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Two Sisters with Moyamoya and Sickle Cell
Abstract Number: RF7A10-372
Abstract Type: Case Report Case Series
Moyamoya disease is known to have an association with Sickle Cell Disease (SCD), but no reports of parturients with both are known. Moyamoya is least rare in those of Japanese and Korean descent while SCD is most common in Africans. We discuss two African American sisters with moyamoya and SCD, who safely gave birth with different anesthetics.
Patient A, a 21 year-old G1P0 at 38w6d, presented in an acute pain crisis the day prior to her scheduled induction of labor (IOL). She underwent exchange transfusion and subsequent IOL. Her echocardiogram was normal as were blood counts and coagulation panel. She was on therapeutic dose enoxaparin for SVC thrombus (last dose day prior to admission). An epidural catheter was placed and bupivacaine 0.125% with fentanyl loaded. Patient controlled epidural analgesia was run with bupivacaine 0.0625% and fentanyl. Instrument-assisted delivery was considered but not implemented before delivery. Patient B, a 20 year-old G2P1 at 38w0d, presented with IUGR, desiring TOLAC. Her history was notable for prior successful cesarean delivery. She had no echocardiogram. Her blood counts and coagulation panel were normal, excepting mild anemia. She was also on prophylactic dose enoxaparin for history of deep vein thrombosis (last dose prior to admission). Oxytocin IOL resulted in fetal intolerance of labor, so she was taken for cesarean delivery. A spinal was dosed with 12 mg bupivacaine 0.75%, fentanyl 15 mcg, and morphine 100 mcg. Concurrently she received crystalloid bolus and a phenylephrine infusion. Cesarean delivery was without complication.
Both patients had no peripartum complications, including no neurologic symptoms. Neither received invasive monitoring. Both had evaluations by hematology and neurology during pregnancy, which recommended no special management other than continued adequate pain and hemodynamic control, hydration, and anticoagulants for their previous thrombotic complications. Neither had a recommendation for intracranial bypass. Obstetric anesthesia management for SCD is well described: normothermia, euvolemia, ensuring adequate analgesia, consideration of cardiovascular complications of sickling, and advance preparation for transfusion with multiple antibodies likely. Peripartum anesthetic management for moyamoya focuses on similar goals of normocarbia, normothermia, and normotension. Epidurals, CSE, and general endotracheal anesthesia have all been safely performed. Conservative reports favor cesareans with slowly-dosed epidurals, but more series with vaginal deliveries (usually instrument-assisted) and CSE cesareans are known. Our cases highlight this trend toward liberalization of delivery method and anesthetic as long as the goals are met.
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