Moyamoya and Extracranial Vascular Involvement: Fibromuscular Dysplasia? A Report of Two Children

Vries, R.R. de; Nikkels, Peter G. J.; Laag, J. van der; Broere, G; Braun, Kpj

doi:10.1055/s-2003-44664

Cited by 21 publications

(10 citation statements)

References 6 publications

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“…Four patients (two children) died 0, 16, 41 and 57 months after presentation to the neurosurgical center. Both children had MMS related to FMD, with systemic vasculopathy and multi-organ involvement [ 16 ]. One died of pulmonary bleeding as a complication of balloon dilatation of stenosis of the pulmonary artery.…”

Section: Resultsmentioning

confidence: 99%

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Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Kronenburg

Kleinloog

Zwan

et al. 2021

JCM

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Information on presentation and outcome of moyamoya vasculopathy (MMV) in European countries is limited. We investigated patient characteristics, treatment and outcome of patients with MMV. We retrieved patient characteristics and treatment information and determined functional outcome (modified Rankin Score (mRS); type of school/work) by structured telephone interviews. We performed uni- and multivariable logistic regression analysis to determine predictors of poor outcome. We included 64 patients with bilateral MMV. In children (31 patients), median age was 5 years (interquartile range (IQR) 2–11) and in adults (33 patients), it was 33 years (IQR 28–41). Predominant mode of presentation was ischemia (children 84%; adults 88%). Modified Rankin Scale (mRS) at presentation was ≤2 in 74%. Revascularization was performed in 42 patients (23 children). Median follow-up time was 46 months (IQR 26–90). During this period, 16 patients had recurrent stroke(s) and four patients died. In 73% of the patients (83% surgical group; 55% medically treated group), mRS was ≤2; 46% were able to return to regular school or work, of whom only 41% were on the same level. Univariable analysis revealed that surgical treatment was associated with lower odds of poor outcome ((mRS ≥ 3), OR 0.24; p = 0.017). This association was no longer statistically significant (OR 3.47; p = 0.067) in the multivariable model, including age and diagnosis (moyamoya disease or moyamoya syndrome). In this cohort of patients with MMV who presented in a single European center, a large proportion had good functional outcome. Nevertheless, less than half were able to attend regular school or were able to work at their previous level, indicating a large impact of the disease on their life.

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Section: Resultsmentioning

confidence: 99%

“…One died of pulmonary bleeding as a complication of balloon dilatation of stenosis of the pulmonary artery. The other child had a sudden death as a result of multiple infarctions in heart and lungs due to FMD [ 16 ]. One adult died because of two hemorrhagic strokes during the diagnostic workup.…”

Section: Resultsmentioning

confidence: 99%

Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Kronenburg

Kleinloog

Zwan

et al. 2021

JCM

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“…30 Intimal thickening, fibromyxomatous or cellular, 30–34 is the most consistent finding. Also observed is medial hyperplasia, 30,35 paucity or fragmentation of medial elastic fibers and mosaic pattern of medial elements, 30,31,33–36 and dilation with medial atrophy distal to areas of narrowing.…”

Section: Definitions and Assumptionsmentioning

confidence: 91%

“…30 Since “non-protected” (by large PA stenosis) regions of the vascular bed are often pathologically remodeled (T2 histopathology), presumably due to ↑P+Q, PH is due to both large and small PA pathology. 32,37 …”

Section: Definitions and Assumptionsmentioning

confidence: 99%

Pulmonary hypertension’s variegated landscape: a snapshot

Kulik

Austin

2017

Pulm. circ.

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The many types of pulmonary hypertension (PH) are so protean in their biological origin, histological expression, and natural history that it is difficult to create a summary picture of the disease, or to easily compare and contrast characteristics of one type of PH with another. For newcomers to the field, however, such a picture would facilitate a broad understanding of PH. In this paper, we suggest that four characteristics are fundamental to describing the nature of various types of PH, and that taken together they define a number of patterns of PH expression. These characteristics are histopathology, developmental origin, associated clinical conditions, and potential for resolution. The “snapshot” is a way to concisely display the ways that these signal characteristics intersect in select specific types of PH, and is an effort to summarize these patterns in a way that facilitates a “big picture” comprehension of this disease.

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“…Pulmonary MMRSV in 2 pediatric cases with pulmonary hypertension showed massive intimal hyperplasia without medial changes. 41,42 Pulmonary MMRSV in 2 adult cases showed marked hyperplasia of the intima and media with irregular waving or disrupted stratification of the internal elastic lamina. 43,44 Three of the 4 case reports diagnosed pulmonary vascular lesions as fibromuscular dysplasia (FMD).…”

Section: Histopathologic Findings Of Mmrsvmentioning

confidence: 98%

Moyamoya Vasculopathy and Moyamoya-Related Systemic Vasculopathy: A Review With Histopathological and Genetic Viewpoints

Abumiya,

Fujimura

2024

Stroke

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Systemic vasculopathy has occasionally been reported in cases of moyamoya disease (MMD). Since the pathological relationship between moyamoya vasculopathy (MMV) and moyamoya-related systemic vasculopathy (MMRSV) remains unclear, it was examined herein by a review of histopathologic studies in consideration of clinicopathological and genetic viewpoints. Although luminal stenosis was a common finding in MMV and MMRSV, histopathologic findings of vascular remodeling markedly differed. MMV showed intimal hyperplasia, marked medial atrophy, and redundant tortuosity of the internal elastic lamina, with outer diameter narrowing called negative remodeling. MMRSV showed hyperplasia, mainly in the intima and sometimes in the media, with disrupted stratification of the internal elastic lamina. Systemic vasculopathy has also been observed in patients with non-MMD carrying the RNF213 (ring finger protein 213) mutation, leading to the concept of RNF213 vasculopathy. RNF213 vasculopathy in patients with non-MMD was histopathologically similar to MMRSV. Cases of MMRSV have sometimes been diagnosed with fibromuscular dysplasia. Fibromuscular dysplasia is similar to MMD not only in the histopathologic findings of MMRSV but also from clinicopathological and genetic viewpoints. The significant histopathologic difference between MMV and MMRSV may be attributed to a difference in the original vascular wall structure and its resistance to pathological stress between the intracranial and systemic arteries. To understand the pathogeneses of MMD and MMRSV, a broader perspective that includes RNF213 vasculopathy and fibromuscular dysplasia as well as an examination of the 2- or multiple-hit theory consisting of genetic factors, vascular structural conditions, and vascular environmental factors, such as blood immune cells and hemodynamics, are needed.

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Moyamoya and Extracranial Vascular Involvement: Fibromuscular Dysplasia? A Report of Two Children

Cited by 21 publications

References 6 publications

Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

Pulmonary hypertension’s variegated landscape: a snapshot

Moyamoya Vasculopathy and Moyamoya-Related Systemic Vasculopathy: A Review With Histopathological and Genetic Viewpoints

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