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Moyamoya Disease in Pregnancy: Management after Intracranial Bypass Grafting

DOI: 10.1155/2012/638471

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Moyamoya disease (MD) is a chronic, progressive cerebrovascular disease distinguished by bilateral stenosis or occlusion of the arteries around the circle of Willis with resulting prominent arterial collateral circulation. We describe a pregnant woman in whom this diagnosis was confirmed by cerebral angiogram and treated with bilateral superficial temporal artery-middle cerebral artery (STA-MCA) bypass grafting prior to conception. The patient was managed with strict blood pressure monitoring and low-dose aspirin antepartum, intrapartum, and postpartum. The patient presented in spontaneous labor at term and underwent a spontaneous vaginal delivery without complications. 1. Introduction MD is a progressive cerebrovascular disease distinguished by bilateral stenosis or occlusion of the arteries supplying the circle of Willis and resulting in a prominent arterial collateral circulation. The disease is characterized by intimal thickening in the walls of the internal carotid arteries bilaterally. Intraventricular, subarachnoid, and intracerebral hemorrhage has been described with MD. There is a paucity of the literature regarding management during pregnancy. We present a case of a woman with MD who underwent intracranial bypass grafting prior to conception and subsequently underwent a successful vaginal delivery. 2. Case A 30-year-old right-handed African American presented for prenatal care at 10 weeks of gestation. The patient had a past medical history that was significant for a “stroke” in 1999. In 2008, the patient presented to the emergency department (ED) with difficulty using her right hand and transient left-sided weakness and numbness. A preliminary diagnosis of transient ischemic attack (TIA) was made. Her evaluation at that time included an MRI which demonstrated encephalomalacia in the right frontal cortex; MRA at that time demonstrated “irregular vessels” but was otherwise interpreted as unremarkable. A follow-up evaluation by a neurosurgeon confirmed the diagnosis of MD based upon findings at a cerebral angiogram. Please see Figures 1(a) and 1(b) for images from her cerebral angiogram. The patient underwent bilateral STA-MCA bypass grafting. One month following the second procedure the patient presented to the ED with aphasia and right hemiplegia. Evaluation revealed a left basal ganglia hemorrhage. Her symptoms subsequently resolved. Figure 1: The attached pictures are digitally subtracted images from a cerebral angiogram of our patient. (a) Right vertebral injection showing irregular, serpiginous ill-defined vessels. (b) Left vertebral


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