Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Overview and differential diagnosis of papilledema

Don C Bienfang, MD
Section Editor
Paul W Brazis, MD
Deputy Editor
Janet L Wilterdink, MD


While the term papilledema is often used broadly to denote a swollen optic nerve head, the term papilledema should be reserved for optic disc swelling that is due to raised intracranial pressure.

This topic will provide an overview and differential diagnosis of papilledema. The entity of increased intracranial pressure and specific causes of papilledema are discussed elsewhere. (See "Evaluation and management of elevated intracranial pressure in adults".)


Papilledema occurs when raised intracranial pressure is transmitted to the optic nerve sheath. The raised pressure mechanically disrupts axoplasmic flow within the nerve. Obstipation of intra-axonal fluid results in swelling of the axons and leakage of water, protein, and other cellular contents into the extracellular space of the optic disc giving rise to optic disc edema [1,2]. Venous obstruction and dilation, nerve fiber ischemia, and vascular telangiectasias are secondary phenomena.

Any entity that increases intracranial pressure may lead to papilledema. These include:

Intracranial mass lesions (eg, tumor, hematoma)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Aug 16, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Minckler DS, Tso MO, Zimmerman LE. A light microscopic, autoradiographic study of axoplasmic transport in the optic nerve head during ocular hypotony, increased intraocular pressure, and papilledema. Am J Ophthalmol 1976; 82:741.
  2. Tso MO, Hayreh SS. Optic disc edema in raised intracranial pressure. IV. Axoplasmic transport in experimental papilledema. Arch Ophthalmol 1977; 95:1458.
  3. Biousse V, Newman NJ. Intracranial vascular abnormalities. Ophthalmol Clin North Am 2001; 14:243.
  4. Adelman JU. Headaches and papilledema secondary to dural arteriovenous malformation. Headache 1998; 38:621.
  5. Costello F, Kardon RH, Wall M, et al. Papilledema as the presenting manifestation of spinal schwannoma. J Neuroophthalmol 2002; 22:199.
  6. Amlashi SF, Riffaud L. Images in clinical medicine. Papilledema and spinal cord tumor. N Engl J Med 2004; 350:e18.
  7. Brockmeier B, Burbach H, Runge M, Altenkirch H. Raised intracranial pressure in chronic respiratory disease. Lancet 1997; 349:883.
  8. Purvin VA, Kawasaki A, Yee RD. Papilledema and obstructive sleep apnea syndrome. Arch Ophthalmol 2000; 118:1626.
  9. Delany C, Jay WM. Papilledema and abducens nerve palsy following ethylene glycol ingestion. Semin Ophthalmol 2004; 19:72.
  10. Morrison KE, Davies PT. Chronic inflammatory demyelinating polyneuropathy presenting with headache and papilledema. Headache 1999; 39:299.
  11. Thomas S, Tan J, Lawden M, Sampath R. Optic nerve sheath fenestration for intracranial hypertension associated with chronic inflammatory demyelinating polyneuropathy. Ophthal Plast Reconstr Surg 2004; 20:325.
  12. Fantin A, Feist RM, Reddy CV. Intracranial hypertension and papilloedema in chronic inflammatory demyelinating polyneuropathy. Br J Ophthalmol 1993; 77:193.
  13. Erşahin Y, Mutluer S, Yurtseven T. Hydrocephalus in Guillain-Barré syndrome. Clin Neurol Neurosurg 1995; 97:253.
  14. Sadun AA, Currie JN, Lessell S. Transient visual obscurations with elevated optic discs. Ann Neurol 1984; 16:489.
  15. Pearson PA, Baker RS, Khorram D, Smith TJ. Evaluation of optic nerve sheath fenestration in pseudotumor cerebri using automated perimetry. Ophthalmology 1991; 98:99.
  16. Wall M, Hart WM Jr, Burde RM. Visual field defects in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol 1983; 96:654.
  17. Frisén L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry 1982; 45:13.
  18. Elliot RH. THE RETINAL PULSE. Br J Ophthalmol 1921; 5:481.
  19. ENGEL S. Venous pulsation as a symptom of early glaucoma. Am J Ophthalmol 1946; 29:1446.
  20. Hedges TR Jr, Baron EM, Hedges TR 3rd, Sinclair SH. The retinal venous pulse. Its relation to optic disc characteristics and choroidal pulse. Ophthalmology 1994; 101:542.
  21. Levin BE. The clinical significance of spontaneous pulsations of the retinal vein. Arch Neurol 1978; 35:37.
  22. Levine DN. Spontaneous pulsation of the retinal veins. Microvasc Res 1998; 56:154.
  23. Lorentzen SE. Incidence of spontaneous venous pulsation in the retina. Acta Ophthalmol (Copenh) 1970; 48:765.
  24. Pines, N. Some clinical notes on the nature of the retinal venous pulse. Brit J Ophthalmol 1938; 22:470.
  25. Walsh TJ, Garden JW, Gallagher B. Obliteration of retinal venous pulsations during elevation of cerebrospinal-fluid pressure. Am J Ophthalmol 1969; 67:954.
  26. Weinstein, P. Significance of venous pulsation of the eyeground. Brit J Ophthalmol 1939; 23:396.
  27. Williamson-Noble, FA. Venous pulsation. Trans Ophthalmol UK 1952; 72:317.
  28. Friedman DI. Papilledema and pseudotumor cerebri. Ophthalmol Clin North Am 2001; 14:129.
  29. Davis PL, Jay WM. Optic nerve head drusen. Semin Ophthalmol 2003; 18:222.
  30. Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47:515.
  31. 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.
  32. Lee AG, Beaver HA. Acute bilateral optic disk edema with a macular star figure in a 12-year-old girl. Surv Ophthalmol 2002; 47:42.
  33. Spencer CG, Lip GY, Beevers DG. Recurrent malignant hypertension: a report of two cases and review of the literature. J Intern Med 1999; 246:513.
  34. Kishi S, Tso MO, Hayreh SS. Fundus lesions in malignant hypertension. II. A pathologic study of experimental hypertensive optic neuropathy. Arch Ophthalmol 1985; 103:1198.
  35. Vaphiades MS. The disk edema dilemma. Surv Ophthalmol 2002; 47:183.
  36. Wu GF, Balcer LJ. Endocrine and metabolic deficiency. Ophthalmol Clin North Am 2004; 17:427.
  37. Pavan PR, Aiello LM, Wafai MZ, et al. Optic disc edema in juvenile-onset diabetes. Arch Ophthalmol 1980; 98:2193.
  38. Bayraktar Z, Alacali N, Bayraktar S. Diabetic papillopathy in type II diabetic patients. Retina 2002; 22:752.
  39. Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol 2003; 135:437.
  40. Rizzo JF 3rd, Lessell S. Optic neuritis and ischemic optic neuropathy. Overlapping clinical profiles. Arch Ophthalmol 1991; 109:1668.
  41. KENNEDY C, CARROLL FD. Optic neuritis in children. Trans Am Acad Ophthalmol Otolaryngol 1960; 64:700.
  42. Balcer LJ, Winterkorn JM, Galetta SL. Neuro-ophthalmic manifestations of Lyme disease. J Neuroophthalmol 1997; 17:108.
  43. Khubchandani R, Rane T, Agarwal P, et al. Bilateral neuroretinitis associated with mumps. Arch Neurol 2002; 59:1633.
  44. Perrotta S, Nobili B, Grassia C, et al. Bilateral neuroretinitis in a 6-year-old boy with acquired toxoplasmosis. Arch Ophthalmol 2003; 121:1493.
  45. Labalette P, Bermond D, Dedes V, Savage C. Cat-scratch disease neuroretinitis diagnosed by a polymerase chain reaction approach. Am J Ophthalmol 2001; 132:575.
  46. Cinefro RJ, Frenkel M. Systemic lupus erythematosus presenting as optic neuritis. Ann Ophthalmol 1978; 10:559.
  47. Pelton RW, Lee AG, Orengo-Nania SD, Patrinely JR. Bilateral optic disk edema caused by sarcoidosis mimicking pseudotumor cerebri. Am J Ophthalmol 1999; 127:229.
  48. Beardsley TL, Brown SV, Sydnor CF, et al. Eleven cases of sarcoidosis of the optic nerve. Am J Ophthalmol 1984; 97:62.
  49. Prisco D, Marcucci R. Retinal vein thrombosis: risk factors, pathogenesis and therapeutic approach. Pathophysiol Haemost Thromb 2002; 32:308.
  50. Quinlan PM, Elman MJ, Bhatt AK, et al. The natural course of central retinal vein occlusion. Am J Ophthalmol 1990; 110:118.
  51. Ellenberger C Jr, Messner KH. Papillophlebitis: benign retinopathy resembling papilledema or papillitis. Ann Neurol 1978; 3:438.
  52. Lonn LI, Hoyt WF. Papillophlebitis: a cause of protracted yet benign optic disc edema. Eye Ear Nose Throat Mon 1966; 45:62 passim.
  53. Mayo GL, Carter JE, McKinnon SJ. Bilateral optic disk edema and blindness as initial presentation of acute lymphocytic leukemia. Am J Ophthalmol 2002; 134:141.
  54. Wabbels B, Demmler A, Seitz J, et al. Unilateral adult malignant optic nerve glioma. Graefes Arch Clin Exp Ophthalmol 2004; 242:741.
  55. Nikaido H, Mishima H, Ono H, et al. Leukemic involvement of the optic nerve. Am J Ophthalmol 1988; 105:294.
  56. Nikoskelainen EK, Huoponen K, Juvonen V, et al. Ophthalmologic findings in Leber hereditary optic neuropathy, with special reference to mtDNA mutations. Ophthalmology 1996; 103:504.
  57. Gass JD, Norton EW. Cystoid macular edema and papilledema following cataract extraction: a fluorescein fundoscopic and angiographic study. 1966. Retina 2003; 23:646.
  58. Shrieve DC, Hazard L, Boucher K, Jensen RL. Dose fractionation in stereotactic radiotherapy for parasellar meningiomas: radiobiological considerations of efficacy and optic nerve tolerance. J Neurosurg 2004; 101 Suppl 3:390.
  59. Jiang GL, Tucker SL, Guttenberger R, et al. Radiation-induced injury to the visual pathway. Radiother Oncol 1994; 30:17.
  60. Orcutt JC, Page NG, Sanders MD. Factors affecting visual loss in benign intracranial hypertension. Ophthalmology 1984; 91:1303.