Signs and Symptoms
The patient with papillophlebitis is younger, typically under the age of 50
years. Further, the patient presents with no history of contributory systemic disease. The
presence of systemic vascular disease in the patient with papillophlebitis is similar to
that found in age-matched controls.
The patient may present with mildly reduced visual acuity, visual field,
or both. Papillophlebitis is most commonly a unilateral condition. There is no racial or
Ophthalmoscopically, there are 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. In mild cases, retinal hemorrhages
may be absent with only an edematous optic disc present.
Papillophlebitis is essentially a central retinal vein occlusion (CRVO)
occurring in a young, healthy patient. Historically, papillophlebitis has been referred to
as Big Blind Spot Syndrome (BBSS), benign retinal vasculitis, presumed phlebitis of the
optic disc, optic disc vasculitis, and non-ischemic CRVO. While CRVO develops in the
elderly patient due to arteriolosclerosis and thrombus formation, this doesnt seem
to be the case in papillophlebitis. Some theorize that papillophlebitis develops from
inflammation of the retinal or papillary vessels. Histopathological sections in
papillophlebitis have demonstrated extensive phlebitis and obliteration of lumens of
arterioles and mononuclear inflammatory infiltration of the central retinal veins.
As with true CRVO, papillophlebitis may develop extensive areas of
retinal non-perfusion with subsequent neovascularization of the posterior or anterior
segment and neovascular glaucoma.
Typically, papillophlebitis is a 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, and referred to a retinal specialist if discovered. A
significant number of patients have a final visual acuity of 20/200 or worse due to
complications such as chronic macular edema and subsequent macular compromise, tractional
retinal detachment, or neovascular glaucoma. Prognosis is determined by the amount of
initial capillary non-perfusion. If papillophlebitis is initially non-ischemic, then
prognosis is good. Fortunately, the vast majority of cases will be non-ischemic.
Due to the unknown etiology of papillophlebitis, the 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.
The patient with papillophlebitis is generally healthy. Hypertension has
been noted in 23-42 percent of patients with papillophlebitis. Diabetes has been found in
only 3-9 percent of patients. Hyperlipidema, hyperviscosity, and hypercoagulable states
have not been found in patients with papillophlebitis in greater incidence than
age-matched controls. Patients should be referred to a primary care physician for systemic
evaluation. If any abnormalities are found, they must be aggressively treated.
Papillophlebitis is a CRVO in a young, healthy adult.
Papillophlebitis is typically benign, but may develop the
same neovascular complications as an ischemic CRVO.
Medical testing is frequently unproductive.
Other reports in this section