Papillophlebitis

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 sexual predilection.

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.

Pathophysiology
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 doesn’t 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.

Management
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.

Clinical Pearls

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.