A case of moyamoya disease presenting with progressive stroke during the late perinatal period successfully managed by bilateral revascularization surgeries

We report a case of moyamoya disease presenting with progressive stroke during the late perinatal period, which was successfully managed by emergency caesarean section and subsequent bilateral revascularization surgeries. A 27-year-old woman at 33 weeks of pregnancy suffered from progressive monoparesis on her left hand. Magnetic resonance (MR) imaging/angiography revealed definitive moyamoya disease presenting with acute spotty cerebral infarctions on the right cerebral hemisphere. At 34 weeks of pregnancy, she was transferred to our hospital, where both neurosurgeons and obstetricians were involved in her management. She underwent emergency caesarean section on the day of admission without deterioration of her neurological status, while MRI demonstrated newly-formed spotty infarction on the left hemisphere. Then she was managed by antithrombotic therapy for 4 weeks, and then underwent right superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis, followed by left STA-MCA anastomosis one month later without complications. Postoperative course was uneventful, and MRI after surgeries showed no expansion of ischemic lesion. The MRA showed apparently patent STA-MCA bypasses bilaterally. She was discharged without complication, and her symptom significantly improved two weeks after the left revascularization surgery.