, że standaryzowany schemat opieki okołooperacyjnej, w tym nawodnienie okołooperacyjne ze szczególnym uwzględnieniem łagodzenia pooperacyjnego bólu i nudności, może pomóc zapobiec okołooperacyjnym udarom i poprawić wyniki.23

Modyfikacje stylu życia

Modyfikacje stylu życia odgrywają istotną rolę w długoterminowym zarządzaniu chorobą moyamoya i mogą pomóc w zmniejszeniu ryzyka powikłań.2425 Zalecenia obejmują:

  • Dieta – bogata w owoce, warzywa, pełne ziarna, chude białka i zdrowe tłuszcze wspiera zdrowie naczyń. Zmniejszenie spożycia tłuszczów nasyconych, cholesterolu i sodu może pomóc w kontroli ciśnienia krwi i poziomu cholesterolu, zmniejszając tym samym ryzyko udaru.25
  • Nawodnienie – odpowiednie nawodnienie pomaga utrzymać objętość krwi i lepkość. Pacjenci są zachęcani do picia wystarczającej ilości wody w ciągu dnia, aby zapobiec odwodnieniu, które może nasilić objawy.26
  • Regularna aktywność fizyczna – dostosowana do możliwości i stanu zdrowia pacjenta, może poprawić zdrowie układu sercowo-naczyniowego i ogólne samopoczucie. Działania powinny być starannie dobrane, aby uniknąć nadmiernego wysiłku, szczególnie u pacjentów z ograniczeniami fizycznymi lub zagrożonych krwawieniem.26
  • Unikanie palenia i ograniczenie spożycia alkoholu – palenie może nasilać problemy naczyniowe, a nadmierne spożycie alkoholu może wpływać na ciśnienie krwi i ogólny stan zdrowia.262
  • Zarządzanie stresem – przewlekły stres może negatywnie wpływać na zdrowie układu sercowo-naczyniowego. Techniki redukujące stres, takie jak uważność, medytacja, joga lub inne techniki relaksacyjne mogą być korzystne.26
  • Odpowiedni sen – regularny i wystarczający sen jest niezbędny dla zdrowia neurologicznego. Pacjenci powinni dążyć do regularnego snu i zapewnić dobrą higienę snu.26

U dzieci z chorobą moyamoya może być konieczne ograniczenie intensywnej zabawy lub sportów, które powodują nadmierny wysiłek, aby zmniejszyć ryzyko udaru.27

Podsumowanie profilaktyki choroby moyamoya

Choroba moyamoya jest schorzeniem postępującym, dla którego nie ma obecnie leczenia przyczynowego ani specyficznych metod prewencji pierwotnej.141 Jednak odpowiednie leczenie może skutecznie zapobiegać udarom i innym powikłaniom.

Najskuteczniejszym sposobem zapobiegania udarom u pacjentów z objawową chorobą moyamoya jest chirurgiczna rewaskularyzacja, która poprawia przepływ krwi do mózgu.528 Leczenie farmakologiczne, w tym leki przeciwpłytkowe, przeciwnadciśnieniowe i obniżające poziom lipidów, może odgrywać rolę uzupełniającą w zapobieganiu zdarzeniom niedokrwiennym.29

Ścisła kontrola dodatkowych czynników ryzyka, takich jak dyslipidemia, nadciśnienie i cukrzyca, jest zdecydowanie zalecana.30 Modyfikacje stylu życia, w tym zdrowa dieta, odpowiednie nawodnienie, regularna aktywność fizyczna, unikanie palenia i alkoholu oraz odpowiedni sen, mogą przyczynić się do ogólnego zdrowia naczyniowego i zmniejszyć ryzyko powikłań.2526

Podejście do profilaktyki powinno być zindywidualizowane, uwzględniając specyficzne cechy pacjenta, takie jak wiek, objawy, stabilność hemodynamiczna i choroby współistniejące.21 Wczesne wykrycie i leczenie choroby moyamoya ma kluczowe znaczenie w zapobieganiu poważnym powikłaniom neurologicznym i poprawie długoterminowych wyników leczenia.4

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

Materiały źródłowe

  • #1 Moyamoya disease | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/moyamoya-disease
    There is no way to prevent moyamoya disease. However, moyamoya treatments can prevent strokes and other complications.
  • #2 Moyamoya disease – Types, Symptoms and treatment – Dr Deepak Agrawal
    https://drdeepakaiims.com/moyamoya-disease-types-symptoms-and-treatment/
    As Moyamoya disease is primarily a genetic disorder, there are no specific preventive measures to avoid its onset. However, early detection and prompt treatment can help prevent complications such as strokes and cognitive impairment. […] While home remedies cannot cure Moyamoya disease, certain lifestyle modifications may help improve overall health and well-being. These include: […] Engaging in regular physical activity to maintain a healthy weight and promote circulation. […] Avoiding tobacco use and excessive alcohol consumption can contribute to vascular problems.
  • #3 Moyamoya disease // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/moyamoya-disease
    There is no way to prevent moyamoya disease. However, moyamoya treatments can prevent strokes and other complications. […] Early detection and treatment can help prevent a stroke and serious complications.
  • #4 Moyamoya disease | Health Library | Memorial Health System
    https://www.mhsystem.org/health-library/con-20251908/
    There is no way to prevent moyamoya disease. However, moyamoya treatments can prevent strokes and other complications. […] Early detection and treatment can help prevent a stroke and serious complications. […] Treatment can help prevent strokes.
  • #5 Moyamoya Disease: Treatment, Procedure, Cost, Recovery, Side Effects And More
    https://www.lybrate.com/topic/moyamoya-disease
    Drugs such as antiplatelet agents (including aspirin) are usually given to prevent clots, but surgery is usually recommended. […] Revascularization surgery for symptomatic MMD is considered the standard treatment for preventing further stroke. […] The main objective of surgery is to augment intracranial blood flow using an external carotid system by either direct bypass or pial synangiosis. […] Treatment measures include aspirin which helps prevent the development of small blood clots developing within the narrowed vessels, anti-seizure medications which is used to stop the patients seizure disorder and calcium channel blockers which may improve symptoms of headache and in some patients reduce symptoms related to transient ischemic attacks. […] Although, if left untreated, the disease will progress, leading to clinical deterioration and potentially irreversible neurological deficits over time. […] The treatment results are usually permanent.
  • #6 Moyamoya Disease – SeattleNeurosciences.com
    https://seattleneurosciences.com/conditions/moyamoya-disease/
    One of the current controversies in managing moyamoya patients is regarding the degree of intervention which is appropriate when patients have presented with vascular changes which are limited to one cerebral hemisphere. […] It was therefore recommended that pediatric patients with known unilateral disease should undergo continued monitoring by diagnostic testing at regular intervals.
  • #7
    https://journals.lww.com/md-journal/fulltext/2015/12080/the_efficacy_of_surgical_treatment_for_the.39.aspx
    Surgical treatment significantly reduced the risk of stroke (odds ratio (OR) of 0.17, 95% confidence interval (CI), 0.120.26, P 0.01). […] Surgery is an effective treatment for symptomatic MMD patients, and direct bypass surgery may bring more benefits for these patients. […] MMD treatment is controversial; therapy mainly depends on the doctor’s experience. Drugs such as aspirin are widely used, but the effect is not beneficial. Thus, surgery is frequently recommended. […] In conclusion, surgical treatment reduced the risk of recurrent stroke in symptomatic MMD patients, especially in hemorrhagic MMD patients. In addition, compared to indirect bypass surgery, direct bypass surgery showed a lower secondary stroke rate and a similar perioperative complication rate. Based on these results, we recommended direct bypass surgery as the first choice for symptomatic MMD patients for a lower recurrent stroke risk. However, perioperative complication and the demanding nature of this surgical technique are important obstacles to consider for the treatment of MMD.
  • #8 Moyamoya Disease | Doctor
    https://patient.info/doctor/moyamoya-disease.htm
    The main treatment focuses on neurological protection, cerebral blood flow reconstruction, and neurological rehabilitation, such as pharmacological treatment, surgical revascularisation, and cognitive rehabilitation. […] Surgical treatment is the most effective method to restore the blood supply and increase cerebral perfusion in order to prevent secondary stroke in ischaemic MMD and to stabilise cerebrovascular haemodynamics to regress fragile moyamoya vessels and so prevent bleeding in haemorrhagic MMD. […] Endovascular treatment has become the current main-stream treatment for MMD-associated aneurysms. […] Direct revascularisation via anastomosis of the superficial temporal artery to the middle cerebral artery has been the most common procedure for addressing the middle cerebral artery territory, but also supports the anterior cerebral artery territory via leptomeningeal anastomoses.
  • #9 Moyamoya Center | Providence
    https://www.providence.org/locations/or/brain-and-spine-institute/programs/moyamoya-center
    Medicine alone cant prevent the disease from progressing. Almost two-thirds of patients with Moyamoya disease develop increasing symptoms over five years. Early diagnosis and appropriate treatment are very important. Surgery can restore blood flow to the brain by opening narrowed blood vessels or by creating bypasses around blockages. After surgery, most people experience no more strokes or related problems. […] Several different surgeries can be used to treat Moyamoya disease. These surgeries create detours around the damaged blood vessels and restore blood flow to the brain. By improving the blood flow, we can help prevent ministrokes, strokes and bleeding. […] The direct bypass method, called an extracranial-tointracranial bypass, improves blood flow immediately. It is the preferred method, and it is the one that our surgeons use most frequently for Moyamoya disease. […] When direct bypass is not an option, surgeons may perform one of three types of indirect bypass grafts. For patients who face higher risks with direct bypass and for those who may not have a suitable graft available, the indirect methods are good options.
  • #10 Moyamoya Disease | Doctor
    https://patient.info/doctor/moyamoya-disease.htm
    Indirect revascularisation relies on neovascularisation of the cortical surface using angiogenic mechanisms from pedicle-based grafts, such as pial synangiosis, and temporal muscle grafts. However, the haemodynamic protective effects may take months to develop and are not very predictable. […] Combined revascularisation includes direct and indirect bypasses, with indirect revascularisation to achieve both immediate and later haemodynamic improvement and serves as a fallback strategy in case the direct bypass fails. […] Neurological rehabilitation plays an essential part of recovery from ischaemic and haemorrhagic MMD, including for cognitive impairment.
  • #11 Mayo Clinic Health Library – Moyamoya disease | Swiss Medical Network
    https://www.swissmedical.net/en/healtcare-library/con-20251908
    There is no way to prevent moyamoya disease. However, moyamoya treatments can prevent strokes and other complications. […] Early surgical treatment can help slow progression of moyamoya disease. Your neurologist may recommend revascularization surgery if you develop symptoms or strokes. Surgery also may be recommended if tests show evidence of low blood flow to your brain. […] Treatment doesn’t cure moyamoya disease. But treatment can help prevent strokes.
  • #12 Moyamoya Disease | Cedars-Sinai
    https://www.cedars-sinai.org/health-library/diseases-and-conditions/m/moyamoya-disease.html
    Surgery is the preferred treatment for moyamoya disease. However drug therapy may be used as an alternative when surgery is not an option. […] When surgery is not an option, medication may be used as treatment. Medicines that prevent clots from forming, including aspirin, may be helpful. No drug, however, can prevent the arteries to the brain from narrowing. Because medications do not offer a permanent solution, surgery is the preferred treatment option whenever possible.
  • #13 Is medical management useful in Moyamoya disease?
    https://www.wjgnet.com/2307-8960/full/v12/i3/466.htm
    Moyamoya disease (MMD) involves progressive arterial stenosis, leading to cerebral infarctions and hemorrhages. Key treatments include revascularization surgery and supplementary medical therapy. Antithrombotic management, crucial for ischemic stroke prevention in MMD, requires a careful balance due to bleeding risks. Understanding cerebral infarction pathways, involving hemodynamic impairment and thromboembolism, is essential. Transcranial doppler is useful for emboli detection and screening. Antiplatelet therapy, especially Acetylsalicylic acid, is common, but its efficacy varies. Antihypertensive management is recommended during initial hemorrhage phases, while lipid-lowering strategies like statins show potential but need more research for specific guidelines in MMD. […] The primary treatment approach for MMD is surgical revascularization, while medical therapy is used as a supplementary treatment. Antithrombotic therapy, such as antiplatelet medications, anticoagulants, and thrombolytic drugs, may be employed to prevent infarctions in MMD. Although hemodynamic impairment is the primary cause of infarction, thromboembolism can also contribute. TCD monitoring can aid in detecting emboli and guide the use of antiplatelet treatment. Commonly used antiplatelet medications include ASA, clopidogrel, and cilostazol. However, the routine use of antithrombotic drugs in MMD lacks strong evidence from RCTs. Antihypertensive treatment is recommended for Moyamoya patients, particularly during the early phase of intracerebral hemorrhage, to prevent hematoma expansion. The target blood pressure remains uncertain, but it is suggested to maintain systolic blood pressure below 180 mmHg and diastolic blood pressure below 105 mmHg. Hypertension is a risk factor for poor outcomes in Moyamoya patients, and long-term antihypertensive treatment is advised for those with established hypertension. First-line antihypertensive agents include angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, diuretics, and calcium channel blockers. The effectiveness of lipid-lowering treatment in MMD is not well-established. However, statins have demonstrated benefits in preventing ischemic strokes in patients with atherosclerotic disease and may also slow the progression of MMD. Further research is necessary to determine the role of lipid-lowering therapy in MMD.
  • #14 Moyamoya Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK535455/
    It is important to keep in mind that there is no curative treatment for moyamoya disease. Early diagnosis of moyamoya disease coupled with timely surgical intervention is of utmost importance as medical therapies act only as secondary prevention and do not halt disease progression. Both, medical and surgical treatments are directed towards improving cerebral blood flow. Acute therapy for strokes and/or intracranial bleeding is performed as per standard protocols. […] Conservative management is mainly directed towards maintaining cerebral blood flow and preventing further strokes. Aspirin has been conventionally used among patients of moyamoya disease to prevent further strokes. However, there is no evidence of a potential benefit of antiplatelet use to stroke prevention since the mechanism of MMD does not involve an endothelial damage and thereby platelet adhesion. Nevertheless, many neurologists around the world use aspirin towards mitigating the chances of further strokes in light of other risk factors and as a maintenance therapy to prevent thrombosis and thromboembolism from the stenosed portion of vessels after surgical revascularization. The usual dose of 50-100 mg is recommended.
  • #15 Moyamoya Disease | Riley Children’s Health
    https://www.rileychildrens.org/health-info/moyamoya
    Moyamoya can occur in both children and adults. […] The only treatment for moyamoya is revascularization surgery, which helps restore blood flow to the brain. […] Aspirin. Children with moyamoya take a daily dose of aspirin for the rest of their lives as a method of stroke prevention.
  • #16 Moyamoya Disease – Goodman CampbellHamburger MenuSearch IconSearch IconX IconFacebook IconInstagram IconYoutube IconLinkedIn Icon
    https://www.goodmancampbell.com/conditions/brain/pediatric/moyamoya-disease/
    Moyamoya is a progressive condition, meaning that it gets worse over time, so children diagnosed with this condition need treatment to reduce their risk of stroke. […] The goal of treatment is to restore blood flow to the brain and remove the risk of stroke. Without surgery, many individuals will experience mental decline and multiple strokes because of the progressive narrowing of arteries. […] Aspirin Children with moyamoya take a daily dose of aspirin for the rest of their lives as a method of stroke prevention.
  • #17 Cilostazol for the management of moyamoya disease: a systematic review of the early evidence, efficacy, safety, and future directions in: Neurosurgical Focus Volume 55 Issue 4 (2023) Journals
    https://thejns.org/focus/view/journals/neurosurg-focus/55/4/article-pE9.xml
    Surgical revascularization remains the standard treatment for symptomatic moyamoya disease (MMD). As with any major surgical treatment, revascularization is associated with risks and limitations, denoting the need for noninvasive treatments to improve ischemic symptoms and prevent strokes. […] Cilostazol is a selective phosphodiesterase III inhibitor with antiplatelet, antithrombotic, and vasodilatory effects commonly used in peripheral vascular disease. […] The evidence corroborates the potential benefits of cilostazol for the management of MMD, although these findings should be interpreted with caution due to the small number of studies and lack of randomized trials. […] Additional studies are needed to assess the efficacy and safety of cilostazol therapy, especially in Western populations.
  • #18 Cilostazol for the management of moyamoya disease: a systematic review of the early evidence, efficacy, safety, and future directions in: Neurosurgical Focus Volume 55 Issue 4 (2023) Journals
    https://thejns.org/focus/view/journals/neurosurg-focus/55/4/article-pE9.xml
    Cilostazol appears to be a good candidate for the maintenance antiplatelet regimen after the acute postoperative period, and empirical evidence is needed to determine its safety and potential efficacy in this setting. […] The evidence on the safety of cilostazol treatment in the MMD population is currently limited. […] Cilostazol has nonetheless shown a reasonably benign safety profile in clinical trials when used in patients with noncardioembolic strokes. […] Cilostazol targets critical pathways in the pathophysiology of MMD and the evidence corroborates its benefits in terms of decreased mortality, improved cerebral perfusion, and cognitive function.
  • #19 Moyamoya Disease Guidelines: Guidelines Summary
    https://emedicine.medscape.com/article/1180952-guidelines
    The 2021 AHA/ASA Scientific Statement for adult moyamoya disease recommends the following for medical care of MDD patients: […] Antiplatelet use for prevention of ischemic events in surgical and nonsurgical patients with ischemic moyamoya vasculopathy may be reasonable. […] Compared with other antiplatelet drugs, the vasodilator cilostazol may improve survival, cerebral blood flow, and cognition, but this requires further validation. […] Recommendations for surgical care of MDD patients include the following: Patients with symptomatic MMD should be referred for surgical revascularization therapy. […] Endovascular treatment with stent or angioplasty is not recommended for ischemic moyamoya. […] Hemorrhagic aneurysms of moyamoya vessels should be treated either endovascularly or with revascularization surgery.
  • #20 Medical Management in Moyamoya Disease | IntechOpen
    https://www.intechopen.com/chapters/74846
    Medical treatment seems to be not entirely helpful in the treatment of Moyamoya disease. No evidence supports the benefits of any drug treatment in Moyamoya disease. The ischemic or hemorrhagic event in Moyamoya disease is not preventable with any medical treatment. However, most of the physicians still prescribe the antithrombotic drug for Moyamoya patients with an ischemic event. […] The preferred treatment for cerebral ischemia focuses on the correction of hemodynamic failure. This rationale makes surgical treatment essential, and medical management is not a principle for Moyamoya patients. […] The role of blood pressure control with antihypertensive drugs is not entirely clear for intracranial hemorrhage prevention in Moyamoya patients. […] The Japanese guidelines for Moyamoya disease management recommend giving antihypertensive drugs to control blood pressure during the acute phase of intracranial hemorrhage for preventing hematoma expansion. […] Routine use of antihypertensive drugs in Moyamoya patients without hypertension for primary hemorrhagic or ischemic prevention is not recommended. […] Experts do not recommend routinely prescribing antihypertensive drugs in Moyamoya disease without hypertension.
  • #21 Medical Management of Adult Moyamoya Disease: A Review and Relevant Cases With Ischemic Events
    https://j-stroke.org/journal/view.php?number=556&viewtype=pubreader
    Surgical revascularization is the primary treatment option for preventing ischemic and hemorrhagic events; however, the role of medical management has become increasingly recognized, particularly in cases involving asymptomatic patients or those at a high risk for surgical complications. […] Given the considerable variability in patient presentation, such as age of onset, symptomatology, and comorbid conditions, it is crucial to adopt tailored therapeutic approaches that address each patients unique characteristics. […] However, individualized strategies may effectively mitigate the risk of ischemic events and improve the overall patient outcomes. […] Medical management of MMD is essential, especially for patients who may not be immediate candidates for surgical treatment, such as (1) hemodynamically stable, asymptomatic, or mildly symptomatic patients; (2) patients at a high risk of perioperative complications; (3) progression after surgical revascularization; or (4) those with systematic vasculopathy related to ring finger protein 213 (RNF213) variants.
  • #22 Medical Management of Adult Moyamoya Disease: A Review and Relevant Cases With Ischemic Events
    https://j-stroke.org/journal/view.php?number=556&viewtype=pubreader
    Therefore, in adult patients, it is essential to confirm that any existing symptoms are not related to hemodynamic compromise. In cases of asymptomatic MMD, periodic vascular assessments or recent techniques such as HR-MRI should be employed to verify that the condition is stable, indicating that surgical intervention may not be necessary. […] Medical management is typically prioritized in these situations. […] Medical management, including antiplatelet use and control of risk factors, such as strict control of blood pressure, could be important to optimize outcomes in this setting. […] Current guidelines for using antiplatelet agents in patients with MMD state that antiplatelet therapy may be reasonable for patients with a history of ischemic stroke or transient ischemic attack (TIA) to reduce the risk of embolic stroke, although with a low supporting quality of evidence.
  • #23 Prevention of postoperative stroke in pediatric moyamoya patients: a standardized perioperative care protocol in: Journal of Neurosurgery: Pediatrics Volume 33 Issue 2 (2023) Journals
    https://thejns.org/pediatrics/view/journals/j-neurosurg-pediatr/33/2/article-p185.xml
    Instituting a standardized perioperative nausea and vomiting protocol was associated with reduction in perioperative strokes in children with moyamoya treated with indirect bypass surgery. […] Postoperative ischemia and stroke are major complications of revascularization surgery in patients with moyamoya. […] Few studies have investigated the role of perioperative management in decreasing the risk of perioperative stroke and long-term outcomes. […] We hypothesized that perioperative management plays a significant role in preventing ischemic complications and that development of a standardized perioperative nausea and vomiting protocol would be associated with decreased number of perioperative strokes. […] Our findings highlight the importance of having standardized protocols for the perioperative and postoperative period following indirect revascularization surgery in children with moyamoya. […] These data suggest that a standardized perioperative care regimen including perioperative hydration with a specific focus on relieving postoperative pain and nausea may help prevent perioperative stroke and improve outcomes.
  • #24 Moyamoya disease and syndrome: Knowing the difference can prevent a stroke | Brain | Pediatrics | UT Southwestern Medical Center
    https://devtest.utswmed.org/medblog/moyamoya-treatment-expertise/
    Moyamoya disease (MMD) predisposes some patients to start developing narrowed, or stenotic, arteries in childhood some patients are diagnosed around age 5 or, if it isn’t prevalent then, MMD can surface in the 20s and 30s. […] Complications from MMS can be prevented in most patients by taking better care of their health, such as managing diabetes. […] Neither MMD nor MMS can be cured, but treatment can reduce symptoms and prevent strokes. […] Lifestyle modifications that address diet and weight changes are very important in management. […] Some patients with MMD or MMS will need to take blood thinners for life after surgery to reduce the risk of stroke. Talk with your doctor about the safest long-term stroke prevention options for you.
  • #25 Management Of Moyamoya Disease: A Neurosurgeon’s Guide – FeM Surgery
    https://www.femsurgery.com/management-of-moyamoya-disease-a-neurosurgeons-guide/
    Surgical intervention is aimed at improving cerebral blood flow and reducing the risk of stroke. […] Regular neurological assessments can help detect early signs of reduced blood flow or mini-strokes. Medications like aspirin can be used to lower the risk of blood clots and strokes. […] Lifestyle and dietary choices play a role in the long-term management of Moyamoya disease. These considerations aim to promote overall health and reduce risk factors associated with the condition. […] A diet rich in fruits, vegetables, whole grains, lean proteins, and healthy fats supports vascular health. Nutrient-dense foods provide vitamins and minerals, which are necessary for maintaining optimal bodily functions. Reducing the intake of saturated fats, cholesterol, and sodium can help manage blood pressure and cholesterol levels, thereby reducing the risk of stroke.
  • #26 Management Of Moyamoya Disease: A Neurosurgeon’s Guide – FeM Surgery
    https://www.femsurgery.com/management-of-moyamoya-disease-a-neurosurgeons-guide/
    Adequate hydration helps maintain blood volume and viscosity. Patients are encouraged to drink sufficient water throughout the day to prevent dehydration, which may exacerbate the symptoms. […] Regular exercise, tailored to the individuals capabilities and health status, can improve cardiovascular health and overall well-being. Activities should be chosen carefully to avoid excessive strain, especially in patients with physical limitations or those at risk of bleeding. […] Avoid smoking, as smoking can exacerbate vascular problems. Alcohol consumption should also be limited, as excessive alcohol can affect blood pressure and overall health. […] Chronic stress can negatively impact cardiovascular health. Stress-reducing activities such as mindfulness, meditation, yoga, or other relaxation techniques can be beneficial. […] Adequate and regular sleep is necessary for neurological health. Patients should aim for consistent sleep schedules and ensure good sleep hygiene.
  • #27 Moyamoya Disease | Boston Children’s Hospital
    https://www.childrenshospital.org/conditions/moyamoya-disease
    Moymoya is treated with surgery to fix the narrowed arteries in the brain and decrease the risk of stroke. […] While surgery is the only viable treatment for moyamoya disease in the long term, your doctor may also recommend medication to manage some of your child’s symptoms. These may include aspirin (to help prevent blood from clotting) and calcium channel blockers, such as verapamil (to help lower blood pressure). […] To lessen the risk of stroke, your child may need to restrict strenuous play or sports that cause overexertion. […] Moyamoya is a progressive condition. This means the dangerous narrowing in the brain’s blood vessels will get worse over time. This can take years or months, but surgery is the only way to reduce the risk of a potentially life-threatening stroke.
  • #28 SciELO Brazil – Moyamoya disease and syndrome: a review Moyamoya disease and syndrome: a review
    https://www.scielo.br/j/rb/a/RnSpk536gD9rhd3QX7HykHj/
    Although there is currently no specific therapeutic strategy that is effective in preventing or reversing the background vascular abnormalities in MMD, interventions used for stroke prophylaxis have probably changed the natural history of the disease. […] The cornerstones of the clinical approach to MMD are prophylactic and generic symptomatic treatment, such as the use of antiplatelet drugs, anticonvulsant drugs, and pain management. […] Treatment with acetylsalicylic acid is strongly recommended to prevent recurrence of ischemic attacks, and clopidogrel or another thienopyridine may be used when acetylsalicylic acid is not tolerated or is ineffective. […] Therefore, pharmacological treatment is directed at aggressive prevention of new neurovascular events and no single-drug regimen is accepted as a gold standard for ischemic or hemorrhagic complications.
  • #29 Medical Management of Adult Moyamoya Disease: A Review and Relevant Cases With Ischemic Events
    https://j-stroke.org/journal/view.php?number=556&viewtype=pubreader
    Antiplatelet therapy (aspirin monotherapy) is recommended for patients with ischemic symptoms or those at a high risk of recurrence, but not for those with hemorrhagic manifestations or those at risk of bleeding. […] Evidence supporting medical management is limited; however, controlling the risk factors and judicious use of antiplatelet agents may help mitigate the risk of ischemic events and improve patient outcomes. […] Further research is needed to efficiently define the role of medical therapy and develop standardized treatment protocols for managing adult patients with MMD.
  • #30 SciELO Brazil – Moyamoya disease and syndrome: a review Moyamoya disease and syndrome: a review
    https://www.scielo.br/j/rb/a/RnSpk536gD9rhd3QX7HykHj/
    In addition, rigid control of additional risk factors, such as dyslipidemia, hypertension, and diabetes, is highly recommended. […] The main goal of surgical treatment is to minimize cerebral ischemia by enhancing CBF and decreasing the hemodynamic stress that causes cerebral hemorrhage. […] Revascularization surgery prevents stroke and secondary hemorrhage in patients with MMD, lowering the rate of recurrence of ischemic attacks and producing clinical outcomes that are significantly more favorable than are those achieved with conservative treatment. […] Surgical revascularization employs external carotid artery branches such as the superficial temporal artery and occipital artery as donor arteries, and it can be divided into direct and indirect types. […] A meta-analysis showed that, in adults with symptomatic MMD, surgery is superior to conservative treatment in terms of preventing future strokes, and direct bypass seems to be more effective than is indirect bypass, producing favorable long-term results.