Sir,

Papilloedema is often the presenting feature of serious intracranial pathology. Commoner causes include space occupying lesions and benign intracranial hypertension. Impaired cerebral venous drainage is also known to increase intracranial pressure and result in papilloedema. Jugular vein thrombosis is uncommon but may rarely cause papilloedema. We present the case of such a patient.

Case report

A 77-year-old man was seen in the ophthalmic clinic with a 6-month history of headache and a 1-month history of deteriorating vision especially in the left eye.

Past medical history included hypertension and previous myringotomy and T-tube insertion for a right middle ear effusion.

Visual acuity was 6/36 left and 6/12 right. There was no afferent pupillary defect and the ocular media were clear. Fundoscopy revealed bilateral optic disc swelling. He underwent an urgent magnetic resonance imaging (MRI) scan; however, no significant pathology was evident. A neurological opinion was sought and the patient underwent a lumbar puncture. The CSF opening pressure was elevated at 28 cm H2O. Brain imaging did not reveal intracerebral venous thrombosis; therefore formal angiography was carried out. This showed slow flow within the venous sinuses and was discovered to be due to a complete occlusion of the right jugular vein and severe narrowing of the left jugular vein (Figure 1). Computerised tomography (CT) scanning of the chest did not show any compressive intrathoracic lesion.

Figure 1
figure 1

Left jugular venogram showing a narrowing at the jugular bulb.

A full range of blood test were performed including blood count, urea and electrolytes, inflammatory markers, tumour markers, immunological screen, and a thrombophilia screen. These investigations revealed no abnormality apart from a mild anaemia with haemoglobin of 12.9 g/dl and a normal mean corpuscular volume.

The patient was initially heparinised for 6 days to see if that would improve the cerebral venous blood flow, especially if the thromboses were fresh. However, upon repeating the lumbar puncture the cerebro-spinal fluid (CSF) pressure remained elevated at 33 cm H2O. A neurosurgical opinion was sought and the patient underwent a lumbo-peritoneal shunt procedure. On discharge his visual acuity had improved to 6/9 right and 6/12 left. He has been reviewed in the ophthalmic clinic over the past 6 months and his condition is stable, although he has lost visual field in both eyes due to optic nerve damage secondary to papilloedema. We have still not found an underlying cause for the bilateral jugular vein thrombosis.

Comment

Spontaneous internal jugular vein thrombosis is a rare vascular disorder. In the pre-antibiotic era this condition was a well-known complication of head and neck infection.1 The leading cause at present is trauma; this can include catheterisation of the internal jugular vein,2 intravenous drug abuse3 or head and neck surgery.4 Other causes include malignancy,5 thrombophilic6 states and use of the oral contraceptive pill.7

Papilloedema secondary to jugular vein occlusion is rare, and to our knowledge has only been described in two previous cases. One patient developed papilloedema due to a skull base metastasis compressing the left jugular vein.8 The second had complex intra-cranial vascular malformations which resulted in bilateral jugular vein occlusion and left optic atrophy suspected to be due to papilloedema.9 Our patient had long standing symptoms and interestingly presented with a reduction in visual acuity due to optic nerve dysfunction. Although his acuity improved once the intracranial hypertension was controlled, he did develop permanent optic nerve dysfunction as evidenced by visual field testing. All investigations in our patient were negative and we are unable to explain why he developed spontaneous bilateral jugular vein thrombosis. This condition is rare, but should be considered in patients with papilloedema and a negative MRI scan.