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Post-Surgical Cognitive Outcomes of Moyamoya Disease: A Systematic Review
Devon L Mitchell1, Nathan A Shlobin1, Melissa A LoPresti1
1Division of Pediatric Neurosurgery, Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Lurie Children's Hospital, Chicago, Illinois, USA.
Background:
Moyamoya disease (MMD) is an occlusive arteriopathy leading to stroke. Progressive if left untreated, revascularization surgery has become the mainstay of treatment. Although clinical and radiographic outcomes of MMD after intervention are well-characterized, cognitive outcomes in pediatric patients remain unclear. We aimed to examine postoperative cognitive outcomes in children with MMD, examine factors associated with cognitive changes after intervention, and define the effect of revascularization surgery on cognitive outcomes.
Methods:
A systematic review was conducted following PRISMA guidelines searching PubMed, Embase, and Scopus databases. Articles met inclusion criteria if they studied pediatric patients undergoing revascularization surgery for MMD and examined cognitive outcomes either qualitatively or quantitatively. All data extracted from included articles was examined descriptively.
Results:
Of 1091 resultant articles, 12 articles containing 446 patients were included. Surgery was associated with maintained or improved full scale intellectual quotient (IQ), performance IQ, perceptual IQ, memory quotient and verbal memory. However, 70% of patients had impaired cognitive function, with associated poor school performance. Improvements in cognition were associated with increased cerebral blood flow, particularly to the middle cerebral artery, due to the development of collaterals. Female sex, shorter duration from symptom onset to surgery, and surgery after age 7 were predictive of cognitive improvement. Completed ischemic stroke prior to surgery was associated with poorer cognitive outcomes.
Conclusions:
Although children with MMD have improved cognitive outcomes following revascularization overall, a distinct subset experience cognitive impairment. Consideration of patient-specific and treatment-related factors is important to enable proper risk stratification and inform management approaches.
Insights
Revascularization surgery improves cognitive outcomes in pediatric Moyamoya disease (MMD) patients, but many still experience impairment. Factors like sex, age at surgery, and prior stroke influence cognitive results.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Cognitive Neuroscience
Background:
- Moyamoya disease (MMD) is a progressive cerebrovascular occlusive disease causing stroke in children.
- Revascularization surgery is the primary treatment for MMD, but its impact on pediatric cognitive function is not well understood.
- Existing research primarily focuses on clinical and radiographic outcomes, leaving a gap in knowledge regarding cognitive changes post-intervention.
Purpose of the Study:
- To systematically review and analyze the postoperative cognitive outcomes in pediatric patients with Moyamoya disease.
- To identify factors associated with cognitive changes following revascularization surgery in children with MMD.
- To define the specific effects of revascularization surgery on the cognitive performance of pediatric Moyamoya disease patients.
Main Methods:
- A systematic literature review was conducted adhering to PRISMA guidelines.
- Searches were performed across PubMed, Embase, and Scopus databases for relevant studies.
- Inclusion criteria focused on pediatric patients with MMD undergoing revascularization surgery and reporting cognitive outcomes (qualitative or quantitative).
Main Results:
- Out of 1091 articles, 12 studies involving 446 patients were included in the analysis.
- Revascularization surgery was linked to maintained or improved intellectual quotient (IQ) and memory functions.
- Despite overall improvements, 70% of patients exhibited cognitive impairment, with poor school performance noted. Factors like increased cerebral blood flow, female sex, shorter symptom-to-surgery duration, and surgery after age 7 predicted better outcomes, while prior stroke predicted poorer outcomes.
Conclusions:
- Pediatric Moyamoya disease patients generally show improved cognitive function after revascularization surgery.
- A significant subset of children with MMD experience persistent cognitive impairment post-surgery.
- Individual patient factors and treatment-related variables are crucial for risk stratification and optimizing management strategies in pediatric Moyamoya disease.
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