C A S E R E P O R T

Bilateral Papillophlebitis in a Patient withAccelerated Essential Hypertension

ARIJIT MITRA, DO, MRCSEdA B S T R A C T

We describe a case of bilateral papillophlebitis with discedema, scattered cotton-wool spots, flame-shaped hemor-rhages, and exudates around the optic discs and in themacula of a 33-year-old patient with essential hyperten-sion. The pathophysiology and natural course of the diseaseis discussed.

INTRODUCTION

Central retinal vein occlusion (CRVO) is usually seen

in older adults and is often associated with systemic

vascular disease. Inflammation of the central retinal

vein has been proposed as a cause of the occlusion in

young adults and, for that reason, it has been called

papillophlebitis. We report a case of bilateral

papillophlebitis in a 33-year-old healthy male with

accelerated essential hypertension. The pathophysi-

ology and natural course of the condition is discussed.

CASE REPORT

A 33-year-old healthy male patient presented to the

Emergency Department with mild loss of vision in the

left eye. He had high myopia and was a contact lens

wearer. On presentation, the best corrected vision in

the right eye was 6/9 and in the left eye it was 6/12. He

had no history of headaches or medical problems, was

not on any medications, and had no significant ocular

history in the past. Anterior segment examination and

intraocular pressures were within normal limits.

There was no relative afferent papillary defect. Fun-

dus examination showed bilateral papillophlebitis,

with disc edema, scattered cotton-wool spots, flame-

shaped hemorrhages, and exudates around the optic

discs and in the macula. The fovea was largely spared

in both eyes (Figs. 1 and 2). There was no evidence of

long-standing hypertensive retinopathy changes.

Blood pressure readings in the Emergency Depart-

ment were 246/136 mm Hg. Capillary blood sugar

R E P R I N T SArijit Mitra, DO, MRCSEd, Specialist Registrar, Wolverhampton and Midland

Counties Eye Infirmary, Compton Road, Wolverhampton, West Midlands WV3

9QR, UK. E-mail: drarijitmitra@yahoo.com.

The author has stated that he does not have a significant financial interest or

other relationship with any product manufacturer or provider of services dis-

cussed in this article. The author also does not discuss the use of off-label

products, which include unlabeled, unapproved, or investigative products or

devices.

The author describes a case of bilateral papillophlebitis in a patient with

essential hypertension in which significant visual improvement is seen following

stabilization of the blood pressure.

Submitted for publication: 3/12/07. Accepted: 6/29/07.

Annals of Ophthalmology, vol. 39, no. 4, Winter 2007

� Copyright 2007 by ASCO

All rights of any nature whatsoever reserved.

1530-4086/07/39:337–339/$30.00. ISSN 1558-9951 (Online)

ANN OPHTHALMOL. 2007;39 (4) ..............................................337

level was normal. The patient was admitted to the

Medical Assessment Unit, where he underwent

extensive blood investigations and had an ultrasound

scan of the kidneys to rule out any secondary causes

of hypertension. No apparent cause was detected

from the investigations and the patient was treated

for essential hypertension. No other end-organ dam-

age was detected.

Within two weeks, the patient’s blood pressure

stabilized to around 150/100 mm Hg with treatment.

His vision improved to 6/4 in the right eye and 6/5 in

the left. Fundoscopy revealed fewer exudates, cotton-

wool spots, and flame-shaped hemorrhages.

DISCUSSION

A hypertensive emergency is a condition in which

elevated blood pressure results in target organ dam-

age. The systems primarily involved include the cen-

tral nervous system, the cardiovascular system, the

kidneys, and the retina. Hypertensive urgency must

be distinguished from emergency. Urgency is defined

as severely elevated blood pressure (i.e., sys-

tolic>220 mm Hg or diastolic>120 mm Hg) with

no evidence of target organ damage. Hypertensive

emergencies require immediate therapy to decrease

the blood pressure within minutes to hours. In con-

trast, no evidence suggests a benefit from rapidly

reducing the blood pressure in patients with hyper-

tensive urgency. In fact, such aggressive therapy may

harm the patient, resulting in cardiac, renal, or cere-

bral hypoperfusion.

The pathogenesis of accelerated hypertension is not

fully understood. The characteristic vascular lesion is

the fibrinoid necrosis of arterioles and small arteries,

which causes the clinical manifestations of end-organ

damage. Red blood cells are damaged as they flow

through vessels obstructed by fibrin deposition,

resulting in microangiopathic hemolytic anemia. In

patients without hypertension, flow is kept constant

over a mean pressure of 60–120 mm Hg. In patients

with hypertension, flow is constant over a mean

pressure of 110–180 mm Hg because of arteriolar

thickening. When blood pressure is raised above the

upper limit of autoregulation, arterioles dilate. This

results in hyperperfusion and end-organ damage.

Papillophlebitis is the term that is often used to

describe a CRVO with preserved visual acuity in a

young patients (1), typically under the age of

50 years. Historically, papillophlebitis has been re-

ferred 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 elderly patients due to arteriolo-

sclerosis and thrombus formation, this does not seem

to be the case in papillophlebitis. It is thought that the

underlying lesion is optic disc swelling with resultant

secondary venous congestion. Histopathological sec-

tions in papillophlebitis have demonstrated extensive

phlebitis and obliteration of lumens of arterioles and

mononuclear inflammatory infiltration of the central

retinal veins.

Papillophlebitis is usually unilateral. The patient

with papillophlebitis is generally healthy. Hyperten-

sion has been noted in 23–42% of patients with pa-

pillophlebitis. Diabetes has been found in only 3–9%

of patients. Hyperlipidema, hyperviscosity, and

hypercoagulable states have not been found in pa-

tients with papillophlebitis in greater incidence than

age-matched controls.

Fundus fluorescein angiographic findings include

dilated and tortuous retinal veins, prominent vascular

staining, blocked fluorescence, microaneurysms, disc

Figure 1—Right eye showing papillophlebitis with mildinvolvement of the fovea.

Figure 2—Left eye showing papillophlebitis without involve-ment of the fovea.

ANN OPHTHALMOL. 2007;39 (4) ..............................................338

edema, and peripapillary dye spillage. Typically, pa-

pillophlebitis is a non-ischemic occlusion that can be

expected to resolve in three to six months. Monthly

observation (preferably with ocular photography) is

indicated.

Occasionally, however, as with true CRVO, papil-

lophlebitis may develop extensive areas of retinal

non-perfusion with subsequent neovascularization of

the posterior or anterior segment and neovascular

glaucoma (2). Most eyes recover vision to better than

20/40, about one-fifth have significant visual loss, and

many suffer ocular sequelae (3). Although many

treatment modalities have been attempted for this

entity, no conclusive evidence exists that any treat-

ment alters its natural history.

Although young patients with CRVO usually

present with no history of contributory systemic dis-

ease, a thorough physical examination may prevent

life-threatening complications, as has been high-

lighted by this case. To the best of our knowledge,

bilateral papillophlebitis secondary to accelerated

hypertension has not been reported before. The nat-

ural clinical course of this condition is one of spon-

taneous resolution and treatment is usually

unnecessary (4). However, all of these patients

should be referred to a primary care physician for

systemic evaluation. If any abnormalities are found,

they must be aggressively treated.

REFERENCES

1. D’Amato RJ, Miller NR, Fine SL, et al. The effect of age and

initial visual acuity on the systemic and visual prognosis of central

retinal vein occlusion. Aust N Z J Ophthalmol 1991;19:118–122.

2. Sachdev MS, Sood NN, Mohan M, et al. Optic disc vasculitis in

epidemic dropsy. Jpn J Ophthalmol 1987;31:467–474.

3. Fong AC, Schatz H. Central retinal vein occlusion in young adults.

Surv Ophthalmol 1993;37:393–417. Erratum in: Surv Ophthalmol

1993;38:88.

4. McLoone EM, Best RM. Pregnancy-related papillophlebitis. Eur J

Ophthalmol 2004;14:65–66.

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