Reversible cerebral vasoconstriction syndrome concomitant with cerebral venous sinus thrombosis following ovarian tumor resection: A report of two cases

Abstract

Background: Reversible cerebral vasoconstriction syndrome (RCVS) presents with characteristic clinical, brain imaging, and angiographic findings. The most common clinical feature of RCVS is a severe acute headache, which is often referred to as a thunderclap headache due to the nature of its presentation. It may occur spontaneously or may be provoked by various precipitating factors. We present a rare case of RCVS concomitant with cerebral venous sinus thrombosis (CVST) in a woman who underwent resection of an ovarian tumor. Case Description: Case 1 – A 42-year-old woman was admitted to our hospital with severe headache radiating to the neck, with associated vomiting. She revealed a medical history of ovarian cancer and underwent an operation for the resection of the tumor, a month before presentation. After resection, her estradiol (E2) levels were reduced from 288 pg/ml to 31 pg/ml (normal range, 0–49 pg/ml). Initial imaging on admission to our hospital revealed the left posterior convexity subarachnoid hemorrhage. Magnetic resonance angiography (MRA) showed findings consistent with RCVS affecting the left posterior cerebral artery. Magnetic resonance venography (MRV) showed CVST of the left transverse and sigmoid sinuses. Single-photon emission computed tomography (SPECT) showed a left posterior ischemic lesion. These findings improved following treatment with nimodipine and anticoagulant. Case 2 – A 39-year-old woman presented with holocranial headache associated with vomiting. She was diagnosed with an ovarian tumor. She underwent an operation 3 months before presentation. After tumor resection, her E2 level decrease from 193 pg/ml to 19 pg/ml (normal range, 0–49 pg/ml). Magnetic resonance angiography (MRA) confirmed the presence of a vasospasm involving the right anterior cerebral artery. Magnetic resonance venography (MRV) confirmed the presence of thrombosis involving the superior sagittal sinus. She was discharged on postpartum day 31 without neurological deficits after treatment with anticoagulants. At her 3-month follow-up, both MRA and MRV were within the normal limits. Conclusion: This is the first report of two women diagnosed with RCVS with concomitant CVST following ovarian tumor resection. Marked reductions in postoperative E2 levels could have contributed to the development of CVST and RCVS.

Reversible Cerebral Vasoconstriction Syndrome and Female Sex: A Narrative Review

“…9 One case series of 2 women who were each diagnosed with both RCVS and cerebral venous thrombosis after ovarian tumor resection suggested that the ovarian tumor was associated with the venous thrombosis, while the rapid decreases in estrogen following surgery may have triggered RCVS. 10…”

“…RCVS and Female Sex diagnosed with both RCVS and cerebral venous thrombosis after ovarian tumor resection suggested that the ovarian tumor was associated with the venous thrombosis, while the rapid decreases in estrogen following surgery may have triggered RCVS. 10 In a comparison of RCVS among men, nonpregnant women, and postpartum women, Topcuoglu et al 6 found that nonpregnant women were older and more likely to have had prior migraine and serotonergic antidepressant use. Postpartum patients were younger and had less vasoconstrictive drug use than nonpregnant women.…”

“…3 In a retrospective study, only few differences in imaging and angiographic characteristics were found between women with and without hysterectomies and between premenopausal and postmenopausal women 6 though case reports have demonstrated potential associations between sex hormone administration or withdrawal and the occurrence of RCVS (eg, as noted by the case series discussed above of 2 women diagnosed with both RCVS and cerebral venous thrombosis in the setting of ovarian tumor resection). 3,10…”