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Moyamoya disease in pregnancy
Abstract Number: F 46
Abstract Type: Case Report/Case Series
Moyamoya (Japanese for misty) disease (MMD) is a cerebrovascular condition characterized by spontaneous bilateral occlusion of the distal portion of the internal carotid arteries. The incidence in Japan is reported to be 0.35 per 100,000 compared to the continental United States where the incidence is about 0.086 per 100,000.1 An increase in blood volume and cardiac output are common physiological changes during pregnancy. These factors, in addition to pregnancy induced hypertensive syndromes, may predispose pregnant patients with MMD to intracerebral hemorrhage.2 We present a case of a Parturient with MMD that was successfully managed during vaginal delivery.
Our patient was a 32 year-old G 7 P4 with MMD and a history of hypertension, cerebrovascular accidents, transient ischemic attacks and diabetes mellitus type II. She underwent STA/MCA bypass on the left side 8 months previously. The patient presented at her 35th week of gestation complaining of headache and had a blood pressure of 170/105 and proteinuria. She was admitted with a diagnosis of preeclampsia and treated with magnesium sulfate and anti-hypertensive medications. Observation and expectant care continued for several days, but with non-relenting severe headache and persistently high blood pressures an induction of labor was started. A lumbar epidural catheter was placed for labor analgesia and the patient delivered vaginally without complications.
MMD is a rare cerebrovascular disease which leads to progressive occlusion of the arteries in and around the Circle of Willis and the development of collateral circulation. Patients with MMD can present with ischemic events such as TIAs or stroke, hemorrhagic stroke, or epilepsy. Ischemic events occur from hypo-perfusion to the stenotic vessels and hemorrhagic events occur because of the delicate nature of the collateral circulation and aneurysms that may form. Pregnant patients have an increase in blood volume, cardiac output increase and an acute rise in blood pressure with pain during labor. Additionally, pregnant patients are at increased risk from hemodynamic changes associated with pregnancy, induced hypertension, pre-eclampsia, and eclampsia. All of these factors place pregnant patients with MMD at an increased risk for intracranial hemorrhage. Therefore tight blood pressure control is essential to prevent hemorrhage from hypertension and ischemic stroke from hypotension. Performing neuroaxial anesthesia allow for frequent neurological exams and it also avoids the stimulation that occur during induction of general anesthesia which can further raise the blood pressure
1.Scott R et.al. Moyamoya disease and moyamoya syndrome. NEJM. 2009;360:1226–1237.
2.Hashimoto K et al. Occlusive cerebrovascular disease with moyamoya vessels and intracranial hemorrhage during pregnancy. Neurol Med Chir 1988; 28: 588-593.