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Reversible cerebral vasoconstriction syndrome as the cause of altered mental status in a parturient with Sickle Cell Disease
Abstract Number: SU-50
Abstract Type: Case Report/Case Series
Etiologies of altered mental status in pregnancy include those unique to the pregnant/postpartum state and those that occur in the general population with increased prevalence in the parturient. They include stroke, cerebral venous thrombosis, subarachnoid hemorrhage, reversible cerebral vasoconstriction syndrome (RCVS), infection, amniotic fluid embolism and opioid overdose. Many of these neurological diseases can lead to devastating complications if not recognized early.
We report a 33 yo G3P1 at 32 weeks with sickle cell disease (SCD) admitted with newly diagnosed oligohydramnios and sickle cell crisis. On arrival, the patient had bilateral lower extremity swelling and elevated blood pressures, concerning for development of preeclampsia (pre-E). Hypertension and pain were treated with IV Labetalol and hydromorphone. Despite escalation of antihypertensives, her BP continued to rise into HD#2 and 1 U pRBCs was given in anticipation of urgent cesarean section for worsening preeclampsia. Prior to going to the OR suite, the patient was somnolent, but arousable, and complaining of a severe headache. Cesarean section was performed under subarachnoid block without complications. In the immediate postoperative period, the patient remained somnolent and continued to complain of headache. Within the next few hours, the patient developed RUE and bilateral LE weakness concerning for a stroke. Head CT was obtained and revealed a frontoparietal intraparenchymal hemorrhage with subdural and subarachnoid extension, as well as mass effect on the lateral and third ventricles. The location of the hemorrhage, neurological symptoms, and diagnosis of pre-E suggested the cause was RCVS, though vasospasm from a subarachnoid hemorrhage was also possible, but less likely. The patient was transferred to the intensive care unit for further care. She was ultimately discharged with left sided upper extremity weakness.
Pregnancy in patients with SCD have high levels of maternal and fetal morbidity and mortality (2). Hemorrhagic or infarctive stroke should be considered patients presenting with acute neurological symptoms, and should have been considered in this patient. Stroke treatment in parturients with SCD requires urgent exchange transfusion to improve outcome, which was delayed in this patient due to the delay in diagnosis of her hemorrhagic stroke caused by RCVS. Pre-E, SCD, and pRBC transfusion were all possible triggers for RCVS in this case. Perhaps familiarity with the most common causes of acute neurological diseases in pregnant patients and those with SCD, management would have included early brain imaging and exchange transfusion, possibly avoiding long term neurologic sequelae.
1. Edlow A et al. The Lancet Neurology 2013; 12: 175-85
2. Boga C and Ozdogu H. Critical Reviews in Hematology/Oncology 2016; 98: 364-74