Grading of moyamoya disease allows stratification for postoperative ischemia in bilateral revascularization surgery

Czabanka, Marcus; Boschi, Andrea; Acker, Güliz; Peña-Tapia, Pablo; Schubert, Gerrit Alexander; Schmiedek, Peter; Vajkoczy, Peter

doi:10.1007/s00701-016-2941-y

Cited by 17 publications

(17 citation statements)

References 14 publications

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“…Lastly, despite the disease being severe and may induce motor, cognitive sequelae in several patients, data on MMA natural history are lacking, mostly in Western countries. Although some factors, such as age, comorbidities, and cerebral hemodynamic impairment, have been recognized as potential outcome predictors, no standardized risk stratification systems are available, and therapeutic decisions are based on clinicians' and surgeons' experience [6]. For this reason, new scoring systems have been developed and are currently being validated.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, ideally, a multimodal approach is advisable to assess MMA patients and estimate their risk profile for cerebrovascular complications, to plan timely interventions and follow-up, always integrating the complementary findings provided by different techniques (Table 3). Accordingly, some classification systems integrating clinical, angiographic, and hemodynamic features have been proposed [6]. If the typical angiographic features are found unilaterally, and without identifiable determining causes, the disease is classified as "unilateral" or "probable" MMA [2].…”

Section: Neuroradiological Diagnosismentioning

confidence: 99%

“…It should be pointed out that the available treatment strategies are essentially shared between idiopathic MMD and MMS, including pharmacological and surgical stroke prevention treatments and symptomatic drugs. Given the absence of randomized trials and guidelines, therapeutic decisions are currently based on clinicians' and surgeons' experience and center tools [6]. In addition, as for other rare diseases, insufficient awareness of healthcare providers about this clinical entity bears a notable risk of delayed or wrong diagnoses and missed or inappropriate treatments [7].…”

Section: Introductionmentioning

confidence: 99%

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Clinical Management of Moyamoya Patients

Canavero

Vetrano

Zedde

et al. 2021

JCM

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Moyamoya angiopathy (MMA) is a peculiar cerebrovascular condition characterized by progressive steno-occlusion of the terminal part of the internal carotid arteries (ICAs) and their proximal branches, associated with the development of a network of fragile collateral vessels at the base of the brain. The diagnosis is essentially made by radiological angiographic techniques. MMA is often idiopathic (moyamoya disease-MMD); conversely, it can be associated with acquired or hereditary conditions (moyamoya Syndrome-MMS); however, the pathophysiology underlying either MMD or MMS has not been fully elucidated to date, and this poor knowledge reflects uncertainties and heterogeneity in patient management. MMD and MMS also have similar clinical expressions, including, above all, ischemic and hemorrhagic strokes, then headaches, seizures, cognitive impairment, and movement disorders. The available treatment strategies are currently shared between idiopathic MMD and MMS, including pharmacological and surgical stroke prevention treatments and symptomatic drugs. No pharmacological treatment able to reverse the progressive disappearance of the ICAs has been found to date in both idiopathic and syndromic cases. Antithrombotic agents are usually prescribed in ischemic MMA, although the coexisting hemorrhagic risk should be considered. Surgical revascularization techniques, which are currently the best available treatment in symptomatic MMA, are associated with good long-term outcomes and reduced ischemic and hemorrhagic risks. Given the lack of dedicated randomized clinical trials, current treatment is mainly based on observational studies and physicians’ and surgeons’ expertise.

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Section: Discussionmentioning

confidence: 99%

Section: Neuroradiological Diagnosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical Management of Moyamoya Patients

Canavero

Vetrano

Zedde

et al. 2021

JCM

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“…No treatment can stop or reverse the primary disease progress. Recurring ischemic symptoms and impaired cerebral blood flow are the main indicators for treatment [ 27 ], but also determine the severity of symptoms and the risk of intervention [ 26 , 28 ]. For asymptomatic patients, randomized trials are still missing to determine therapeutic procedures.…”

Section: Treatmentmentioning

confidence: 99%

The Genetic Basis of Moyamoya Disease

Mertens

Graupera

Gerhardt

et al. 2021

Transl. Stroke Res.

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Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by progressive spontaneous bilateral occlusion of the intracranial internal cerebral arteries (ICA) and their major branches with compensatory capillary collaterals resembling a “puff of smoke” (Japanese: Moyamoya) on cerebral angiography. These pathological alterations of the vessels are called Moyamoya arteriopathy or vasculopathy and a further distinction is made between primary and secondary MMD. Clinical presentation depends on age and population, with hemorrhage and ischemic infarcts in particular leading to severe neurological dysfunction or even death. Although the diagnostic suspicion can be posed by MRA or CTA, cerebral angiography is mandatory for diagnostic confirmation. Since no therapy to limit the stenotic lesions or the development of a collateral network is available, the only treatment established so far is surgical revascularization. The pathophysiology still remains unknown. Due to the early age of onset, familial cases and the variable incidence rate between different ethnic groups, the focus was put on genetic aspects early on. Several genetic risk loci as well as individual risk genes have been reported; however, few of them could be replicated in independent series. Linkage studies revealed linkage to the 17q25 locus. Multiple studies on the association of SNPs and MMD have been conducted, mainly focussing on the endothelium, smooth muscle cells, cytokines and growth factors. A variant of the RNF213 gene was shown to be strongly associated with MMD with a founder effect in the East Asian population. Although it is unknown how mutations in the RNF213 gene, encoding for a ubiquitously expressed 591 kDa cytosolic protein, lead to clinical features of MMD, RNF213 has been confirmed as a susceptibility gene in several studies with a gene dosage-dependent clinical phenotype, allowing preventive screening and possibly the development of new therapeutic approaches. This review focuses on the genetic basis of primary MMD only.

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“…The prevalence of TC in moyamoya vasculopathy is reported to be 42% in adults [4], while in the pediatric population, they are seen in 50% of all preoperative arteriograms [5, 6]. The presence of TC is related to disease severity (as suggested by the Berlin grading system) [7], advanced Suzuki stages, and disease progression [5, 6, 8]. TC might also predict a more pronounced response to revascularization surgery, as evidenced by better Matsushima grades at 1 year when compared to patients without TC [6].…”

Section: Introductionmentioning

confidence: 99%

Use of Intraoperative Neuronavigation to Identify Transdural Collaterals in Moyamoya Vasculopathy: A Simple Way to Make It Safer

Costa¹,

Kozyrev²,

Srinivasan³

et al. 2022

Pediatr Neurosurg

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Introduction Transdural collaterals (TC) from the external carotid artery (ECA) must be preserved when operating on patients with moyamoya vasculopathy. Several techniques have been used to identify the superficial temporal artery (STA) and middle meningeal artery (MMA) during surgery and prevent their damage. However, the use of neuronavigation for this specific purpose has never been described in the literature. We describe an operative case in which neuronavigation was used to preserve the TC (originating from the MMA), detailing our technique step by step and reviewing alternative methods previously reported. Case presentation A 6 year-old girl with moyamoya disease, who had developed marked bilateral TC from the MMA sparing the midle cerebral artery (MCA) territory, underwent staged bilateral indirect revascularization surgery. Intraoperative neuronavigation was used to identify the STA and MMA with their main branches during skin incision, craniotomy and dural opening. The neuronavigation matched the intraoperative findings exactly, and the target structures remained undamaged. The patient was discharged home after both surgeries with no neurological deficits. One year following surgery, the patient has excellent collateralization from both STAs, and is asymptomatic and neurologically intact. Conclusion With the use of intraoperative neuronavigation, the STA, MMA and their main branches, as well as their relationship to the bone, can be identified and preserved. This approach can help in preventing undesirable injury to TC during surgery, and may potentially prevent peri-opertaive stroke in patients with moyamoya vasculopathy undergoing revascularization surgery.

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Grading of moyamoya disease allows stratification for postoperative ischemia in bilateral revascularization surgery

Abstract: The proposed grading system allows to stratify for ischemic complications in MMD patients that receive bilateral, one-staged revascularization surgery. Future studies will have to investigate its use for predicting ischemic complications in other revascularization strategies for MMD.

Cited by 17 publications

References 14 publications

Clinical Management of Moyamoya Patients

Clinical Management of Moyamoya Patients

The Genetic Basis of Moyamoya Disease

Use of Intraoperative Neuronavigation to Identify Transdural Collaterals in Moyamoya Vasculopathy: A Simple Way to Make It Safer

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