Acute bilateral vision loss secondary to cerebral venous thrombosis–induced intracranial hypertension: a case report

Abstract

Background

The present case describes a rare presentation of sudden onset acute bilateral vision loss due to cerebral venous thrombosis secondary to malignant rise in intracranial pressure.

Case presentation

A 23-year-old male patient presented with sudden onset bilateral loss of vision of one week duration. Best-corrected visual acuity was 6/18, N12, and perception of light in the right and left eye, respectively. Fundus examination revealed papilledema. Magnetic resonance imaging showed thrombotic occlusion of the superior sagittal, straight, and inferior sagittal sinuses. Cerebrospinal fluid opening pressure was elevated to 40 cm H₂O. Laboratory investigations showed the presence of polycythemia (Hemoglobin- 18.6 g/dl) and elevated serum homocysteine (38.64 µmol/L) levels. Early initiation of appropriate treatment resulted in improvement of vision to 6/9, N8 and 6/6, N6 in the right and left eye, respectively. One month following treatment, there was complete resolution of papilledema and retinal haemorrhages in both eyes.

Conclusion

Malignant rise in intracranial pressure can rarely lead to the sudden onset bilateral vision loss. Early diagnosis and prompt treatment can be associated with a good visual prognosis.

Background

Increased intracranial pressure (ICP) results from the elevation of the pressure within the cranium [1]. Excessive cerebrospinal fluid production or obstruction of its outflow leads to raised ICP [1]. It can be life-threatening and requires immediate medical attention. Most cases are due to idiopathic intracranial hypertension, space-occupying lesions, cerebral venous thrombosis (CVT), infections, cerebrovascular accidents, aneurysms, and systemic medications [1]. CVT is an uncommon type of stroke with an incidence of 3–4 cases per million adults [2]. It is associated with raised intracranial tension presenting as a new-onset headache in 70 to 90% of cases, and less frequently with papilledema and abducens nerve palsy [2]. Visual acuity and colour vision are usually preserved in the early stages of papilledema [3]. Chronic papilledema can result in optic atrophy with progressive vision loss. We report a rare case of CVT where a malignant rise in ICP led to the acute onset of bilateral vision loss.

Case report

A 23-year-old male was referred to the Ophthalmology clinic complaining of a sudden onset of bilateral blurring of vision of one week’s duration. Two days prior to the Ophthalmology referral, the patient had been hospitalized elsewhere with complaints of mild fever, severe headache, blurring of vision, pain, and numbness of both upper limbs. The patient had no systemic ailments, was non-smoker, non-alcoholic, and was not on any medications prior to this. There was no positive family history. In the recent hospitalization elsewhere, he had been treated conservatively with systemic Azithromycin, Doxycycline and intravenous fluids. A systemic diagnosis was still pending at the time of the Ophthalmology referral. On examination, the best-corrected visual acuity was 6/18, N12 in the right eye, and only light perception with accurate projection of rays in the left eye. Extraocular movements were full and free bilaterally. Pupils were circular and reacting sluggishly to light on the right side. A relative afferent pupillary defect was noted in the left eye. Anterior segment examination was unremarkable in both eyes. The patient could identify 15/17 plates of Ishihara’s colour vision chart in the right eye. Dilated fundoscopy revealed marked disc oedema, dilated retinal veins, and extensive retinal haemorrhages in all quadrants bilaterally (Fig. 1a and b). Optical coherence tomography showed a normal foveal contour. An initial diagnosis of bilateral papilledema of unknown etiology was made, and the patient was referred to the Neurology department for further investigations.

Fig. 1.

a, b Color fundus photograph of right and left eyes at presentation showing massive disc edema, venous dilation, and extensive retinal hemorrhages. c Magnetic resonance imaging with contrast showing thrombotic occlusion of the superior sagittal (white arrow), straight, and inferior sagittal sinuses

Magnetic resonance imaging revealed thrombotic occlusion of the superior sagittal, straight, and inferior sagittal sinuses (Fig. 1c). A therapeutic and diagnostic Cerebrospinal fluid tap (20 ml) was done. Cerebrospinal fluid opening pressure was elevated to 40 cm H₂O (normal: 10 to 20 cm H₂O). Cerebrospinal fluid protein, glucose, and cytology were within normal range (Table 1). Complete blood counts revealed polycythemia with neutrophilic leucocytosis and erythrocytosis [Hemoglobin- 18.6 g/dl, red blood cell count- 6.38 × 106/µl, and Total leukocyte count- 15.79 × 103/µl]. The sickling test was negative. Serum homocysteine was elevated to 38.64 µmol/L. Urine microscopy showed abundant pus cells (648.7 per high-power field), with E. coli isolated on culture. A bone marrow biopsy was planned by the department of haematology to confirm the diagnosis of polycythemia vera and evaluate the bone marrow cellularity. The bone marrow biopsy showed cellular marrow with erythroid predominance and nonspecific reactive changes. Serum erythropoietin was 4.25 mIU/ml (2.6–18.5 mIU/ml). Considering the young age, the patient was advised to undergo the thrombophilia panel tests. However, the patient could not afford the tests due to his poor socioeconomic status. Blood pressure was normal. The patient was diagnosed with CVT with a fulminant rise in intracranial pressure, leading to bilateral vision loss. The CVT was possibly due to polycythemia and raised homocysteine. The patient was managed conservatively with intravenous mannitol (100 ml four times/day), Enoxaparin sodium (60 mg twice/day), Levetiracetam (500 mg twice/day), oral Acetazolamide (250 mg thrice/day), oral folic acid, and other supportive measures. A phlebotomy with the removal of 350 ml of blood was performed to treat polycythemia. The patient was discharged from the hospital after 15 days in a hemodynamically stable condition.

Table 1.

Cerebrospinal fluid analysis at the time of presentation

Variable	Actual value	Normal
Appearance	Clear	Clear
Opening pressure (cm H2O)	40	10–20
Protein (mg/dl)	38.8	15–45
Glucose (mg/dl)	63	40–70
Lymphomononuclear cells	90%
Polymorphs	10%
Atypical cells	Absent

One month following the initial presentation, best-corrected visual acuity had improved to 6/9, N8, and 6/6, N6 in the right and left eyes, respectively. The patient could correctly identify 15/17 colour vision plates on the right and 17/17 on the left. Pupils were sluggishly reacting bilaterally. A dilated fundus examination showed a pale optic disc with complete resolution of disc oedema and retinal haemorrhages in both eyes (Fig. 2a and b). The patient has been asked for regular follow-up examinations.

Fig. 2.

a, b Color fundus photographs of right and left eyes, one month following presentation, pale optic disc, resolved disc edema, and retinal hemorrhages

Discussion

The cerebral venous system is an uncommon site for thrombosis, with a particularly high incidence in young adults [4]. The annual incidence in adult patients is 2 to 5 cases per million individuals. The mean age of patients affected by CVT is 35 years, and it is more common in women than men due to gender specific risk factors [4]. The most common presenting symptoms following CVT include headache (88.8%), seizures (39.3%), paresis (37.2%), papilledema (28.3%), and mental status changes (22%) [5]. Vision loss is a rare complication associated with CVT. Vision loss has been reported in around 13% of cases of CVT, with severe vision loss in 1.4% of patients [6]. The various mechanisms of vision loss in CVT include dysfunction of the optic nerve secondary to raised ICP, venous/arterial infarcts in the occipital lobe, and raised ICP on the optic nerve following the development of a secondary dural arterio-venous fistula [7]. Sub-acute or chronic occlusion of the dural venous sinuses in CVT can lead to raised ICP, which in turn causes optic nerve dysfunction. Multiple venous sinuses involved in our case may have caused a higher rise in cerebrospinal fluid pressure, leading to a significantly greater amount of optic nerve dysfunction. This was responsible for the acute vision loss in our case. Risk factors associated with CVT include a hypercoagulable status, oral contraceptive use, autoimmune diseases, hormone replacement therapy, malignancy, pregnancy, infections, traumatic head injury, and arteriovenous malformations [8]. Our case had multiple risk factors in the form of raised serum homocysteine and polycythemia. Both these conditions can cause thrombosis. Raised homocysteine levels cause thrombosis by endothelial injury, promotion of smooth muscle proliferation, activation of Factor V, increased platelet adhesion, and decreased production of nitric oxide and prostacyclin [9]. The presence of numerous risk factors might have resulted in greater severity of the CVT, resulting in acute vision loss.

This case highlights the rapid loss of visual function in a patient with raised ICP secondary to CVT. Malignant idiopathic intracranial hypertension has been defined as a constellation of acute onset of symptoms of elevated ICP, rapid worsening of vision loss within 4 weeks from the onset of symptoms, and rapid worsening of vision over a few days [10]. Patients with raised intracranial tension are usually associated with good visual acuity initially [3]. Acute onset bilateral loss of vision with bilateral disc edema is more common in the setting of optic neuritis, malignant hypertension, and anterior ischemic optic neuropathies. Raised cerebrospinal fluid opening pressure and radiological findings of CVT clinched the diagnosis in favour of a malignant rise in intracranial tension secondary to CVT.

Aggressive medical and surgical treatment is essential in the presence of vision-threatening disease. Thambisetty et al. [10] used high doses of acetazolamide and repeated lumbar punctures to bring down the ICP. Both unfractionated heparin and low molecular weight heparin are routinely used to manage CVT [11]. This patient responded well to medical treatment in the form of high doses of acetazolamide and systemic anticoagulation. At the same time, the presence of systemic risk factors associated with thrombosis was dealt with. Previous studies have highlighted the role of surgical interventions in bringing down the intracranial tension [12]. However, our patient responded well to medical management, and surgical treatment was not required. Sutraye et al. [3] have reported a case of rapidly progressive vision loss in 44-year-old obese woman due to fulminant idiopathic intracranial hypertension. The cerebrospinal fluid opening pressure was raised to 42 cm of H₂O. Management with medical therapy and urgent thecoperiteoneal shunt improved visual function in both eyes. The presence of optic disc pallor in spite of good visual recovery is indicative of residual mild optic nerve damage. This highlights the importance of early diagnosis and treatment initiation in such cases to avoid severe optic nerve damage.

Conclusion

CVT can present with an acute onset of bilateral vision loss. This is potentially reversible with prompt diagnosis and intervention. Prompt neuroimaging was pivotal in diagnosing CVT and guiding effective intervention. Ophthalmologists must be aware of such an uncommon presentation in patients with papilledema secondary to CVT. Multidisciplinary approach will be required for successful management in all such cases.

Abbreviations

ICP

Intracranial pressure

CVT

Cerebral venous thrombosis

Authors’ contributions

Dr PKP: Examined and interpreted the data, manuscript writing, and manuscript handling. Dr SKS: Examined and interpreted the data, manuscript writing, and manuscript handling. Dr KAG: Data collection, manuscript writing. DR SM: Data collection, manuscript writing. All authors read and approved the manuscript.

Funding

Open access funding provided by Siksha 'O' Anusandhan (Deemed To Be University). Nil.

Data availability

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

Declarations

Ethics approval and consent to participate

Waived.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report.

Competing interests

The authors declare no competing interests.