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Erratum. Results of more than 20 years of follow-up in pediatric patients with moyamoya disease undergoing pial synangiosis

Edward R. Smith

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Editorial. Growing research: how neurosurgeons can lead the development of nonsurgical treatments for moyamoya disease

Edward R. Smith

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Progression of disease in unilateral moyamoya syndrome

Edward R. Smith and R. Michael Scott


Progression of vasculopathy associated with moyamoya syndrome is extremely variable. The authors review their experience in patients with unilateral moyamoya syndrome to identify factors predictive of contralateral clinical and imaging-documented disease progression.


The authors reviewed the clinical and imaging records of all patients with moyamoya syndrome and unilateral disease who underwent cerebral revascularization surgery between January 1985 and June 2006 by using a standardized surgical procedure, pial synangiosis.


Of 235 surgically treated patients with moyamoya syndrome, 33 (14%) presented with unilateral disease (4 adults and 29 children). There were 16 female and 17 male patients, with an average age of 10.4 years (26.8 years for adults and 8.1 years for children; range 1.5–39 years). Twenty patients presented with left-sided disease and 13 with right-sided disease.

The average follow-up after surgery was 5.3 years (3.1 years for adults and 5.6 years for children; range 1–16 years). During this period, 10 (30%) of 33 patients progressed to bilateral disease. The mean time until disease progression was 2.2 years (range 0.5–8.5 years). Factors associated with progression in this series included contralateral abnormalities on initial angiography, previous history of congenital cardiac anomaly, cranial irradiation, Asian ancestry, and familial moyamoya syndrome. Young age at diagnosis was associated with a more rapid rate of progression (age < 7 years, 0.9 years to progression and age ≥ 7 years, 3.1 years to progression).


Of patients with unilateral moyamoya syndrome, 30% will have progression of arteriopathy during long-term follow-up. In this series, the average time of progression from unilateral to bilateral angiographic disease was 2.2 years. Several factors, including contralateral abnormalities on initial imaging, congenital cardiac anomaly, previous cranial irradiation, Asian ancestry, and familial moyamoya syndrome, were associated with an increased risk of progression. Patients with known unilateral angiographic disease should undergo continued monitoring by using MR imaging and MR angiography at regular intervals. Treatment with pial synangiosis is safe and confers durable protection against stroke in patients with both bilateral and unilateral moyamoya syndrome.

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Introduction: Moyamoya Disease

R. Michael Scott and Edward R. Smith

This issue of Neurosurgical Focus is devoted to the topic of moyamoya disease/syndrome. When the senior editor (R.M.S.) was a neurosurgical resident in the late 1960s and early 1970s, the condition was virtually unknown in the Western hemisphere, and patients with “cerebrovascular insufficiency” and the typical arterial findings on angiography were believed to have a type of arteritis. The refinement of catheter angiography techniques and the development of the imaging modalities of CT and MR imaging clarified the significance of making the correct diagnosis of moyamoya disease in affected patients, and with the development of direct and then indirect revascularization procedures during this same period, neurosurgeons became involved in the disease's treatment.

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Editorial. A terrible border wall: a study of pediatric moyamoya exposes socioeconomic barriers to care in the United States

Edward R. Smith

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Late morbidity and mortality following revascularization surgery for moyamoya disease in the pediatric population

Madeline B. Karsten, Edward R. Smith, and R. Michael Scott


There are limited reports on long-term morbidity in pediatric patients who have undergone surgical revascularization for moyamoya disease (MMD). Here, the authors report long-term morbidity and mortality in a population of pediatric patients who underwent pial synangiosis for MMD from 1988 through 2016.


A single-center retrospective review of the hospital and personal operative databases of the senior authors was carried out to identify all patients who were treated for MMD at Boston Children’s Hospital between 1988 and 2016, and who experienced any episode of late morbidity or mortality, which the authors defined as an event resulting in significant neurological deficit or death occurring more than 1 year after revascularization surgery. Hospital records were reviewed to determine pertinent demographic data, the initial mode of patient presentation, and associated comorbidities. Radiographic studies, when available, were reviewed for documentation of the diagnosis and for confirmation of the late complication, and the literature on this topic was reviewed.


In total, 460 patients with MMD underwent surgery between 1988 and 2016 using the pial synangiosis surgical technique; 15 (3.3%) of these patients (9 females and 6 males) experienced documented late death (n = 14) or severe morbidity (n = 1). The median age at revascularization surgery was 8.0 years (range 1–21 years). The causes of these late complications were grouped into three etiologies: intraventricular or intracerebral hemorrhage (n = 8), systemic complications related to associated comorbidities or preoperative disabilities (n = 5), and the development of malignant brain tumors (n = 2). Four patients whose MMD was associated with a history of cranial radiation therapy died. These events occurred from as early as 2 years to as late as 27 years postoperatively.


The risk of late morbidities and mortality following pial synangiosis for MMD in the pediatric patient appeared to be low. Nevertheless, the occurrence of catastrophic cerebrovascular events, particularly intracerebral and intraventricular hemorrhage in the otherwise neurologically stable revascularized patient, was concerning. Although there is value in long-term surveillance of patients who have undergone surgery for MMD, from both a neurological and a general medical standpoint, particularly in patients with the risk factor of prior cranial radiation therapy, it is not clear from the data how the late deaths in this population could have been prevented.

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Introduction. Translational research advances in the evaluation and management of moyamoya disease

Edward R. Smith, Giuseppe Lanzino, Gary K. Steinberg, and Bin Xu

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Incidentally discovered lesions

Cormac O. Maher, Paul Klimo Jr., and Edward R. Smith

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Increasing precision in the management of pediatric neurosurgical cerebrovascular diseases with molecular genetics

Kristopher T. Kahle, Daniel Duran, and Edward R. Smith

Recent next-generation DNA and RNA sequencing studies of congenital and pediatric cerebrovascular anomalies such as moyamoya disease, arteriovenous malformations, vein of Galen malformations, and cavernous malformations have shed new insight into the genetic regulation of human cerebrovascular development by implicating multiple novel disease genes and signaling pathways in the pathogenesis of these disorders. These diseases are now beginning to be categorized by molecular disruptions in canonical signaling pathways that impact the differentiation and proliferation of specific venous, capillary, or arterial cells during the hierarchical development of the cerebrovascular system. Here, the authors discuss how the continued study of these and other congenital cerebrovascular conditions has the potential to replace the current antiquated, anatomically based disease classification systems with a molecular taxonomy that has the potential to increase precision in genetic counseling, prognostication, and neurosurgical and endovascular treatment stratification. Importantly, the authors also discuss how molecular genetic data are already informing clinical trials and catalyzing the development of targeted therapies for these conditions historically considered as exclusively neurosurgical lesions.

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Indirect bypass for maternal symptomatic moyamoya in the first trimester of pregnancy: case report

Katie P. Fehnel, Craig D. McClain, and Edward R. Smith

There are no practice guidelines for the treatment of moyamoya disease in pregnant women. The need for such guidelines, however, is evidenced by the numerous case reports, case series, and systematic reviews in the literature highlighting an at-risk period for female moyamoya patients of childbearing age. Here the authors review and interpret the existing literature as it applies to their index patient and expand the literature in support of treating select patients during pregnancy. The authors describe what is to their knowledge the first case reported in the literature of a patient successfully treated with indirect surgical revascularization during the first trimester, who went on to deliver a healthy term baby without complications.