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Published on: April 21, 2017
Posterior Cerebral Artery Insufficiency in Pediatric Moyamoya Disease
Ji Yeoun Lee1, Seung-Ki Kim2, Ji Hoon Phi2
1Department of Anatomy, Seoul National University College of Medicine, Seoul, Korea. ; Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Abstract:
The majority of clinical studies on moyamoya disease (MMD) have focused on anterior circulation. The disease involvement of posterior circulation in MMD, mainly in the posterior cerebral artery (PCA), has been mentioned since the early 1980s, and it has been repeatedly emphasized as one of the most important factors related to poor prognosis in MMD. However, its clinical features and outcome have only been elucidated during the last few years. In this review, the angiographic definition of PCA stenosis is summarized. The clinical features are elucidated as being either early-onset or delayed-onset, according to the time of PCA stenosis diagnosis in reference to the anterior circulation revascularization surgeries. The surgical strategy and hypothesis on the mechanism of PCA stenosis is also briefly mentioned. It appears that some MMD patients may show PCA stenosis during the early or late course of the disease and that the presenting symptoms may vary. Because the hemodynamic compromise caused by PCA stenosis may respond well to surgical treatment, clinicians should be aware of the condition, especially during follow-up of MMD patients.
Insights
Moyamoya disease (MMD) can affect posterior circulation, specifically the posterior cerebral artery (PCA). Understanding PCA stenosis is crucial for MMD prognosis and management, as it may benefit from surgical intervention.
Area of Science:
- Neurology
- Vascular Surgery
- Radiology
Background:
- Moyamoya disease (MMD) research predominantly focuses on anterior circulation.
- Posterior circulation involvement, particularly posterior cerebral artery (PCA) stenosis, is a known poor prognostic factor in MMD.
- Recent research has begun to elucidate the clinical features and outcomes associated with PCA stenosis in MMD.
Purpose of the Study:
- To review and summarize the current understanding of posterior cerebral artery (PCA) stenosis in moyamoya disease (MMD).
- To describe the clinical features of PCA stenosis, differentiating between early- and delayed-onset presentations.
- To briefly discuss surgical strategies and proposed mechanisms for PCA stenosis in MMD.
Main Methods:
- Review of existing literature on moyamoya disease and posterior cerebral artery stenosis.
- Summarization of angiographic definitions of PCA stenosis.
- Analysis of clinical features based on the timing of PCA stenosis diagnosis relative to anterior circulation revascularization surgeries.
Main Results:
- PCA stenosis can occur in MMD patients, either early or late in the disease course.
- Clinical presentations of PCA stenosis vary and can be categorized as early- or delayed-onset.
- Hemodynamic compromise due to PCA stenosis may be responsive to surgical treatment.
Conclusions:
- Posterior cerebral artery (PCA) stenosis is an important, though often understudied, aspect of moyamoya disease (MMD).
- Awareness of PCA stenosis and its varied clinical presentations is essential for clinicians managing MMD patients.
- Prompt recognition and potential surgical intervention for PCA stenosis may improve patient outcomes.

