Crescent-shaped intracranial hemorrhage in cerebral venous sinus thrombosis: A case report and review of literature

INTRODUCTION

Cerebral venous sinus thrombosis (CVST) is a rare but potentially life-threatening condition characterized by the formation of blood clots in the cerebral veins and sinus. This condition can lead to significant disruption of venous blood flow and increased intracranial pressure (ICP), causing a variety of neurological symptoms.^[1,2] CVST can involve different venous sinuses within the brain, with the superior sagittal sinus (SSS) being the most commonly affected. The clinical presentation of CVST is highly variable, making diagnosis challenging and necessitating a high index of suspicion, especially in young patients presenting with unexplained neurological symptoms such as headaches, seizures, or focal neurological deficits. Given its rarity and diverse presentation, CVST requires prompt recognition and treatment to prevent long-term complications or mortality.^[1,3] This case report discusses a 16-year-old female who presented with acute neurological symptoms secondary to CVST, highlighting the diagnostic approach, management, and outcomes in this rare but serious condition.

CASE REPORT

A 16-year-old female had been referred to our hospital after experiencing a 12-day history of persistent headaches. The headache had been described as intense, stabbing, and sharp, occasionally spreading from the eyes to the back of the head, and had been accompanied by dizziness. Eleven days before admission, the patient had suffered a generalized tonic-clonic seizure lasting approximately 2 min, followed by unconsciousness. During this period, she had experienced ten episodes of generalized seizures, each lasting several minutes. After the seizures had subsided, she had felt lethargic and fallen asleep. Upon regaining consciousness, she had exhibited motor aphasia, which was characterized by difficulty expressing words despite understanding instructions, as well as numbness and weakness on the right side of her body. She had initially been treated at a local hospital, where she had been diagnosed with status epilepticus and had received phenytoin 100 mg twice daily and piracetam 700 mg three times daily to control the seizures. Although the seizures had been managed effectively, the headache and neurological symptoms had persisted, prompting her referral to our facility for further evaluation. The patient had reported a history of fever two weeks before admission, which had resolved on its own. She had denied any prior use of oral contraceptives or a history of trauma, hypertension, diabetes, cardiovascular disease, or tuberculosis. On initial evaluation, she had been fully conscious (Glasgow Coma Scale E4M6V5) with motor aphasia, moderate right-sided weakness (muscle strength scale 4), and hyperreflexia in the right lower limb. Babinski and other pathological reflexes had been absent. Laboratory investigations had revealed elevated D-dimer levels (4.96 ng/mL) and raised liver transaminases: Serum glutamic oxaloacetic transaminase (132 U/L) and serum glutamic pyruvic transaminase (162 U/L), while coagulation studies, including prothrombin time, activated partial thromboplastin time, and international normalized ratio, had been normal.

The axial magnetic resonance imaging images of the brain findings [Figure 1] in this case provided clear evidence of CVST involving the SSS. On the axial T1-weighted (T1W), T2-weighted (T2W), T2-fluid-attenuated inversion recovery (T2-FLAIR), and diffusion-weighted imaging (DWI) sequences [Figure 1a], hyperintense signals were present in both parietal regions, particularly near the midline, indicating areas of venous congestion or ischemia due to impaired venous outflow. These changes were consistent with the involvement of the SSS, one of the brain’s major venous structures. There was also a distinct hyperintense lesion in the right frontal lobe [Figure 1b], likely representing a subacute hemorrhage, which is a common complication of CVST resulting from elevated venous pressure and vessel rupture. Additional bilateral hyperintense signals visible on the FLAIR images further suggested ischemic or congestive changes related to CVST.

Close

Figure 1:

Axial MRI images of the brain: T1-weighted (T1W), T2-weighted (T2W), T2-FLAIR, and diffusion-weighted imaging (DWI) sequences in a patient with CVST. (a) The axial T1W, T2W, T2-FLAIR, and DWI sequences reveal hyperintense signals in the bilateral parietal regions (blue arrow), indicative of venous ischemia or infarction. These lesions are localized near the midline, suggesting involvement of the superior sagittal sinus, the most common site for CVST. The distribution and pattern of these hyperintensities are consistent with venous congestion or edema, resulting from impaired venous drainage due to thrombosis; (b) The axial T1W, T2W, T2-FLAIR, and DWI images also demonstrate a hyperintense region in the right frontal lobe (yellow arrow), likely representing a subacute hemorrhagic lesion secondary to CVST, characterized by crescent-shaped intracranial hemorrhage. In addition, the presence of bilateral hyperintense areas on the FLAIR sequence in the parietal regions further supports the diagnosis of venous congestion or ischemic changes, common complications associated with superior sagittal sinus thrombosis. MRI: Magnetic resonance imaging, CVST: Cerebral venous sinus thrombosis, FLAIR: Fluid-attenuated inversion recovery.

Export to PPT

The magnetic resonance venography (MRV) and magnetic resonance angiography (MRA) brain sequences [Figure 2] confirmed the diagnosis by showing a lack of normal blood flow in the SSS and cortical veins, indicative of thrombosis. These images together demonstrated the effects on the SSS and cortical veins, including venous obstruction, elevated ICP, and secondary ischemic and hemorrhagic brain injury. The combination of T1W, T2W, T2-FLAIR, DWI, MRV, and MRA imaging provided a comprehensive understanding of the underlying pathology in this patient, underscoring the critical role of advanced imaging techniques in diagnosing and managing CVST.

Close

Figure 2:

MRV and MRA brain sequences in a patient with CVST. (a1) The anterior-posterior view of the MRV demonstrates a segmental cutoff or occlusion in the superior sagittal sinus and cortical veins (yellow arrows), confirming the diagnosis of CVST; (a2) The lateral view of the MRV similarly shows a segmental cut-off or occlusion in the superior sagittal sinus and cortical veins (yellow arrow), further supporting the diagnosis of CVST; (a3) The posterior view of the MRV reveals a segmental cut-off or occlusion in the superior sagittal sinus and cortical veins (yellow arrows), confirming the presence of CVST; (b) The anterior cranial view of the MRA indicates contrast leakage (red arrow), confirming the presence of late subacute hemorrhage in the right frontal lobe accompanied by perifocal edema. This phenomenon is attributed to venous occlusion, which disrupts normal blood flow, leading to venous hypertension, edema, and ischemic changes in the affected cortical regions. MRI: Magnetic resonance imaging, CVST: Cerebral venous sinus thrombosis, MRA: Magnetic resonance angiography, MRV: Magnetic resonance venography.

Export to PPT

To further assess the patient’s condition, cerebral digital subtraction angiography [Figure 3] was performed, which revealed reduced blood flow to the SSS and bilateral cortical veins, particularly on the right side.

Close

Figure 3:

Cerebral digital subtraction angiography: Venous phase in a patient with CVST. (a1) Contrast injection from the right internal carotid artery (Rt-ICA) in the anterior-posterior view reveals a normal pattern of the cerebral venous sinus; (a2) The lateral view of contrast injection from the Rt-ICA also displays a normal pattern of the cerebral venous sinus; (a3) Contrast injection from the left internal carotid artery (LtICA) in the anterior-posterior view shows a normal pattern of the cerebral venous sinus; (a4) The lateral view of contrast injection from the Lt-ICA similarly demonstrates a normal pattern of the cerebral venous sinus; (b1) Contrast injection from the Rt-ICA in the anterior-posterior view indicates reduced flow to the superior sagittal sinus and bilateral cortical veins, particularly on the right side, raising the suspicion of thrombosis in both the superior sagittal sinus and cortical veins (red arrows); (b2) The lateral view of contrast injection from the Rt-ICA corroborates the finding of reduced flow to the superior sagittal sinus and bilateral cortical veins, especially on the right side, suggesting possible thrombosis (red arrows); (b3) Contrast injection from the Lt-ICA in the anterior-posterior view reveals diminished flow to the superior sagittal sinus and bilateral cortical veins, predominantly on the right side, further indicating potential thrombosis (red arrows); and (b4) The lateral view of contrast injection from the Lt-ICA also shows reduced flow to the superior sagittal sinus and bilateral cortical veins, particularly on the right side, reinforcing the possibility of thrombosis in these regions (red arrows). CVST: Cerebral venous sinus thrombosis.

Export to PPT

Based on the clinical, radiological, and angiographic findings, a diagnosis of CVST had been made. Treatment had included a subcutaneous injection of 2.5 mg of fondaparinux (anti-factor Xa) once daily, which was later transitioned to a direct anticoagulant on day 7. Additional supportive care had included analgesia with ketorolac 30 mg intravenously twice daily. The patient’s headache had fully resolved by day 9 of treatment.

DISCUSSION

This case of a 16-year-old female with CVST highlights key clinical features and the complexity of managing the condition. Headache was the initial and dominant symptom, which is consistent with studies reporting that headaches occur in 60–90% of CVST cases. The character of the headache, often throbbing or pressure-like, can progress gradually over days and is typically linked to increased ICP. In this case, the persistence of headaches despite adequate seizure management aligns with findings from studies in Pakistan, India, and Turkey, where headache is frequently reported as the most prevalent symptom among CVST patients. Globally, the incidence of CVST is significantly higher in females, with studies consistently demonstrating a marked predominance among women, particularly those of reproductive age, with an average age commonly reported as between 36 and 39 years. Headache continues to be the most commonly reported clinical manifestation. On a global scale, CVST has an incidence rate of 2.62 cases/100,000 individuals, with hormonal influences such as pregnancy and contraceptive use serving as significant risk factors. These findings strengthen the understanding that headache is the predominant clinical feature of CVST, especially among female patients.^[1,3,4] Seizures, a common symptom of CVST, were also observed in this patient. Seizures often arise in patients with supratentorial hemorrhagic lesions or cortical vein thrombosis and are particularly common in the acute phase of CVST. In this case, the patient’s imaging studies showed thrombosis in the SSS and cortical veins, supporting the pathophysiology linking seizures to this anatomical involvement. Early seizure activity is common, with reports showing early seizures in 44.3% of CVST patients and status epilepticus in up to 12.8%. The use of anticonvulsants, such as phenytoin in this case, is crucial for managing recurrent seizures.^[1,5] Aphasia, a focal neurological deficit, was another significant finding in this patient, emerging after the CVST diagnosis. Aphasia is often associated with thrombosis in the dominant hemisphere’s cortical veins, particularly in the left lateral sinus, as was the case in this patient. Literature suggests that aphasia occurs in 19–24% of CVST cases, and its presence in this patient further underscores the multifaceted neurological impact of CVST.^[1,6]

In addition, intracerebral hemorrhage (ICH) developed in this patient, a complication known to affect 30–40% of CVST cases. ICH in the setting of CVST is associated with a worse prognosis and is linked to increased venous pressure and compromised neurovascular integrity. The rupture of dilated veins due to CVST can result in hemorrhagic transformation, as occurred in this case, where imaging showed a right frontal hematoma. This complication raises concerns for long-term morbidity and mortality.^[4,7] Previous studies indicate that D-dimer serves as a sensitive diagnostic tool for excluding CVST in low-risk patients. Notably, a meta-analysis revealed a high negative predictive value for D-dimer levels in patients presenting with isolated headaches suspected of having CVST.^[8,9]

In our case, the initial D-dimer level of 4.96 ng/mL, followed by a decrease to 2.78 ng/mL after treatment, underscores the utility of this biomarker not only in confirming the diagnosis but also in monitoring the effectiveness of anticoagulation therapy in a patient without traditional risk factors for CVST. This highlights the importance of D-dimer testing in assessing patients with unexplained headache symptoms, reinforcing its role in clinical decision-making.^[10,11]

The management of CVST in this patient followed established guidelines. Anticoagulation therapy is a mainstay of CVST treatment, even in the presence of ICH. In this case, fondaparinux, a factor Xa inhibitor, was administered subcutaneously, followed by oral anticoagulation. According to the European Stroke Organization and American Heart Association/American Stroke Association guidelines, anticoagulation should be continued for 3–6 months in provoked cases and 6–12 months in unprovoked cases. Warfarin was chosen for long-term management in this patient, as direct oral anticoagulants and factor Xa inhibitors are not the first-line choice for CVT according to current guidelines.^[1,10,12] Table 1 summarizes several cases of CVST based on their clinical presentations, imaging findings, therapy, and outcomes.

CONCLUSION

This case underscores the critical elements involved in the diagnosis and management of CVST, emphasizing the interplay between clinical evaluation, imaging diagnostics, and therapeutic interventions. The patient presented with hallmark features of CVST, including persistent headache, seizures, and focal neurological deficits, which are well-documented manifestations of this condition. The occurrence of intracerebral hemorrhage, while complicating the clinical scenario, did not preclude the initiation of anticoagulation therapy, which remains the foundation of CVST treatment. The integration of anticoagulation with adjunctive measures for symptomatic management, such as analgesics for headache and antiepileptics for seizure control, resulted in significant clinical improvement, highlighting the efficacy of current evidence-based management strategies. This case emphasizes the necessity of prompt recognition, the use of advanced imaging modalities for accurate diagnosis, and strict adherence to established treatment protocols to achieve optimal clinical outcomes in patients with CVST.