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Papillophlebitis

History
- 1961 Lyle and Wybar described six young, healthy patients with a unilateral, relatively benign condition characterized by mild blurring of vision, essentially normal visual acuity, dilated and tortuous retinal vessels, a varying amount of retinal hemorrhage, and optic disc edema
- all six patients improved spontaneously, but were left with sheathing of retinal vessels and the formation of vessels on the optic disc
- they called this condition "retinal vasculitis" and believed it to be due to a central retinal vein occlusion secondary to an inflammatory vasculitis of the venous system.
- Lonn and Hoyt agreed with this etiology, but felt that "papillophlebitis" was a more appropriate descriptive term
- Hart and co-workers, however, pointed out that an inflammatory etiology for this disease is tenuous, and no well-documented cases have been studied histopathologically.

Signs and Symptoms
- typically under the age of 50 years
- no history of contributory systemic disease
- presence of systemic vascular disease in the patient with papillophlebitis is similar to that found in age-matched controls.
- mildly reduced visual acuity, visual field, or both
- most commonly a unilateral condition
- no racial or sexual predilection.
- dilated and tortuous retinal veins, dot & blot as well as flame shaped hemorrhages, exudates, cotton wool spots, retinal and macular edema, and disc edema
- in extreme cases, there is posterior and anterior segment neovascularization and possibly neovascular glaucoma
- mild cases, retinal hemorrhages may be absent with only an edematous optic disc present.

Pathophysiology
- essentially a central retinal vein occlusion (CRVO) occurring in a young, healthy patient
- aka: Big Blind Spot Syndrome (BBSS), benign retinal vasculitis, presumed phlebitis of the optic disc, optic disc vasculitis, and non-ischemic CRVO
- some theorize that papillophlebitis develops from inflammation of the retinal or papillary vessels
- histopathological sections have demonstrated extensive phlebitis and obliteration of lumens of arterioles and mononuclear inflammatory infiltration of the central retinal veins
- may develop extensive areas of retinal non-perfusion with subsequent neovascularization of the posterior or anterior segment and neovascular glaucoma
- patient is generally healthy
- hypertension has been noted in 23-42%
- diabetes has been found in only 3-9%
- hyperlipidema, hyperviscosity, and hypercoagulable states have not been found in greater incidence than age-matched controls

Management
- non-ischemic occlusion that can be expected to resolve in three to six months
- monthly observation (preferably with ocular photography) is indicated
- potential neovascularization of the disc, retina, iris, and angle must be carefully monitored
- 33% have a final visual acuity of 20/200 or worse due to complications such as chronic macular edema (37%), venous disc collaterals (33%)NVI (19%), NVG (8%)
- Central retinal vein occlusion in young adults (papillophlebitis). Fong AC et al. Retina 1992.
- prognosis is determined by the amount of initial capillary non-perfusion
- if papillophlebitis is initially non-ischemic, then prognosis is good
- optimal treatment has yet to be determined
- it is unclear whether or not anticoagulant therapy improves final visual outcome
- though it is theorized that papillophlebitis is an inflammatory condition, systemic steroids have not been demonstrated to be effective


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