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January/February 2009 - Louisiana Society of Health-System ...

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<strong>Louisiana</strong> <strong>Society</strong> <strong>of</strong> <strong>Health</strong>-<strong>System</strong> Pharmacists Page 6<br />

Central Serous Chorioretinopathy<br />

Nicole Ehrlicher, PharmD Candidate & Lisa DiGioia-Ross, PharmD<br />

Central serous chorioretinopathy or central serous<br />

retinopathy (CSR) is a disease that involves a retinal detachment<br />

<strong>of</strong> the macular layers <strong>of</strong> the eye in which fluid leaks into the<br />

subretinal space. The detachment can cause tears or breaks in<br />

the retinal pigment epithelium which leads to the build up <strong>of</strong> this<br />

endogenous fluid or edema. CSR is usually idiopathic in nature<br />

and self-limiting with about 80% <strong>of</strong> patients regaining 20/25<br />

vision or better. Unfortunately, patients with CSR have a 40-<br />

50% risk <strong>of</strong> recurrence in the same eye. CSR usually results in<br />

blurred, distorted vision or metamorphopsia, a gray blind spot in<br />

the central vision, and/or flashes <strong>of</strong> light or photopsia.<br />

CSR occurs most commonly in males between the ages<br />

<strong>of</strong> 20-55 who are considered to have Type A personalities.<br />

People with Type A personalities are usually extremely<br />

competitive, impatient, time conscious, aggressive, and<br />

sometimes considered workaholics. CSR is uncommon in the<br />

African American population; however, it is extremely severe in<br />

the Hispanic and Asian populations. Women tend to develop<br />

CSR at a later age compared to men. Patients diagnosed after<br />

the age <strong>of</strong> 50 usually have the disorder bilaterally and usually<br />

have a history <strong>of</strong> corticosteroid use or severe uncontrolled<br />

hypertension.<br />

Even though the pathophysiology <strong>of</strong> CSR is not well<br />

understood, stress seems to play a role in the development <strong>of</strong> the<br />

disease. Stress causes stimulation <strong>of</strong> the sympathetic nervous<br />

system which in turn can cause local weakness in the Bruch’s<br />

membrane (a transparent inner membrane <strong>of</strong> the choroid which<br />

is in contact with retinal pigment epithelium). This subsequently<br />

leads to the leakage <strong>of</strong> fluid secondary to a defect in the retinal<br />

pigment epithelium and ultimately, could cause a focal<br />

detachment <strong>of</strong> the retina. Another hypothesis claims the<br />

pathophysiology involves an abnormal ion transport across the<br />

retinal pigment epithelium which can result in focal choroidal<br />

vasculopathy. Other hypothetical systemic causes include<br />

hypertension, organ transplantation, systemic lupus<br />

erythematosus (SLE), Cushing’s syndrome, steroid use, and<br />

gastroesophogeal reflux disease (GERD). All <strong>of</strong> these systemic<br />

conditions are associated with a high level <strong>of</strong> stress and cortisol<br />

release. Also there has been a correlation between Helicobacter<br />

pylori and CSR. Approximately 70% <strong>of</strong> CSR patients are<br />

infected with Helicobacter pylori. Further studies are needed to<br />

determine if an infection with Helicobacter pylori is a risk<br />

factor.<br />

It has been shown in many clinical trials that prolonged<br />

corticosteroid use can exacerbate central serous retinopathy.<br />

Corticosteroids play a role in the expression <strong>of</strong> adrenergic<br />

receptors which when stimulated by the sympathetic nervous<br />

system, cause the release <strong>of</strong> catecholamines (epinephrine,<br />

norepinephrine, and dopamine). This release occurs mainly<br />

during stressful situations and therefore, may contribute to the<br />

pathogenesis <strong>of</strong> CSR. A study <strong>of</strong> 50 CSR patients showed that<br />

26 patients (52%) had a history <strong>of</strong> exogenous steroid use. In<br />

addition, most retinas reattach spontaneously once systemic<br />

corticosteroids are discontinued. <strong>System</strong>ic corticosteroid use is<br />

the most prevalent risk factor for developing or exacerbating<br />

CSR and should be avoided in patients with this disease.<br />

There has also been some speculation that Viagra®<br />

(sildenafil) may have a relationship to CSR, although a causal<br />

relationship has not been established. However, a retrospective<br />

study showed that 8 out <strong>of</strong> 11 patients taking sildenafil and<br />

diagnosed with CSR had improved vision upon discontinuation.<br />

Therefore, most physicians do not recommend the use <strong>of</strong><br />

sildenafil in patients with refractory CSR. Other agent that<br />

should be avoided in patients with CSR includes aspirin and<br />

caffeine plus other stimulants and possibly non-steroidal antiinflammatory<br />

drugs (NSAIDs). In addition, since elevated<br />

opiate levels from use <strong>of</strong> heroin or morphine have also been<br />

associated with CSR it would be best to avoid using these agents<br />

as well.<br />

Patients with CSR can present with symptoms <strong>of</strong> visual<br />

loss, metamorphopsia, decrease or distortion in central vision,<br />

scotoma, loss <strong>of</strong> color perception and loss <strong>of</strong> contrast sensitivity.<br />

Most patients complain <strong>of</strong> objects being distorted in some way<br />

and miniaturized. However, CSR can be asymptomatic and is<br />

usually unilateral. Some classic signs <strong>of</strong> CSR include local<br />

serous detachment <strong>of</strong> the retina usually within the region <strong>of</strong> the<br />

macula, serous fluid void <strong>of</strong> any lipid or heme, retinal<br />

epithelium irregularities, and possible subretinal fibrin<br />

deposition. CSR is commonly diagnosed by using optical<br />

coherence topography (OCT) and fluorescein angiogram (FA).<br />

If the OCT shows subretinal fluid, retinal pigment epithelium<br />

detachment, neurosensory macula detachment, and any retinal<br />

atrophy resulting from a chronic disease state, it may be<br />

suggestive <strong>of</strong> CSR. The FA is able to detect leaks at the level <strong>of</strong><br />

the retinal pigment epithelium, and in about 10-15% <strong>of</strong> patients,<br />

a classic “smoke stack” appearance <strong>of</strong> the leak will be present.<br />

Even though CSR has serious signs and symptoms, the<br />

prognosis is usually good if the disease in caught in the early<br />

stages. As mentioned earlier, about 80% <strong>of</strong> patient will have<br />

20/25 vision or better leaving only 20% failing to recover vision<br />

<strong>of</strong> 20/30 or better. Most <strong>of</strong> the patients failing to regain adequate<br />

vision usually have a chronic form <strong>of</strong> CSR secondary to another<br />

uncontrolled disease (i.e. hypertension or SLE). Patients with<br />

chronic CSR usually have chronic serous retinal detachment<br />

caused by severe retinal pigment epithelium atrophy and only<br />

regain about 20/200 vision. Even if the disease is detected early,<br />

there is a risk <strong>of</strong> about 50% recurrence <strong>of</strong> CSR in the same the<br />

eye.<br />

The treatment and management <strong>of</strong> CSR is not well<br />

defined. Laser photocoagulation is commonly used in the<br />

treatment <strong>of</strong> CSR to cauterize the subretinal leak areas and<br />

prevent fluid from escaping. Laser photocoagulation is usually<br />

only used during the following complications <strong>of</strong> CSR: a serous<br />

detachment <strong>of</strong> greater than four months or recurrence <strong>of</strong> CSR in<br />

the same or opposite eye with visual deficits. Laser<br />

photocoagulation is also used if the serous detachment is more<br />

than 300 micrometers from the center <strong>of</strong> the fovea. If laser<br />

treatment is concentrated too close to the macula, it can leave a<br />

permanent blind spot. If the leak is too close to the macula, then<br />

transpupillary thermotherapy has been used as a lower risk<br />

alternative laser treatment. Laser treatment has been shown to<br />

CSR continues on next page

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