Abstract

Introduction: Moyamoya disease is a progressive bilateral steno-occlusive disease of the terminal internal carotid arteries accompanied by development of collateral moyamoya vessels. It affects a younger population and thus, women in their reproductive years. We aim to define the practices, comorbidities and outcomes of pregnancies in women with moyamoya disease. Method: We conducted a retrospective review of 33 patients and 49 successful pregnancies between 1992 and 2017 at Stanford Health Care/Lucile Packard Children’s Hospital from our institutional database by surveys and chart review. Results: To date we have completed 1483 revascularization procedures in 930 patients. Out of these 930 patients we identified 45 patients who had pregnancies immediately prior to the diagnosis of moyamoya disease and some after being diagnosed with moyamoya. 33 out of these 45 patients’ charts and consents were available for review. These 33 patients had 49 successful pregnancies. There were 20 single pregnancies, 10 women had 2 pregnancies each and 3 woman had 3 successful pregnancies. 22 pregnancies were NVD (normal vaginal deliveries), 2 were assisted vaginal and 25 were C-sections. Maternal age varied from 19 years to 40 years. Gestational age ranged between 32 weeks to 40 weeks. 8/49(16.3%) pregnancies were associated with transient ischemic attacks or stroke. 44 pregnancies had a history of revascularization before or during pregnancy, and 5 pregnancies occurred prior to any revascularization. 8/49(16.3%) pregnancies were associated with eclampcia and pre-eclampcia and 9/49(18%) with hypertension. Neonatal complications included low birth weight 2/49 (4%), Mild jaundice 2/49 (4%), Tachypnea 1/49 (2%), Acid reflux 1/49 (2%), NICU stay 3/49 (6%). Conclusion: Our study shows that the trend in the USA is towards vaginal delivery in patients with moyamoya disease: 51.1%, is less than whein compared with studies in Asia. We also show that both the patients and babies do well. The complication rate is minimal, including no strokes in the peripartum period in patients who have been treated. We would advocate vaginal deliveries in the absence of any other contraindications along with peripartum blood pressure control.

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