Long-Term Outcomes of Pediatric Moyamoya Disease Monitored to Adulthood

Imaizumi, Tomoichi; Hayashi, Kitami; Saito, Kayoko; Ōsawa, Makiko; Fukuyama, Yukio

doi:10.1016/s0887-8994(97)00209-9

Cited by 173 publications

(105 citation statements)

References 12 publications

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“…Other measures to define this high-risk population, such as patient weight or radiographic measures of brain development, such as myelination status, might be equally useful Young children with moyamoya usually present with a severe ischemic burden and advanced arteriopathy and are frequently noted to have a poor prognosis. 5,6,8,12 Matsushima et al 12 studied mental and cognitive outcomes following encephaloduroarteriosynangiosis (EDAS) in pa tients with onset of disease before 5 years of age. They found that children who presented at less than 2 years of age had a poor mental prognosis, with 7 of 8 patients having an IQ less than 80 (less than 61 in all 7 cases).…”

Section: Discussionmentioning

confidence: 99%

Pial synangiosis in patients with moyamoya younger than 2 years of age

Jackson¹,

Lin²,

Manjila³

et al. 2014

PED

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Object Patients with moyamoya who are younger than 2 years of age represent a therapeutic challenge because of their frequent neurological instability and concomitant anesthetic risks. The authors report their experience with pial synangiosis revascularization in this population. Methods The authors reviewed the clinical and radiographic records of all patients with moyamoya in a consecutive series of patients under 2 years of age, who underwent cerebral revascularization surgery using pial synangiosis at a single institution. Results During a 12-year period (1994–2005), 34 procedures (bilateral in 15 patients, unilateral in 4) were performed in 19 patients younger than 2 years (out of a total of 456 procedures in 240 patients). Eighteen of these patients presented with either stroke or transient ischemic attack. The average age of the 19 patients at first surgery was 1.4 years (range 6 months–1.9 years). Unanticipated staged operations occurred in 3 patients, due to persistent electroencephalographic changes during the initial surgery in 2 cases and due to brain swelling during the procedure requiring ventriculostomy in the other. There were 2 perioperative strokes; both patients had postoperative seizures but made clinical recoveries. The average follow-up was 7 years (range 1–14 years). Long term, at follow-up, 13 patients (68%) were clinically independent for their age, with 8 (42%) having no significant deficit. Late complications included subdural hygroma evacuation (1), additional revascularization procedures performed years later for frontal lobe ischemia (2), late infarction (1), and asymptomatic ischemic change on routine follow-up MRI studies (1). All patients who had both pre- and postoperative angiography demonstrated progression of disease. Conclusions Despite the challenges inherent to this population, the majority of children with moyamoya under the age of 2 years have a good long-term prognosis. The data from this study support the use of pial synangiosis as a safe, effective, and durable method for treatment of moyamoya for most children in this potentially high-risk population.

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Section: Discussionmentioning

confidence: 99%

Pial synangiosis in patients with moyamoya younger than 2 years of age

Jackson¹,

Lin²,

Manjila³

et al. 2014

PED

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“…27 While Phi et al reported good long-term social outcomes in terms of education and employment, their results also revealed that a certain proportion of patients had difficulty planning their marriages and acquiring driver's licenses, and that approximately 20% of the respondents were dissatisfied with their treatment outcomes. 29 Several factors, including preoperative neurological impairment, 10,17,29 infarction, 13,14 age of symptom onset, 10,13 and duration after onset, 9 have been reported as affecting social or functional outcomes. Our result in univariate analysis is consistent with these reports.…”

Section: Discussionmentioning

confidence: 99%

Impact of posterior cerebral artery involvement on long-term clinical and social outcome of pediatric moyamoya disease

Funaki

Takahashi

Takagi

et al. 2013

PED

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Object In the study of pediatric moyamoya disease, information on long-term social outcomes and risk factors for unfavorable social outcomes remains insufficient. The authors analyzed the long-term results of surgical revascularization for pediatric patients with moyamoya disease to determine whether the involvement of a stenoocclusive lesion in the posterior cerebral artery (PCA), relatively common in pediatric moyamoya disease, represents an underlying predictor for unfavorable social outcomes. Methods Prospectively collected data on 61 consecutive patients with moyamoya disease who had undergone combined bypass surgery were analyzed. Neuroradiological features and other baseline clinical factors were incorporated into univariate and multivariate analyses to determine any association with an unfavorable social outcome, defined as difficulty attending regular school or obtaining regular employment. Results Posterior cerebral artery involvement detected by angiography on admission was noted in 22 (36.1%) of the 61 patients. Follow-up data were acquired in 56 patients (91.8%), and the mean follow-up period was 15.8 years. While transient ischemic attacks were eliminated in 52 (92.9%) of these 56 patients after surgery, and late-onset ischemic stroke was observed in only 1 patient during the follow-up period, 10 (17.9%) experienced an unfavorable social outcome. Although younger age at onset, longer duration between onset and surgery, infarction present on preoperative neuroradiological images, and PCA involvement had been identified as risk factors for an unfavorable social outcome in univariate analysis, only infarction present on preoperative images and PCA involvement remained statistically significant after multivariate adjustment. Conclusions Posterior cerebral artery involvement can be considered one of the underlying risk factors for unfavorable social outcome and should be studied further to improve social outcome in pediatric moyamoya disease.

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“…Many series have reported the efficacy of revascularization in this syndrome by using primarily direct superficial temporal artery (STA)-MCA bypass. [82][83][84][85][86][87][88][89] PET studies in Moyamoya disease have demonstrated re- duced CBF and CO 2 response and increased CBV, MTT, and OEF. 90 CBF is significantly lower in patients with Moyamoya disease than in healthy subjects of the same age.…”

Section: Moyamoya Diseasementioning

confidence: 99%

The Acetazolamide Challenge: Techniques and Applications in the Evaluation of Chronic Cerebral Ischemia

Vagal¹,

Leach²,

Fernández-Ulloa³

et al. 2009

AJNR Am J Neuroradiol

167

116

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SUMMARY:The acetazolamide (ACZ) challenge test is a useful clinical tool and a reliable predictor of critically reduced perfusion. In patients with chronic steno-occlusive disease, the ability to maintain normal cerebral blood flow by reducing vascular resistance secondary to autoregulatory vasodilation is compromised. Identification of the presence and degree of autoregulatory vasodilation (reflecting the cerebrovascular reserve) is a significant prognostic factor in patients with chronic cerebrovascular disease. The pharmacologic challenge of a vasodilatory stimulus such as ACZ can also be used to optimize the treatment strategies for these patients. The pathophysiology, methods, and clinical applications of the ACZ challenge test are discussed in this article. Viability of the cerebral parenchyma is dependent on the ability of the brain vasculature to provide adequate levels of cerebral blood flow (CBF). In patients with chronic stenoocclusive disease, the ability to maintain normal CBF by reducing vascular resistance is compromised. Identification of the degree of autoregulatory vasodilation reflects cerebrovascular reserve (CVR), which is a significant prognostic factor in chronic cerebrovascular disease.1-5 Flow reserve can be assessed with the use of paired blood flow measurements, with the initial measurement obtained at baseline and the second, after a vasodilatory stimulus, such as acetazolamide (ACZ). 6 Pathophysiology of Chronic Cerebrovascular DiseaseChronic cerebral hypoperfusion is usually the result of occlusion or stenosis of large arteries in the neck or the circle of Willis. Clinical symptoms and manifestations of brain ischemia in patients with chronic cerebrovascular disease (CVD) develop as a consequence of 2 main mechanisms: embolic events from atherosclerotic plaques resulting in local compromise of blood flow and systemic hemodynamic alterations that further reduce an already compromised cerebral perfusion state. 7The hemodynamic changes due to a decline in cerebral perfusion pressure have been studied by many investigators. 6,[8][9][10][11] Chronic CBF restriction causes a progressive decrease in cerebral perfusion pressure (CPP). Initially, decreases of CPP cause varying degrees of autoregulatory vasodilation of small distal arterioles.12 Powers 10 and Powers et al 11 proposed a 2-stage classification of hemodynamic impairment in patients with CVD. In stage I (autoregulatory vasodilation), autoregulation reduces cerebral vascular resistance. CBF and oxygen extraction fraction (OEF) are not significantly changed. Increases of cerebral blood volume (CBV) and mean transit time (MTT) are 2 parameters that reflect this initial phase of compensatory autoregulatory vasodilation. Further decreases of CPP beyond cerebral autoregulatory vasodilation capacity eventually result in stage II (autoregulatory failure), characterized by decreases of CBF and increases of OEF. When the CBF decreases, neurons increase the fraction of oxygen extracted from the blood to maintain normal neurologic function. ...

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Long-Term Outcomes of Pediatric Moyamoya Disease Monitored to Adulthood

Cited by 173 publications

References 12 publications

Pial synangiosis in patients with moyamoya younger than 2 years of age

Pial synangiosis in patients with moyamoya younger than 2 years of age

Impact of posterior cerebral artery involvement on long-term clinical and social outcome of pediatric moyamoya disease

The Acetazolamide Challenge: Techniques and Applications in the Evaluation of Chronic Cerebral Ischemia

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