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.

Keywords: Cerebral venous sinus thrombosis; Edoxaban; Ovarian tumor; Reversible cerebral vasoconstriction syndrome; Single-photon emission computed tomography.

Figure 1:

(a,b) Sagittal and axial T2-weighted MR image shows a large cystic ovarian tumor of 5cm at maximum diameter. The tumor had arisen from the right ovary, the margin was smooth and the uterus was normal size. (c) Ovarian endometrioid tumor of low malignant potential showing glands similar to the complex hyperplasia of the uterine endometrium.

Figure 2:

(a) Brain magnetic resonance venography image obtained on admission showing occlusion of the left transverse and sigmoid sinuses. Magnetic resonance venography image obtained 28 days after admission showing recanalization of the venous sinus (d). (b) 3D TOF MRA showing high-grade left PCA stenosis. Improvement in vasoconstriction is observed on day 14 from ictus (e). (c) SPECT images obtained on day 2 from ictus showing left posterior ischemic lesion. SPECT image obtained on day 28 from ictus showing absence of the ischemic lesion (f).

Figure 3:

(a,c) Magnetic resonance angiography (MRA) confirmed the presence of a vasospasm involving the right anterior cerebral artery. On admission, MRA revealed vasoconstriction of the anterior cerebral artery. Vasoconstriction was normalized within 3 months. (b,d) Magnetic resonance venography (MRV) confirmed the presence of a thrombosis involving the superior sagittal sinus. On admission, MRV revealed thrombosis of the superior sagittal sinus, which normalized within 3 months.