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Published on: October 20, 2017
Rational approach to treatment of moyamoya disease in childhood
1Department of Neurosurgery, Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka, Japan. nobu@ns.med.kyushu-u.ac.jp
Insights
Early diagnosis and treatment of moyamoya disease in children are crucial. Evaluating cerebral perfusion reserve guides surgical indications, and noninvasive magnetic resonance angiography aids follow-up, improving patient outcomes.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Radiology
Background:
- Moyamoya disease in children necessitates early intervention to prevent cognitive and physiological deficits.
- Current management strategies in Japan focus on preoperative assessment and noninvasive follow-up.
Purpose of the Study:
- To report on the current treatment of childhood moyamoya disease in Japan.
- To detail preoperative evaluation methods for surgical indications.
- To describe the role of magnetic resonance angiography in noninvasive follow-up.
Main Methods:
- Review of treatment approaches for childhood moyamoya disease in Japan.
- Evaluation of cerebral perfusion reserve using positron emission tomography (PET) or single photon emission tomography (SPECT) with acetazolamide or CO2 challenge.
- Follow-up assessment using magnetic resonance angiography (MRA).
Main Results:
- Approximately 20% of pediatric moyamoya disease patients were managed medically or observed.
- Surgical interventions included single indirect bypass (30%), combined direct/indirect bypass (20%), and multiple indirect bypass (18%).
- Postoperative improvements in cerebral perfusion reserve correlated better with reduced ischemic attacks than with collateral formation.
Conclusions:
- Cerebral circulation and metabolism evaluation are vital for understanding moyamoya disease and optimizing treatment.
- Reduced perfusion reserve is a key surgical indication.
- Noninvasive MRA offers advantages for follow-up compared to conventional angiography, avoiding general anesthesia.
Abstract:
Early diagnosis and treatment of moyamoya disease in children is essential to minimize residual mental and physiologic deficits. Current treatment of childhood moyamoya disease in Japan, preoperative evaluation of perfusion reserve as a surgical indication, and the role of noninvasive follow-up by magnetic resonance angiography are reported. Approximately 20% of children with definite moyamoya disease were observed or treated medically. Among surgical procedures, single indirect bypass surgery was used in approximately 30% of all patients; combinations of direct and indirect bypass surgery, 20%; and multiple-indirect bypass surgery, 18%. Both adequate understanding of the primary condition and determination of optimal treatment, including specific operative procedures, required evaluation of cerebral circulation and metabolism. Surgical indications included reduced perfusion reserve in affected brain by positron emission tomography or single photon emission tomography with administration of acetazolamide or a CO2 load. Postoperative improvements of cerebral perfusion reserve show better correlation with disappearance of ischemic attacks than does angiographically demonstrated collateral formation. Follow-up evaluation with magnetic resonance angiography has advantages over conventional angiography because it is noninvasive and avoids general anesthesia.
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