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Mommy and Moyamoya: A Case Report of A Parturient with Moyamoya Disease
Abstract Number: T3D-9
Abstract Type: Case Report/Case Series
Best described in the Japanese literature, Moyamoya disease (MMD) is a rare genetically inherited cerebrovascular disease that affects roughly 0.086 per 100,000 persons in the United States. It is characterized by stenosis of the intracranial circulation leading to formation of fragile collateral vessels that are more susceptible to occlusion and/or rupture. Initial presenting symptoms include: TIAs, ischemic stroke, intracranial hemorrhage, seizures, and headaches. Diagnosis is confirmed by the visualization of collateral vessels as a “puff of smoke” on angiogram. (1) We present a case report of a parturient with MMD who presented for scheduled dilation and curettage (D&C) under spinal anesthesia to review unique challenges MMD presents for the obstetric anesthesiologist.
A 31 y/o African American G4P3A1 female with past medical history significant for MMD and Grave’s disease presented for a D&C. She started developing a series of ischemic events in 2013 with no residual deficits. Epidural analgesia was used during her previous term vaginal deliveries without complications. During the current D&C procedure, spinal anesthesia with 9 mg of bupivacaine 0.75% and 20 μg of fentanyl was used with titrated anxiolysis with midazolam. Phenylephrine 5-10 μg/min was infused throughout anesthesia and surgery to maintain her blood pressure within 20% of her baseline values. There were no complications or neurological deficits during her hospital stay and after discharge.
It is paramount for the obstetrical anesthesiologist to implement cerebro-protective strategies to maintain normotension and normocapnia to prevent both ischemic and hemorrhagic events in the peripartum management of women with MMD. Maintaining hemodynamic stability can be achieved with use of narcotics, anxiolytics, fluids, vasoactive agents, and neuraxial techniques as needed. Neuraxial anesthesia for surgery may allow detection of intraoperative neurologic symptoms. (2) With regards to mode of delivery, cesarean delivery may be associated with increased morbidity compared to vaginal delivery based on limited evidence.(3) Ultimately, the decision for operative vs. labor management requires a multidisciplinary discussion between obstetricians, neurosurgeons, and anesthesiologists to prevent neurologic morbidity and mortality for the parturient with MMD.
1. Parray T, et al. Moyamoya Disease: A Review of the Disease and Anesthetic Management. J Neurosurg Anesthesiol. 2011;23:100–9
2. Kato R, et al. Anesthetic management for cesarean section in moyamoya disease: a report of five consecutive cases and a mini-review. Int J Obstet Anesth. 2006;15:152-8
3. Weston E, et al. Moyamoya disease in pregnancy and delivery planning: A case series and literature review. Obstet Med. 2016;9:177-80