Bilateral papilledema: A case of cerebral venous sinus thrombosis.

Introduction

The diagnosis of cerebral venous thrombosis (CVT) remains challenging due to a wide range of clinical manifestations. Headache is the most common presenting symptom and there can be other neurologic signs and symptoms,[1] like focal seizures with or without secondary generalization, unilateral or bilateral paresis, impaired consciousness or visual disturbance. Papilledema is present in about 28% of patients with CVT.[2]

Case Report

A 29-year-old male patient presented with severe persistent headache of 1-2 months duration which was acute in onset and was not relieved by any oral analgesic. There was no history of visual disturbance, diplopia, vomiting, seizures, trauma, or head injury. Patient had neither medical history of blood disorders nor was on any medications.

Distant and near visual acuity of the patient was 20/20 and N6 respectively in both eyes.

Pupils were bilaterally central and reacting to light with no evidence of relative afferent pupillary defect. Anterior segment examination was within normal limits.

Following are the colored and red-free fundus photographs [Figures 1 and 2].

Figure 1

OD (right eye) color and red-free fundus photographs show disc elevation, hyperemia with blurring of margins, obscuration of blood vessels, and Paton's lines suggestive of disc edema

Figure 2

OS (left eye) color and red-free fundus photographs show disc elevation, hyperemia with blurring of margins, obscuration of blood vessels, and Paton's lines suggestive of disc edema

B-scan was performed which ruled out optic nerve head drusen.

Fundus fluorescein angiography showed leakage from disc in early phase which persisted through late phase depicting bilateral papilledema [Figure 3].

Figure 3

Fundus fluorescein angiography shows leakage in early phase which persists through late phase suggestive of disc edema

A plain computed tomography (CT) scan brain was within normal limits.

Magnetic resonance (MR) venography was performed with the following findings:

Partial thrombus involving cranial part of right sigmoid sinus, transverse sinus, sinus confluence, superior sagittal sinus, and terminal part of left transverse sinus [Figure 4].

Figure 4

Magnetic resonance venography showing partial thrombus involving cranial part of right sigmoid sinus, right transverse sinus, sinus confluence, superior sagittal sinus, and terminal part of left transverse sinus. (Red arrows)

Hematological investigations revealed haemoglobin at 18.4 g/dL (13-17) and red blood cell (RBC) count at 6.04 million (4.5-5.5). Rest of histogram, prothrombin time, activated partial thromboplastin time, vitamin B12, serum total calcium, protein, thyroid stimulating hormone, VDRL, human immunodeficiency virus which were within normal limits. Hematology consultation was obtained which ruled out polycythemia as the patient had only raised RBC and hemoglobin with a normal erythrocyte sedimentation rate, platelets, and total counts without splenomegaly.

Diagnosis of CVT was kept and a neurology consultation was obtained. Patient was started on treatment as per guidelines from the American Stroke Association.[3] A trial of aspirin was given first and LMW heparin therapy was kept in plan if the symptoms did not improve. After 2 days, patient was symptomatically better with relief from headache.

On subsequent follow-up visits, the visual acuity remained 20/20 with normal color vision.

Fundus examination repeated 1 week later and 1 month later showed resolving papilledema [Figure 5].

Figure 5

OD (right eye) and OS (left eye) fundus photographs a month after starting anticoagulants show resolving papilledema with complete visualization of disc margins and surrounding vasculature

Discussion

Thrombosis most commonly affects the superior sagittal sinuses (SSS) and lateral sinuses[4] due to its high position, low pressure, and slow flow. Since CT scan is nonspecific, MR venography remains the diagnostic modality of choice and must be performed in such patients.[5]

Bilateral hemorrhagic infarction of the cortex and adjacent white matter can be fatal consequence of SSS thrombosis. Early intervention is life-saving in these patients. Thus, suspecting CVT and its early diagnosis and management plays an important role in preserving the visual function as well as neurological integrity of the patient.