Papilloedema: Causes, Symptoms and Treatment
Papilloedema is a swelling of the optic nerve head caused by raised intracranial pressure. It is a serious medical emergency requiring prompt evaluation.
Things worth knowing about "Papilloedema"
Papilloedema is a swelling of the optic nerve head caused by raised intracranial pressure. It is a serious medical emergency requiring prompt evaluation.
What Is Papilloedema?
Papilloedema refers to swelling of the optic disc – the point where the optic nerve enters the eye and becomes visible on the retina. This swelling is typically caused by raised intracranial pressure (ICP) and usually affects both eyes simultaneously. Papilloedema is a serious neurological warning sign and always requires urgent medical investigation to identify and treat the underlying cause.
Causes
Papilloedema almost always results from elevated pressure within the skull. Common causes include:
- Brain tumours (benign or malignant) obstructing cerebrospinal fluid (CSF) flow
- Hydrocephalus (accumulation of CSF within the brain)
- Meningitis or encephalitis (infection or inflammation of brain coverings)
- Intracranial haemorrhage (e.g., subdural or subarachnoid bleeding)
- Idiopathic intracranial hypertension (pseudotumour cerebri) – most common in overweight women of childbearing age
- Cerebral venous sinus thrombosis (clots in the brain's venous sinuses)
- Hypertensive crisis with severely elevated blood pressure
Symptoms
In early stages, papilloedema may cause few or no visual symptoms. As pressure increases, the following may occur:
- Brief, transient visual obscurations lasting seconds (greying or blackening of vision)
- Headaches, often worse in the morning or when bending forward
- Nausea and vomiting
- Double vision (diplopia), often due to sixth cranial nerve palsy
- Enlargement of the blind spot on visual field testing
- In advanced stages: permanent visual field loss or even blindness
Diagnosis
Diagnosis is primarily made through an ophthalmological examination:
- Fundoscopy (ophthalmoscopy): Direct visualisation of the optic disc; papilloedema appears as blurred disc margins, disc elevation, and peripapillary haemorrhages
- Optical coherence tomography (OCT): Precise measurement of the retinal nerve fibre layer thickness
- Visual field testing (perimetry): Detection of visual field defects
- Neuroimaging: MRI or CT scan of the brain to identify causes such as tumours, bleeds, or hydrocephalus
- Lumbar puncture: Measurement of CSF opening pressure and analysis of spinal fluid
- Blood pressure measurement: Exclusion of hypertensive crisis
Treatment
Treatment is directed at the underlying cause of raised intracranial pressure:
- Brain tumour: Surgery, radiotherapy, or chemotherapy depending on tumour type and location
- Hydrocephalus: CSF shunting procedures
- Meningitis or encephalitis: Antibiotics or antiviral therapy
- Idiopathic intracranial hypertension: Weight loss, acetazolamide (to reduce CSF production), therapeutic lumbar puncture, or optic nerve sheath fenestration in severe cases
- Hypertensive crisis: Emergency blood pressure reduction
- Cerebral venous sinus thrombosis: Anticoagulation therapy
Early treatment is essential to prevent permanent damage to the optic nerve and irreversible vision loss.
Distinction From Pseudopapilloedema
Not every elevated or unusual-looking optic disc indicates true papilloedema. Pseudopapilloedema – caused by optic disc drusen or high hyperopia – can mimic the appearance of papilloedema without raised intracranial pressure. Differentiation is achieved through OCT, fluorescein angiography, and neuroimaging.
References
- Hayreh, S. S. (2009): Optic disc edema in raised intracranial pressure. In: Survey of Ophthalmology, 54(6), 654–678.
- Wall, M. et al. (2014): The idiopathic intracranial hypertension treatment trial: Clinical profile at baseline. In: JAMA Neurology, 71(6), 693–701.
- Mollan, S. P. et al. (2018): Idiopathic intracranial hypertension: Consensus guidelines on management. In: Journal of Neurology, Neurosurgery & Psychiatry, 89(10), 1088–1100.
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