Thomas A. Ciulla, MD Indianapolis
Though central serous retinopathy is common, much about the disorder remains
a mystery. Controversy continues over its cause, as well as the best way
to treat it. In this article, I'll explain what we know about the condition,
and go over the various management options.
What causes central serous retinopathy?
Central serous retinopathy occurs when a defect in the retinal pigment epithelium
allows choroidal fluid to leak into the subretinal space, forming a cyst-like
collection of subretinal fluid. No one knows exactly why this happens, but
many believe the disease may be stress-related; circulating catecholamines
resulting from stress may upset the metabolism of the macular RPE.1-6 In
fact, some researchers believe that this disease occurs predominantly in
"type A" individuals who lead stressful lifestyles.7,8 In support
of this theory, investigators have been able to reproduce a similar condition
in rabbits and primates with intravenous injection of epinephrine, a catecholamine
released in times of stress.9,10 It's important to note that pregnancy and
corticosteroid use have also been associated with this disorder.
Central serous retinopathy is 10 times more common in men than in women,11,12
and it's much more common in younger than older patients. The onset typically
occurs between the ages of 20 and 45. In fact, when patients who are older
than 50 report such symptoms, age-related macular degeneration should be
considered in the differential diagnosis.
The disease is uncommon in African Americans. There is no association with
high myopia.
Presentation and diagnosis
Patients typically present complaining of unilateral blurred vision, relative
central scotomas, metamorphopsia, chromopsia, or micropsia. Some complain
of headaches. The visual acuity with the patient's customary correction
can range from 20/20 to 20/200; it's typically possible to improve the visual
acuity to 20/20 by adding plus sphere to the refraction. Amsler grid testing
typically reveals relative central scotomas or metamorphopsia, and there
may be a delay in recovery time to bright light.
The posterior segment evaluation usually shows a serous retinal detachment
in the macula which correlates to the location of the scotoma or metamorphopsia.
This lesion usually ranges from one to two disc diameters in size. Alternatively,
patients can present with one or several retinal pigment epithelial detachments,
which are usually less than one disc diameter in size. In addition, there
can be mottling of the retinal pigment epithelium, which may show in the
fellow eye or be unassociated with the symptomatic lesion, and this is consistent
with prior episodes of central serous retinopathy. In some cases, you may
see subretinal yellow punctate precipitates. Some patients present with
multifocal disease, which is a poor prognostic indicator.
In general, it's a good idea to perform fluorescein angiography to rule
out choroidal neovascularization, especially if the diagnosis is uncertain
or atypical. In central serous retinopathy, the early phase angiogram usually
shows a punctate leak from the choroid through the involved incompetent
RPE. As the angiogram progresses, the dye accumulates beneath the serous
retinal detachment. Alternatively, in approximately 10 percent of cases,
the angiogram can show a classic "smokestack pattern," in which
the leaking dye rises in a thin line superiorly within the serous retinal
detachment and then billows laterally, like smoke from a smokestack.
In exudative age-related macular degeneration, by contrast, fluorescein
angiography typically shows classic choroidal neovascular membranes (discrete
early hyperfluorescence with late leakage) or occult choroidal neovascular
membranes (late leakage of undetermined source, or fibrovascular pigment
epithelial detachments which often present as stippled leakage).
Pigment epithelial detachments often present as discrete accumulation of
dye within the lesion which does not leak (enlarge in size or intensity)
in the later frames of the angiogram.
In central serous retinopathy, indocyanine green angiography will show areas
of presumed choroidal hyperpermeability surrounding the focal leak identified
on fluorescein angiography. Specifically, diffuse hyperfluorescence occurs
early on the ICG angiogram, followed by central hypofluorescence with a
surrounding ring of hyperfluorescence in the mid and late frames.13 An added
benefit of ICG angiography is that it will also reveal occult serous RPE
detachments, as well as other discrete areas of presumed choroidal hyperpermeability,
not evident clinically or on fluorescein angiography.13
The differential diagnosis of this disorder includes:
Exudative age-related macular degeneration. Unlike central serous retinopathy,
this disorder most commonly occurs in patients over 50. In fact, according
to the international disease classification system, age-related macular
degeneration cannot be diagnosed in individuals under the age of 50.13 Key
differentiating signs of the nonexudative "dry" form include drusen,
RPE hypertrophy and RPE atrophy. The key differentiating signs of the "wet"
form include subretinal fluid, retinal pigment epithelial detachments, subretinal
lipid, or flecks of subretinal hemorrhage. Age-related macular degeneration
also tends to occur bilaterally, whereas central serous retinopathy does
not.
Other causes of choroidal neovascularization, including presumed histoplasmosis
syndrome, myopic degeneration, angioid streaks, and idiopathic neovascularization.
Presumed ocular histoplasmosis syndrome patients often exhibit peripapillary
atrophy as well as "histo-spots" in the periphery. They also have
a history of living in the Ohio-Mississippi river valley. Myopic degeneration
patients typically have tilted discs, peripapillary atrophy, and thinned
RPE generally. In patients with more than 6 diopters of myopia, you may
also see laquer cracks. Also, both the presumed ocular histoplasmosis syndrome
and myopic degeneration are bilateral disorders.
Optic nerve pits with associated serous detachment of the macula. Optic
nerve pits, or small hypopigmented defects in the optic nerve, can be associated
with contiguous detachment of the macula. This disorder is usually unilateral.
The differential diagnosis also includes:
Choroidal tumors, including choroidal melanoma, metastasis, and hemangioma.
Inflammatory and infectious disorders, such as posterior scleritis, Harada's
disease or sympathetic ophthalmia, sarcoidosis, and the uveal effusion syndrome.
Vascular disorders, such as malignant hypertension or toxemia of pregnancy.
Other maculopathies, including Best's disease.
Treatment
Once the diagnosis is made, some physicians tell patients that they are
living a "Type A" lifestyle and counsel them to calm down. I recommend
a more cautious approach. If I am the first to make the diagnosis, I mention
that some doctors feel that stress and a "type A" personality
may play a role, but hasten to add that, "I'm not sure these findings
apply to all patients." I do this because some patients actually become
upset and change doctors when counseled to modify their lifestyles.
Typically, the first course of action is simply to monitor the patient,
following up every four to six weeks to watch for resolution of the signs
and symptoms.
If the condition does not resolve in the first several visits, or if the
patient has had an earlier bout of central serous retinopathy and done poorly
with it, the next step typically is treatment with either medications or
laser.
One option is alpha/beta-blockers. Although no studies exist proving the
efficacy of these drugs, the idea of prescribing them is reasonable if one
accepts that epinephrine, a powerful stimulator of both types of receptors,
is involved in the pathogenesis of the disease.7,14 Some believe that the
mixed /ß receptor antagonist Labetolol, 100 mg bid po, speeds resolution.14
Always be sure to discuss beta-blockers with the patient's internist prior
to proceeding.
Some authors have also advocated acetozolamide, suggesting that this may
enhance the RPE pump or favorably alter choriocapillaris hemodynamics.
When the disorder persists for longer than four to six months, when patients
have persistent visual field defects from earlier episodes, and occasionally
when patients need excellent vision for their work, the preferred therapy
may be laser treatment of the leaking focal spot. Compared to simple observation,
this treatment can speed resolution by up to two months. However, it does
not enhance the ultimate outcome,11,15,16 and it can result in complications,
include misplaced laser spots, hemorrhage, and rupture of Bruch's membrane
with subsequent choroidal neovascularization. To perform the treatment,
use an argon green laser to apply three to five low-intensity spots (0.2
sec duration, 200 µm, 100 mW argon green) to the leaking focus to
until you see a faint white treatment spot.16 Always avoid treating the
fovea directly. Follow treated patients at two-week intervals.
The prognosis for central serous retinopathy is generally good. In most
cases, it resolves spontaneously within three months, although complete
resolution of the symptoms may take as much as six months longer.11,12 Approximately
95 percent of all patients recover 20/30 visual acuity or better. After
resolution of the symptoms, you may still observe mottling of the RPE.
In a subset of patients, the disease takes a more malignant course, failing
to resolve even after treatment. Patients with prolonged detachment or recurrent
detachment of the macula can suffer permanent loss of central visual acuity.
As many as 45 percent of all patients experience recurrences,12 sometimes
many years after the original episode. Central serous retinopathy can also
affect the fellow eye. In most cases, treat recurrences the same as primary
occurrence. However, it may be best to initiate treatment early if the prior
episodes have left any permanent sequellae.
Central serous retinopathy can cause hardship or even permanently damage
vision. But with accurate diagnosis and appropriate treatment, most patients
will experience quick resolution and full visual recovery.
Dr. Ciulla is a surgeon in the retina service at the Indiana University
School of Medicine.
References
1. Spitznas M. Pathogenesis of central serous retinopathy: a new working
hypothesis. Graefes Arch Clin Exp Ophthalmol. 1986;224:321-4.
2. Marmor MF. New hypotheses on the pathogenesis and treatment of serous
retinal detachment. Graefes Arch Clin Exp Ophthalmol. 1988;226:548-52.
3. Marmor M. On the cause of serous detachments and acute central serous
chorioretinopathy. Br J Ophthalmol. 1997;81:812-3.
4. Negi A, Marmor MF. Experimental serous retinal detachment and focal pigment
epithelial damage. Arch Ophthalmol. 1984;102:445-9.
5. Prunte C, Flammer J. Choroidal capillary and venous congestion in central
serous chorioretinopathy. Am J Ophthalmol. 1996;121:26-34.
6. Prunte C. Indocyanine green angiographic findings in central serous chorioretinopathy.
Int Ophthalmol. 1995;19:77-82.
7. Yannuzzi LA. Type A behavior and central serous chorioretinopathy. Trans
Am Ophthalmol Soc. 1986;84:799-845.
8. Yannuzzi LA. Type A behavior and central serous chorioretinopathy. Retina.
1987;7:111-31.
9. Yoshioka H, Katsume Y, Akune H. Experimental central serous chorioretinopathy
in monkey eyes: fluorescein angiographic findings. Ophthalmologica. 1982;185:168-78.
10. Nagayoski K. Experimental study of chorioretinopathy by intravenous
injection of adrenaline. Acta Soc Ophthalmol Jpn. 1971;75:
1720-1727.
11. Gilbert CM, Owens SL, Smith PD, Fine SL. Long-term follow-up of central
serous chorioretinopathy. Br J Ophthalmol. 1984;68:815-20.
12. Klein M, van Buskirk E, Friedman E, Gragoudas E, Chandra S. Experience
with nontreatment of central serous choroidopathy. Arch Ophthalmol. 1974;91:247-250.
13. Guyer D, Yannuzzi L, Slakter J, et al. Digital indocyanine green videoangiography
of central serous retinopathy. Arch Ophthalmol. 1994;94:1057-1062.
14. Prunte C, Sommer M, Orgul S, Flammer J. Mixed alpha-beta-adrenergic
antagonist treatment of central serous chorioretinopathy. Investig Ophthalmol
Vis Sci (Suppl). 1998;39:830.
15. Robertson DM, Ilstrup D. Direct, indirect, and sham laser photocoagulation
in the management of central serous chorioretinopathy. Am J Ophthalmol 1983;95:457-66.
16. Robertson DM. Argon laser photocoagulation treatment in central serous
chorioretinopathy. Ophthalmology. 1986;93:972-4.
Review of Ophthalmology March 1999