Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions

Abstract

<jats:p>✓Neurosurgical interventions for moyamoya disease (MMD) in pediatric patients include direct, indirect, and combined revascularization procedures. Each technique has shown efficacy in the treatment of pediatric MMD; however, no single study has demonstrated the superiority of one technique over another. In this review, the authors explore the various studies focused on the use of these techniques for MMD in the pediatric population. They summarize the results of each study to clearly depict the clinical outcomes achieved at each institution that had utilized direct, indirect, or combined techniques. In certain studies, multiple techniques were used, and the clinical or radiological outcomes were compared accordingly.</jats:p> <jats:p>Direct techniques have been shown to aid a reduction in perioperative strokes and provide immediate revascularization to ischemic areas; however, these procedures are technically challenging, and not all pediatric patients are appropriate candidates. Indirect techniques have also shown efficacy in the pediatric population but may require a longer period for revascularization to occur and perfusion deficits to be reversed. The authors concluded that the clinical efficacy of one technique over another is still unclear, as most studies have had small populations and the same outcome measures have not been applied. Authors who compared direct and indirect techniques noted approximately equal clinical outcomes with differences in radiological findings. Additional, larger studies are needed to determine the advantages and disadvantages of the different techniques for the pediatric age group.</jats:p>

Understanding and treating moyamoya disease in children

“…91 Furthermore, if surgical revascularization is performed prior to infarction, the prognosis tends to be excellent even in the face of severe angiographic changes. 27,91,113 However, if left untreated, the angiographic process and clinical symptoms invariably progress, leading to clinical deterioration and possible irreversible neurological deficits.…”

“…In pediatric patients who have undergone surgical revascularization for treatment of symptomatic moyamoya disease, TIAs rapidly decrease or disappear and strokes rarely recur. 16,17,29,34,91,113 Because stroke can occur and lead to severe neurological impairment, it is unwise to wait for evidence of ischemic symptoms before recommending surgery. 89,90 Moreover, if surgical revascularization is performed before infarction occurs, patients with moyamoya disease who present with TIAs will frequently have excellent outcomes.…”

Headache attack followed by rapid disease progression in pediatric moyamoya disease—how should we manage it?

“…Based on these observations, follow-up MR examination should be planned with shorter interval, for example, every 3 to 6 months. In addition, it is well known that functional and/or intellectual outcomes are poor in pediatric patients presenting with ischemic stroke due to moyamoya disease [13][14][15]. To prevent the occurrence of cerebral infarction, therefore, regular imaging studies should also be planned with shorter intervals in the conservatively treated children with moyamoya disease, because MRI and MRA studies enable it to accurately detect disease progression at outpatient clinic [16].…”

Bilateral pial synangiosis in a child with PHACE syndrome

“…Many techniques have been described for surgical revascularization in children. 6,24,25,27,30,31 Most of the surgical literature distinguishes between direct and indirect revascularization techniques. Questions within pediatric neurosurgery still exist on which is the best technique.…”

“…In one reported case series, a direct superficial temporal artery to middle cerebral artery anastomosis is considered in children over the age of 4 when the donor vessel is at least 0.7 mm in diameter. 31 Many surgical treatment options have been outlined for conditions such as moyamoya syndrome, 6,24,27,30 and the durability of pial synangiosis in preventing stroke for children with moyamoya syndrome has been previously described. 25 It is unclear with PHACE syndrome if the extracranial donor vessel will continue to function and protect the brain against further cerebral ischemia.…”