Papilledema

A patient with papilledema presents with symptoms of increased intracranial pressure like headache and brief transient obscuration of vision. The headache is characteristic of increased intracranial pressure, worse on awakening and exacerbated by coughing. There can also be nausea and vomiting if the intracranial pressure is very high. A pulsatile tinnitus is sometimes present [6].

Visual acuity is usually well preserved. Some patients may have monocular or binocular visual blackouts that last for a few seconds, especially when rising from a recumbent position to a sitting or standing position. There may be transient flickering of lights, dyschromatopsia and constriction of the visual field. Relative afferent pupillary defect is usually absent. Fundus examination shows bilateral signs of optic disc edema like blurring of the optic disc margins, filling in of the optic disc cup, edema of the nerve fiber layer, retinal or choroidal folds, venous congestion, peripapillary hemorrhages, cotton-wool spots, hyperemia of the optic nerve head and hard exudates of the optic disc [7].

Apart from a detailed fundus examination, fundus fluorescein angiography may help in the diagnosis of papilledema. B-scan ultrasonography of the eye may be helpful in ruling out buried disc drusen. Visual fields should be tested, especially in chronic cases.

As soon as papilledema is diagnosed, urgent neuroimaging is required. Computed tomography (CT) scan and magnetic resonance imaging (MRI) of the brain with contrast are required to detect an intracranial mass lesion. At times, magnetic resonance venography may be required to detect venous sinus thrombosis. If MRI fails to show a lesion, a lumbar puncture should be performed to assess the opening pressure of the cerebrospinal fluid and to obtain fluid sample for histopathological and microbiological analysis [8].

Blood tests have limited value except when required to rule out infectious, inflammatory or metabolic causes. In such cases, complete blood count, blood sugar, erythrocyte sedimentation rate, and syphilis serology may be done.

The treatment of papilledema associated with visual loss depends largely on the cause, symptoms, signs, and progression of the underlying condition. Medical treatment usually consists of diuretics, especially carbonic anhydrase inhibitors. Weight reduction in obese patients with idiopathic intracranial hypertension is recommended. Serial lumbar punctures may also be effective in lowering the intracranial pressure. Corticosteroids may be useful in sarcoidosis. If medical treatment is not sufficient, optic nerve sheath decompression or a ventriculo- or lumboperitoneal shunt may need to be carried out [9] [10].

The prognosis for papilledema depends on the cause. Patients with metastatic brain tumors have the worst prognosis. Individuals suffering from ventricular obstructive disease may have a better prognosis with successful shunting. Patients with idiopathic intracranial hypertension usually have the best outcomes. The diagnosis of papilledema requires a prompt workup with neurological, neurosurgical, or neuroradiologic consultations. Long-standing papilledema leads to irreversible visual loss in the form of secondary optic atrophy, constriction of visual fields and poor color vision [5].

Common causes of increased intracranial pressure leading to papilledema are [2]:

• Intracranial tumors or space-occupying lesions 
• Idiopathic intracranial hypertension (Pseudotumor cerebri)
• Decreased cerebrospinal fluid drainage (eg. venous sinus thrombosis, inflammatory processes, meningitis, subarachnoid hemorrhage)
• Increased production of cerebrospinal fluid due to tumors
• Cerebral edema/encephalitis
• Sarcoidosis
• Obstruction of the ventricular system may lead to a very rapid rise in intracranial pressure and fulminant papilledema
• Craniosynostosis
• Drugs like tetracycline, minocycline, lithium, nalidixic acid, and corticosteroids (more commonly from withdrawal than from use)

Papilledema affects both sexes equally and can present at any age. During infancy elevated intracranial pressure may not lead to papilledema because the fontanels are not closed. Among young adults, papilledema is more likely to be caused by idiopathic intracranial hypertension than by a tumor. Idiopathic intracranial hypertension is often seen in obese, young women [3]. Intracranial masses most commonly cause papilledema in adults.

The subarachnoid space of the brain is continuous with the optic nerve sheath. Therefor conditions increasing the intracranial pressure can lead to compression of optic nerve leading to papilledema. Usually a combination of mechanical and vascular factors results in edema, ischemia, and eventual visual impairment or loss [4].

Sometimes a patient may be asymptomatic and papilledema may be detected incidentally. However, on detection of papilledema, urgent neurological workup is important to save vision and/or life. Patients with known etiological factors should have regular fundus examination or relevant neurological follow up. For chronic conditions, follow up can prevent or detect in time any future occurrences of papilledema.

Papilledema is the swelling of the optic nerve head in the absence of any other optic nerve pathology, due to elevated intracranial pressure usually from causes like intracranial tumors and idiopathic intracranial hypertension.

The vision in papilledema is usually well preserved till late in the course of the disease, unlike in the other causes of optic nerve head swelling. The signs and symptoms are typical for increased intracranial tension such as nausea, vomiting, headache, transient obscuration of vision and diplopia. The treatment is directed at management of the underlying cause of elevated intracranial pressure. Diuretics may be given for decreasing the intracranial pressure. Early treatment and resolution of papilledema leads to complete visual recovery. Long-standing or severe papilledema may result in bilateral optic nerve dysfunction and secondary optic atrophy [1].

• Definition: Papilledema is a swelling of the optic nerve, at the point where it enters the retina in the back of the eye. The optic nerve carries visual signals from the eye to the brain.
• Cause: It is caused by an increase in the pressure of the fluid within the skull, with no infection or inflammation of the optic nerve itself. The common causes of rise in the intracranial pressure are tumors of the brain, spine or skull or any structure inside the skull, infection of the brain or meninges, hemorrhage, trauma, an abnormal closure of the bones of the skull, or an accumulation of cerebrospinal fluid within the skull, known as hydrocephalus.
• Symptoms: Headache, that is worse on waking up and is exacerbated by coughing. Nausea and vomiting may be there. There may be temporary and transient blurring of vision, poor color perception, flickering sensation, or double vision. 
• Diagnosis: The eye doctor will examine your retina and optic nerve head with an ophthalmoscope. Angiography of the eye with a dye and ultrasound of the eye may be required at times. Computerized tomography and magnetic resonance imaging of brain is commonly used to detect the underlying disease. A testing of the spinal fluid may be required. 
• Treatment and follow up: Treatment is directed at treating the cause, eg. a brain tumor. Diuretic drugs are given to decrease the intracranial pressure. Corticosteroids may be given if there is an inflammation in the brain like with sarcoidosis. With complete treatment and resolution of papilledema, no permanent visual loss occurs. Even after the underlying disease is managed, it is important to continue consultations with the eye doctor until the papilledema goes away. Chronic papilledema may lead to blindness. 

1. Ehlers JP, Shah CP, eds. Papilledema. In: The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 5th ed. Baltimore, Md: Lippincott Williams & Wilkins; 2008:252-254.
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3. Friedman DI. Papilledema and pseudotumor cerebri. Ophthalmol Clin North Am 2001; 14:129.
4. Sinclair AJ, Burdon MA, Nightingale PG, Matthews TD, Jacks A, Lawden M, et al. Rating papilloedema: an evaluation of the Frisén classification in idiopathic intracranial hypertension. J Neurol. Jan 12 2012.
5. Corbett J.J., Thompson H.S. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989; 46:1049-1051.
6. Scott CJ, Kardon RH, Lee AG, Frisén L, Wall M. Diagnosis and grading of papilledema in patients with raised intracranial pressure using optical coherence tomography vs clinical expert assessment using a clinical staging scale. Arch Ophthalmol. Jun 2010; 128(6):705-11.
7. Yanoff M, Duker JS. Ophthalmology. 1999:11.5.1-5.4.
8. Hayreh SS, Hayreh MS. Optic disc edema in raised intracranial pressure. II. Early detection with fluorescein fundus angiography and stereoscopic color photography. Arch Ophthalmol 1977; 95(7):1245–54.
9. Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuro-Ophthalmol 2004; 24(2):138–45.
10. Rubin RC, Henderson ES, Ommaya AK, Walker MD, Rall DP. The production of cerebrospinal fluid in man and its modification by acetazolamide. J Neurosurg 1966; 25(4):430–6.

• Hemorrhages from papilledema
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