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