Abstract

BACKGROUNDTransient neurological deficits (TNDs) develop after cerebral revascularization in patients with moyamoya disease (MMD). The authors report a rare pediatric MMD case with extensive decreased cerebral blood flow (CBF) and prolonged TNDs after combined revascularization.OBSERVATIONSA 9-year-old boy presented with transient left upper limb weakness, and MMD was diagnosed. A right-sided combined surgery was performed. Two years after the surgery, frequent but transient facial (right-sided) and upper limb weakness appeared. The left internal carotid artery terminal stenosis had progressed. Therefore, a left combined revascularization was performed. The patient’s motor aphasia and right upper limb weakness persisted for approximately 10 days after surgery. Magnetic resonance angiography showed that the direct bypass was patent, but extensive decreases in left CBF were observed using single photon emission tomography. With adequate fluid therapy and blood pressure control, the neurological symptoms eventually disappeared, and CBF improved.LESSONSThe environment of cerebral hemodynamics is heterogeneous after cerebral revascularization for MMD, and the exact mechanism of CBF decreases was not identified. TNDs are significantly associated with the onset of stroke during the early postoperative period. Therefore, appropriate treatment is desired after determining complex cerebral hemodynamics using CBF studies.

Highlights

  • Transient neurological deficits (TNDs) develop after cerebral revascularization in patients with moyamoya disease (MMD)

  • Observations We report a pediatric patient with MMD who experienced a paradoxical symptomatic cerebral blood flow (CBF) decrease after combined revascularization surgery

  • Numerous studies were performed on the development of TNDs and the risk of stroke associated with focal hyperperfusion after direct bypass for adult MMD.[11,12,13,19,20]

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Summary

BACKGROUND

Transient neurological deficits (TNDs) develop after cerebral revascularization in patients with moyamoya disease (MMD). Moyamoya disease (MMD) is characterized by stenoocclusive changes at the terminus of the bilateral internal carotid artery (ICA) and the development of basal collateral networks.[1,2] Direct and indirect combined revascularization surgeries were established for effective treatment, and these surgeries comprise a superficial temporal artery (STA) middle cerebral artery (MCA) bypass for direct bypass and the placement of the vascularized pedicle using temporal muscle, dura mater, and periosteum for indirect bypass Evidence shows that these treatments reduce cerebral ischemia and the risk of future hemorrhagic events.[3,4,5,6] transient neurological deficits (TNDs), such as numbness or weakness in the extremities, aphasia, and dysarthria, are wellrecognized major events that develop in the early postoperative period.[7,8,9,10] Cerebral blood flow (CBF) studies indicated that these symptoms were caused by focal hyperperfusion as a result of rapid increase in blood flow due to the direct bypass.[11,12,13] CBF studies showed that TNDs may occur in patients without focal.

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