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Steroid-Induced Chronic Central Serous Chorioretinopathy Srinivas, S and Frick, R Veterans Affairs Hospital White River Junction, Vermont ABSTRACT (limit 35 words) One of the side effects of oral corticosteroid use is Central Serous Chorioretinopathy (CSCR). The following case is about a patient with Steroid-Induced Chronic CSCR following prednisone use during renal transplants. I. Case History -Patient demographics 49 year-old Caucasian male -Chief complaint (7/16/12) Vision was variable around the time of renal transplant in 2004 and 2011. Patient reported that vision has stabilized since after the transplant. No other visual complaints at present visit in 7/2012. -Ocular, medical history Ocular Hx: (Assessment // Plan as of last eye exam on 5/3/2005) Macular defect OS, longstanding since acute renal failure in 2000 // Monitor with dilated exam Essential Emmetropia with Presbyopia OU // Near prescription given No history of eye surgery or eye injury Medical Hx: Chronic Kidney Disease Stage 3; (Status-post renal two renal transplants, both at Dartmouth Hitchcock Medical Center in 2004 and 2011). Patient had been on dialysis prior to each transplant). Patient was on dual immunosuppression with Prograf and Cellcept. Anemia of Chronic Kidney Disease Hypertension Secondary HyperParaThyroidism, on calcitriol -Medications Diltiazem Tacrolimus Mycophenolate Metoprolol Simvastatin Calcitrol Sildenafil Prograf Cellcept -Other salient information Last Eye Exam: 5/3/2005 at Veterans Hospital White River Junction (VA WRJ). Patient had first eye exam at VA WRJ on 5/3/2005. He was lost to follow-up since then and did not show for eye appointments. Page 1 of 9 II. Pertinent findings (7/2012) -Clinical Visual acuity was OD 20/20-2 and OS 20/40 with a myopic and astigmatic prescription: OD: plano-0.25X20 and OS: -0.25-0.50X105 with a +1.75 add. Pupils were equal, round, reactive to light without an afferent pupillary defect. Extraocular muscles were smooth and full without diplopia or pain OU. Amsler Grid was within normal limits OD and revealed mild central metamorphopsia OS. Slit lamp exam was within normal limits. Intraocular pressures were 14 OD and 14 OS by applanation. The patient was dilated with 1% Tropicamide and 2.5% Phenylephrine OU. The lens had trace nuclear sclerotic cataracts OU. The vitreous was clear OU. The Cup to Disc ratio was 0.20 round in OD and OS. The rims were pink and healthy and the margins were distinct OU. The macular exam OD showed one circular area of pigment hyperplasia superior nasal to the macula and one less pigmented hyperplasia inferior nasal to the macula with mild retinal pigment epithelium mottling. The macula of OS revealed two circular areas of pigment hyperplasia adjacent to each other and moderate mottling. The vessels showed mild tortuosity OU. There was one spot of hyperpigmentation in the inferior temporal arcade OS. The periphery appeared flat and did not show any holes, tears or detachments OU. -Studies OCT: (Please see scans attached at the end of this document) OD: Quality: 9/10; Impression: Macula appeared flat and intact. OS: Quality: 9/10; Impression: Revealed a few areas of pigment clumps inferior to the foveal pit Fundus photos: (Please see photos attached at the end of this document) III. Differential diagnosis -Primary/leading Steroid-Induced Chronic Central Serous Chorioretinopathy OU -Others Age Related Macular Degeneration Macular detachment as a result of Rhegmatogenous Retinal Detachment or Macular Hole Optic Pit with Serous Retinal Detachment Choroidal Tumor Pigment Epithelial Detachment Idiopathic Choroidal Effusion Inflammatory Choroidal Disorders IV. Diagnosis and discussion -Elaborate on the condition Central Serous Chorioretinopathy (CSCR) is a retinal disease that causes a serous detachment of the neurosensory retina and/or the retinal pigment epithelium. It often involves the macula (Khairallah, 2012). In CSCR there is a leakage of the choriocapillaris vessels and a retinal pigment epithelium dysfunction. The localized exudation results in retinal edema and small retinal pigment epithelium detachments. These detachments can happen in the macular and the peri-macular area (Katsanos, 2012). A fundus exam along with imaging is used to diagnose CSCR. The imaging will be helpful in identifying subtle findings such as subretinal fluid, shallow pigment epithelial detachments and retinal atrophy. The Optical Coherence Tomography (OCT) is used to highlight these subtle changes. Fluorescein Angiography Page 2 of 9 (FA) highlights the leakage of the fluorescein dye from the choroid through the retinal pigment epithelium. Classically, a smokestack, inkblot, or mushroom shaped appearance of hyperfluorescence is evident on FA. Indocyanine Green Angiography (ICG) can be used to confirm the diagnosis because it shows areas of choroidal hyperpermeability which is not evident on FA. Initially ICG shows a localized delay in arterial filling (hypofluorescence). A midphase hypofluorescence and a late phase washout of dye is evident. Persistent hypofluorescence is seen in the area of retinal pigment epithelium damage (Quin, 2012). -Expound on unique features CSCR occurs more commonly in men than women (Liew, 2012) and occurs between the ages of 25 to 50 (Khairallah, 2012). CSCR has been shown to occur in comparable rates among all ethnic groups (Liew, 2012). The risk factors for CSCR includes Type A personality, stress, pregnancy, Cushing’s Syndrome, adrenocortical adenoma, carcinoma and secondary to exogenous corticosteroid use (Liew, 2012). CSCR can occur with corticosteroid use via oral, topical, epidural, intramuscular, intravenous, intraarticular, intranasal and inhaled administrations (Khairallah, 2012). This case is unique in that the patient developed CSCR as a result of oral prednisone use during renal transplants. V. Treatment, management -Treatment and response to treatment Treatment is not typically indicated for CSCR. Acute CSCR resolves within six months. Patients should be monitored every 6 to 8 weeks to evaluate if the CSCR resolves spontaneously. If not, monitor every 4 to 6 months for resolution. Recurrence of CSCR is common and results in persistent shallow retinal detachments. CSCR can often reoccur within the first year of the primary episode (Ehlers, 2008). Discontinuation of corticosteroid therapy is indicated in steroid-induced CSCR to prevent progression of disease and subsequent visual loss. Studies showed that CSCR improved with discontinuation of steroids. The outcome measures were resolution of serous retinal detachment, resolution of visual symptoms and improvement in visual acuity. In cases of treatment for systemic diseases such as Systemic Lupus Erythematosus (SLE) or post-organ transplantation, long-term corticosteroid therapy is required and cannot be stopped. In these cases, dosage of corticosteroid therapy can be reduced as a treatment for CSCR (Khairallah, 2012). There are a few other alternatives to corticosteroid withdrawal or reduction of dosage of corticosteroid. Another instance when steroid therapy cannot be stopped is myasthenia gravis. Photodynamic Therapy (PDT) is the application of verteporfin, a photosensitizing dye. The verteporfin damages the defective choriocapillaris vessels and stops leakage of fluid. The authors reported that CSCR and visual acuity improved with PDT for the patient with myasthenia gravis. Other treatments to consider when steroids cannot be discontinued include argon laser photocoagulation, transpupillary thermotherapy, intravitreal use of anti-vascular endothelial growth factors or micropulse diode photocoagulation (Katsanos, 2012). The duration and dosage of the prednisone for our patient is unknown as he received care at Dartmouth Hitchcock Medical Center and we do not have prior records at the Veterans Affairs Medical Center. Page 3 of 9 VI. Conclusion -Clinical pearls, take away points if indicated CSCR is a neurosensory detachment of the retina that is more common in middle-aged men The pathophysiology of CSCR includes disorder of retinal pigment epithelium & choroidal circulation The common risk factors are type-A personality, stress, pregnancy, and systemic corticosteroid use Treatment is not typically indicated for CSCR. Patients should be monitored every 6 to 8 weeks to evaluate if the CSCR resolves spontaneously. If not, monitor every 4 to 6 months for resolution. Acute CSCR resolves within 6 months although recurrence is common and results in persistent shallow retinal detachments CSCR can often reoccur within the first year of the primary episode Discontinuation of steroid therapy or using low doses of steroid is the initial treatment in Steroid-Induced CSCR -Refer to research where appropriate/ Bibliography, literature review encouraged [Please see References below] Page 4 of 9 References: 1. Katsanos A, Stefaniotou M. Central serous chorioretinopathy in patients who cannot discontinue steroids. J Crohns Colitis. 2012 Jul 17. [Epub ahead of print] PubMed PMID: 22818165. 2. Khairallah M, Kahloun R, Tugal-Tutkun I. Central serous chorioretinopathy, corticosteroids, and uveitis. Ocul Immunol Inflamm. 2012 Apr;20(2):76-85. Review. PubMed PMID: 22409559. 3. Ehlers JP and Shah CP, eds. (2008). The Wills Eye Manual. Baltimore: Lippincott Williams & Wilkins. 4. Liew G, Quin G, Gillies M, Fraser-Bell S. Central serous chorioretinopathy: a review of epidemiology and pathophysiology. Clin Experiment Ophthalmol. 2012 Jul 12. doi: 10.1111/j.1442-9071.2012.02848.x. [Epub ahead of print] PubMed PMID: 22788735. 5. Quin G, Liew G, Ho IV, Gillies M, Fraser-Bell S. Diagnosis and interventions for central serous chorioretinopathy: review and update. Clin Experiment Ophthalmol. 2012 Jul 12. doi: 10.1111/j.1442-9071.2012.02847.x. [Epub ahead of print] PubMed PMID: 22788713. 6. Sherman, J. Review of Optometry - Retina Revealed. Case 3: Central Serous Chorioretinopathy or Choroidopathy (CSC) 7. Sherman, J. Review of Optometry - Retina Revealed. Case 20: Central Serous Chorioretinopathy Page 5 of 9 Date obtained: 7/16/12 Signal Strength: 9/10 Page 6 of 9 Date obtained: 7/16/12 Signal Strength: 9/10 Page 7 of 9 Fundus Photo: Right Eye (OD) Page 8 of 9 Fundus Photo: Left Eye (OS) Page 9 of 9