Introduction: Moya Moya disease (MMD) is a rare idiopathic progressive vasculopathy, characterized by angiographic stenosis of the terminal portion of the internal carotid arteries and proximal portions of its major branches with development of thin collateral network of small vessels presenting with transient ischemic attacks (TIA), headaches, seizures, ischemic/ hemorrhagic strokes. Surgical revascularization is indicated for patients who are symptomatic and for asymptomatic patients with high-risk neuroimaging findings. Pregnancy exacerbates the progression of this disease or induce cerebrovascular accidents.
Case summary: 25-year-old female presented postpartum with left sided weakness and numbness from Acute infarct of Right centrum semi-ovale. Magnetic resonance angiography brain showed severe stenosis with near complete occlusion of bilateral terminal internal carotid arteries, M1 segment of bilateral middle cerebral arteries and A1-2 segment of anterior cerebral arteries with collateral formation suggestive of Stage 3 Suzuki classification of MMD. She was planned for revascularization surgery without advice on contraception. She presented 3 months post-stroke at 11 weeks' gestation with headaches and TIAs. She was on clopidogrel, nifedipine LA. She was counselled on high risk of neurological complications (70%) due to her symptomatic MMD, MRA findings. An informed termination of pregnancy was done at 12 weeks' gestation.
Conclusion: There are case series of successful pregnancies with non-revascularized MMD. Data from large cohort studies indicate that post-bypass pregnancy is accompanied by low complication rates. Multidisciplinary Pregnancy Risk stratification should guide planning for pregnancy in patients with high risks. Contraception should be offered in high-risk symptomatic cases while awaiting surgery