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Papilledema

Choked Disk

Papilledema is used to denote a swelling of the optic nerve head (disc) resulting from raised intracranial pressure. There is a lack of inflammation, infiltration or infection of the optic nerve itself. Any edema of optic nerve head arising from causes other than increased intracranial pressure is termed optic disc edema, and not papilledema.


Presentation

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].

Nausea
  • The signs and symptoms are typical for increased intracranial tension such as nausea, vomiting, headache, transient obscuration of vision and diplopia.[symptoma.com]
  • After renal transplantation, the patient had complete resolution of headaches, nausea and the papilledema.[ncbi.nlm.nih.gov]
  • Symptoms include headache, nausea and vomiting, ringing or other noises in the ears, and vision problems including temporary vision blackouts or “gray-outs” that last up to 30 seconds and affect one or both eyes or flashing lights.[neuroeyeorbit.com]
  • When you have a headache or unexplained nausea and vomiting, your doctor will look into your eye with an ophthalmoscope. This handheld instrument shines a bright light into your eye.[health.harvard.edu]
Prognathism
  • Abstract Crouzon syndrome is an autosomal dominant disorder characterized by cranial synostosis, hypertelorism, orbital proptosis, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism, without extremity involvement[ncbi.nlm.nih.gov]
Enlarged Blind Spot
  • Goldmann visual field revealed an enlarged blind spot in the right eye only ( Figure 2 ).[omicsonline.org]
  • Visual field testing may detect an enlarged blind spot. Later, visual field testing may show defects typical of nerve fiber bundle defects and loss of peripheral vision.[merckmanuals.com]
  • The visual hallmarks are relatively spared visual acuity in the setting of bilateral optic disc edema with enlarged blind spots, nasal visual field loss, or constriction of the visual fields.[medlink.com]
Blurred Vision
  • Some people with optic nerve compression experience blurred vision or loss of vision from one or both eyes.[verywellhealth.com]
  • Presentation Pearls Diagnosis Treatment Media Patient will present as a 57-year-old male with a history of hypertension who complains of an acute onset of intermittent headaches and blurred vision of the right eye.[smartypance.com]
  • Fleeting vision changes—blurred vision, double vision, flickering, or complete loss of vision—typically lasting seconds are characteristic of papilledema. Other symptoms may be caused by the elevated pressure in the brain.[msdmanuals.com]
  • Three weeks later, partial sinus recanalization, but she developed tinnitus, blurred vision and diplopia, with new onset severe papilledema, constrictive field deficits, partial left abducens nerve palsy, but normal visual acuity.[n.neurology.org]
Central Scotoma
  • One week after admission, his visual acuity remained 20/40 bilaterally with resolution of his partial central scotoma while disc edema improved 1.5-2 OD, 1.5 OS.[surgicalneurologyint.com]
  • In the left eye, there was a dense central scotoma and severe generalized depression. Findings from fundus examination showed bilateral disc edema, mild on the right and marked on the left, with peripapillary hemorrhages in both eyes.[jamanetwork.com]
  • scotoma should raise a red flag that this is not papilledema.[e-tjo.org]
Beak Nose
  • Abstract Crouzon syndrome is an autosomal dominant disorder characterized by cranial synostosis, hypertelorism, orbital proptosis, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism, without extremity involvement[ncbi.nlm.nih.gov]
Beaked Nose
  • Abstract Crouzon syndrome is an autosomal dominant disorder characterized by cranial synostosis, hypertelorism, orbital proptosis, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism, without extremity involvement[ncbi.nlm.nih.gov]
Hypertelorism
  • Abstract Crouzon syndrome is an autosomal dominant disorder characterized by cranial synostosis, hypertelorism, orbital proptosis, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism, without extremity involvement[ncbi.nlm.nih.gov]
Mandibular Prognathism
  • Abstract Crouzon syndrome is an autosomal dominant disorder characterized by cranial synostosis, hypertelorism, orbital proptosis, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism, without extremity involvement[ncbi.nlm.nih.gov]
Distractibility
  • She underwent a frontofacial monobloc distraction advancement which successfully corrected her papilledema and obstructive sleep apnoea.Pycnodysostosis is caused by a loss of function mutation in the CTSK gene that codes for the lysosomal cysteine protease[ncbi.nlm.nih.gov]
Papilledema
  • KEYWORDS: Mild papilledema; Optic nerve; Papilledema; Severe papilledema; Support vector machine[ncbi.nlm.nih.gov]
  • Chronic papilledema may lead to blindness.[symptoma.com]
  • Sex Papilledema affects both sexes equally. Age Papilledema can present at any age, though, during infancy, before the fontanelles close, the finding of papilledema may fail to occur despite elevated intracranial pressure.[emedicine.medscape.com]
Headache
  • Abstract Headaches associated with papilledema may be both life-threatening as well as vision-threatening.[ncbi.nlm.nih.gov]
Altered Mental Status
  • In 2 patients with new headache and altered mental status, symptoms and ventriculomegaly were dismissed as long-standing and not reflective of current ICP elevation.[ncbi.nlm.nih.gov]
Sciatica
  • After three months the patient started suffering, especially at night, of right-leg-sciatica. A lumbar MRI showed a neurinoma of the cauda equina. The patient was operated and after surgery the papilledema slowly shrank.[ncbi.nlm.nih.gov]
Polyneuropathy
  • It has features that may overlap with osteosclerotic myeloma or POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome.[ncbi.nlm.nih.gov]

Workup

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.

Central Scotoma
  • One week after admission, his visual acuity remained 20/40 bilaterally with resolution of his partial central scotoma while disc edema improved 1.5-2 OD, 1.5 OS.[surgicalneurologyint.com]
  • In the left eye, there was a dense central scotoma and severe generalized depression. Findings from fundus examination showed bilateral disc edema, mild on the right and marked on the left, with peripapillary hemorrhages in both eyes.[jamanetwork.com]
  • scotoma should raise a red flag that this is not papilledema.[e-tjo.org]

Treatment

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].

Prognosis

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].

Etiology

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

Epidemiology

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.

Sex distribution
Age distribution

Pathophysiology

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].

Prevention

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.

Summary

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].

Patient Information

  • 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. 

References

Article

  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.
  2. Rosenberg MA, Savino PJ, Glaser JS. A clinical analysis of pseudopapilledema. I. Population, laterality, acuity, refractive error, ophthalmoscopic characteristics, and coincident disease. Arch Ophthalmol 1979; 97:65.
  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.

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Last updated: 2019-07-11 22:30