Reversible Cerebral Vasoconstriction Syndrome during Cesarean Section Complicated by Seizure Activity, Intraparenchymal and Subarachnoid Hemorrhage

Abstract

Body of the Abstract: (1) To report a rare case of Reversible Cerebral Vasoconstriction Syndrome (RCVS) and associated intraparenchymal and subarachnoid hemorrhage following induction of spinal anesthesia in an uncomplicated, elective C-section (2) To discuss its potentially life threatening or well-being altering effects (3) To review the pathophysiology, diagnosis and potential treatments of the syndrome. Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is a rare condition defined clinically by symptoms of a severe recurrent headache described as a thunderclap headache, cerebral vasoconstriction of at least two different arteries on imaging, and resolution of vasoconstriction within three months. Complications include non-aneurysmal subarachnoid hemorrhage, seizure, stroke, and intracerebral hemorrhage. More than half of cases of RCVS occur postpartum or after use of vasoactive substances such as adrenergic or serotonergic drugs. Triggers include illicit drugs, eclampsia, or strenuous physical or sexual activity. As RCVS most commonly occurs during the postpartum period, it is extremely rare to encounter RCVS during the antepartum or peripartum periods. There has been only one other reported case of RCVS while undergoing spinal anesthesia for a C-section. Presented is a patient diagnosed with RCVS after spinal anesthesia during an elective C-section subsequently complicated by seizure, intracerebral and subarachnoid hemorrhage. Case Presentation: A 32-yearold G2 now P2002 patient presented at 39 6/7 weeks gestational age for elective primary C-section for a history of delivery complications with the first pregnancy. Medical history was unremarkable. First pregnancy labor epidural analgesia was uncomplicated. Spinal anesthesia induction was uneventful. Shortly thereafter, dizziness was reported and hypotension and bradycardia (P 42, BP 76/41) developed. Treatment included IV Ephedrine and Glycopyrrolate. Frontal headache developed. The patient then developed a tonic-clonic seizure with gaze deviation and upper limb rigidity lasting 45 seconds. An oral airway was placed for respiratory assistance with 100% oxygen. The patient became alert five minutes later. Vital signs: BP 168/98 P 122.C-section was performed. After incision, the patient had a second tonic-clonic seizure lasting 30 seconds. Midazolam was given. A healthy baby was delivered with Apgars of 7 and 9. The C-section was otherwise uncomplicated. Postoperatively, the patient was alert with stable vital signs. The neurologic exam was intact. The CT showed a 1.9 x 3.8 x 1.2 cm intraparenchymal hemorrhage-right frontal lobe with a subarachnoid hemorrhage in both the right frontal lobe and right sylvian fissure. Exam was normal. Headache continued. Tertiary care facility transfer was arranged. Tertiary care evaluation with 24-hour EEG monitoring, CT angiography (CTA) and CT venogram showed no evidence of venous sinus or cortical vein thrombosis. Magnetic resonance imaging (MRI) and transcranial doppler showed vasospasm diagnostic of RCVS. Subsequent CTA showed vasospasm of three branches of the carotid artery. Hospital discharge occurred on day 6. Six-month neurological follow-up showed resolution of hemorrhage and minimal residual symptoms. Discussion: Literature review discovered only one other case of RCVS with induction of regional anesthesia. To our knowledge the associated seizure activity coupled with intraparenchymal/subarachnoid hemorrhages has not been previously reported. RCVS, an extremely rare phenomenon is estimated to occur in 0.26% of headache patients; is more frequent in middle aged women and may have variable triggering factors. It may be associated with antidepressants, illicit drugs or sympathomimetic meds. Pregnancy and vasoconstrictive meds are risk factors with an occurrence rate of 5%. The pathophysiology is unknown but felt to be due to impaired cerebral vessel autoregulation. It is hypothesized that other factors and substances including endothelial dysfunction, pro and antiangiogenic factors, serotonin, cytokines and vascular endothelial growth factor may play some role. While MRI and doppler imaging may be of assistance, CTA is most useful for diagnosis. Since static imaging alone does not confirm the diagnosis, most cases likely go undiagnosed. Rarely, complications of RCVS include stroke, seizure, cerebral hemorrhage and Posterior Reversible Encephalopathy Syndrome. There is no prophylactic or therapeutic treatment proven effective for RCVS. Recommendations include symptomatic treatment of the headache, blood pressure, and delivery if eclampsia is suspected. Calcium channel blockers are often used for vasodilatory effects, although they have not been proven to improve symptoms or outcome in RCVS. Conclusion: RCVS is a very rarely occurring vascular disorder occasionally seen postpartum. This is the first reported case of associated seizure activity and resultant intraparenchymal and subarachnoid hemorrhage occurring after induction of spinal anesthesia for an uncomplicated C-section. It demonstrates the need for awareness of this disorder as well as knowledgeable hemodynamic anesthesia management specific to its unique features. The RCVS diagnostic and management challenges dictate that case collection and research be continued.