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Carrasquillo Outline When lightning strikes twice: Seven Cases of Stevens-­‐Johnson Syndrome and Corneal Ectasia Abstract Corneal vascularization, scarring, thinning, trichiasis and conjunctivalization are often ocular complications of Stevens Johnson syndrome. In cases of SJS and corneal ectasia, ocular management proves to be challenging. We review the course and management of seven cases with concomitant ocular surface disease and corneal ectasia. History o Retrospective medical record review of cases extracted by search on diagnoses of SJS and corneal ectasia on manufacturing database of PROSE devices fabricated from 2002-­‐
2011. o Review of history on causal vs. coincidental relationship between these syndromes was conducted. o All patients were referred to the Boston Foundation for Sight for treatment of their ocular surface disease and for their visual rehabilitation. o All patients had attempted soft lenses, gas permeable lenses, piggy-­‐back lenses, and/or hybrid contact lenses without success; poor tolerance. Pertinent Findings o Seven patients 5F:2M o All with SJS/Corneal Ectasia diagnosis o Average age at SJS diagnosis – 15 years old. o Average age at Ectasia diagnosis – 22 years old. o Most patients were diagnosed with corneal ectasia post SJS. o All patients received PROSE treatment for reduced vision and visual function (13 eyes of 7 patients) o Best corrected visual acuity on presentation ranged from 20/25 to counting fingers (CF) with various modes of correction, including specialty contact lens. o Visual acuity improved in all eyes. All patients achieved driving vision (≥20/40) o Average wear time of PROSE device ranged from 8-­‐16 hours. o Improvement in visual function was demonstrated both subjectively and as measured by NEI VFQ-­‐25 o All patients were fitted successfully with PROSE devices Diagnosis and Discussion o Stevens-­‐Johnson Syndrome can lead to severe ocular complications during chronic stage of disease: • Scarring of conjunctiva • Symblepharon formation Entropion Trichiasis Tear film disturbances Corneal involvement i.
Scarring ii.
Neovascularization iii.
Keratinization iv.
Thinning v.
Conjunctivilization • Lacrimal Duct involvement i.
Cicatrization ii.
Destruction of Goblet Cells • Limbal stem cell deficiency o SJS eyes often develop: • Corneal Opacities • Corneal erosions • Epithelial breakdowns • Ulceration • Infectious Keratitis o Because of limbal stem cell deficiency, SJS eyes are poor candidates for corneal transplants. o Corneal ectasia: • Decreased best corrected visual acuity • Often times spectacle correction or soft lens correction are not viable options • Require specialty contact lenses; rigid gas permeable (GP) lens correction • For highly ectatic and irregular corneas, corneal GP lenses may be unstable • Often if complications with GP lenses, alternative approach is corneal transplants. o Concomitant SJS and corneal ectasia is particularly challenging: • Traditional GP lenses for ectasia visual rehabilitation are poorly tolerated in SJS patients • Option of penetrating keratoplasty is not viable one in concomitant cases of SJS Treatment and Management o Prosthetic replacement of the ocular surface system (PROSE) treatment • Use of scleral prosthetic devices. o These are FDA-­‐approved custom designed and fabricated prosthetic devices: • Restore vision, • Support healing • Reduce symptoms • Improve quality of life for patients suffering with complex corneal disease •
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Large diameter (average 15.5–23 mm) § Designed to rest entirely on the sclera § Vault entirely the cornea § Creating a space filled with preservative-­‐free artificial tears or saline § They are fluid-­‐ventilated § Allow the containment of an oxygenated pool of artificial tears free of air bubbles over the cornea This case: • All patients were custom-­‐fitted with a scleral prosthetic device • Devices were worn on a daily wear schedule • Improved best corrected visual acuity and visual function in all eyes. • Significant decrease in dry eye symptoms, pain, and photophobia. • In five out of seven patients, ectasia developed after SJS onset. • PROSE treatment: § Adequate ocular surface support in refractory disease § Viable and excellent choice for both SJS and corneal ectasia i.
Excellent treatment option for concomitant cases of both complex ocular surface disease and ectasia § Passive and non-­‐invasive treatment ii.
No surgical intervention iii.
Potentially precludes need for further surgical intervention need •
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Conclusion o
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References Patients with corneal ectasia and concomitant ocular surface disease from Stevens-­‐
Johnson syndrome pose a special challenge for visual rehabilitation. Contact lens may be poorly tolerated, difficult to fit, and penetrating keratoplasty is high risk. PROSE treatment is a useful option for patients such as these with complex corneal disease. There is not enough data in the literature to confirm a causal vs. coincidental relationship between SJS and corneal ectasia. The fact that 5 out of the 7 patients in our series were diagnosed with ectasia some time after SJS diagnosis may suggest that severe ocular surface microtrauma maybe a factor in developing ectasia. More data is needed to further conclude on a causal vs. coincidental relationship between SJS and corneal ectasia. 1. Rosenthal P, Croteau A. Fluid-­‐ventilated, gas permeable Scleral contact lens is an effective option for managing severe ocular surface disease and many corneal disorders that would otherwise require penetrating keratoplasty. Eye & Contact Lens. 2005; 31(3) 130-­‐134. 2. Mangione C, Lee P, Gutierrez P, Spritzer K, Berry S, Hays R. Development of the 25-­‐item National Eye Institute Visual Function Questionnaire (VFQ-­‐25). Arch Ophthalmol 2001; 119:1050 –1058. 3. Romero-­‐Rangel T, Stavrou P, Cotter J, Rosenthal P, Baltatzis S, Foster CS. Gas-­‐permeable scleral contact lens therapy in ocular surface disease. Am J Ophthalmol. Jul 2000;130(1):25-­‐32. 4. Stason WB, Razavi M, Jacobs DS, Shepard DS, Suaya JA, Johns L, Rosenthal P. Clinical Benefits of the Boston Ocular Surface Prosthesis. Am J Ophthalmol. 2010; 54-­‐61. 5. Sunita Agarwal, Athiya Agarwal, David J Apple (2002) Textbook of Ophthalmology; Jaypee Brothers Publishers, pg. 872. Dr. Saurin Patel, O.D. and Dr. Sonal Patel, O.D. James J. Howard VA Eye Clinic Abstract: Despite rare indications for prosthetic iris implantations, there are complications that need prompt recognition and treatment. This case depicts complications of the procedure, devastating course of disease, and importance of patient education on proper management and treatment. I.
Case History A. 32 YO white female presents for first time at VA eye clinic in Chicago 1. Chief complaint is dissatisfaction of color of cosmetic iris implants, dryness, and glare at night. B. Past ocular history: 1. Bilateral PRK in 2005 2. Bilateral dry eyes 3. Bilateral iris implants C. Past medical history/social history: 1. Anxiety 2. Hyperlipidemia 3. Liposuction in face 4. Breast implants 5. Rhinoplasty 6. Eyebrow lift 7. Lip surgery D. Medications: 1. Simvastatin 2. Clonazepam II. E. Other Salient Information 1. Allergy to codeine with reaction of emesis Pertinent Findings A.
Clinical 1. Visual Acuities (without correction) a. OD: 20/20 b. OS: 20/20 2. Direct pupillary response OD, however, pupil reaction OS could not be determined due to blockage of pupillary frill from implant. Consensual pupillary response and presence or absence of afferent pupillary defect could not be assessed. 3. EOMs: NL, CVF: Full to Finger Count OD/ OS 4. Cornea: tr SPK OU, 3+ dispersed endothelial pigment and epithelial scars OU. Anterior chamber: occasional pigment cell OU. The patient’s true iris was 5.
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B. C. III. minimally visible behind the green intraocular implant of artificial iris OD, but not visible at all OS IOP: OD 28mmHg, OS 28mmHg at 9:05am by applanation Lens: clear to extent seen OU Dilated Fundus Exam: a. OD: CD: 0.3 b. OS: CD: 0.3 Pachymetry: OD: 472um, OS: 485um. Gonioscopy: Ciliary body in all quadrants with significant pigment in the trabecular meshwork with areas of implant obstructing views OU HVF: OD: full, OS: full Baseline corneal topography and anterior segment photos taken. Physical 1. BP: 120/78 right arm sitting. 2. Pulse 69 bpm; strong, regular Lab work including CBC, thyroid, and lipids: NL on medication Differential Diagnosis A. Primary/Leading – Ocular hypertension OU secondary to cosmetic eye surgery IV. Diagnosis and Discussion A. Dry eyes OU s/p PRK OU B. Ocular Hypertension s/p intraocular implant of artificial iris OU. Thin CCT, but NL VF OU. C. Anterior nongranulamatous iritis OU s/p intraocular implant of artifical iris D. The reason for adverse effects in implants anterior to the iris is likely directly related to chronic inflammation, pigment dispersion, and compression of the trabecular meshwork by the implant and indirectly to chronic steroid use. E. Indications for iris implants, despite there being no FDA approval, include trauma, post-­‐
excision of iris melanoma, iris coloboma, congenital aniridia, iridocorneal-­‐endothelial syndrome and other inflammatory causes of iris damage F. Risks included intraoperative development of hyphema, suprachoroidal hemorrhage and retinal detachment, and post-­‐operative cataract development, IOP increase, corneal edema/decompensation, uveitis, or endophthalmitis. G. Explantation should be performed at the most earliest sign of any complication V. Treatment Management A. AT QID OU B. Begin Travatan qbedtime OU C. Begin Brimonidine bid OU D. Pt educated to have cosmetic iris implants explantation E. Bibliography 1. Avliffe W., Groth, Sponsel, WE. “Small-­‐incision insertion of artificial iris prostheses. J Cataract Refract Surg. 2012 Feb;38(2):362-­‐7. 2. Hoquet A., Ritterband D., et al. “Serious ocular complications of cosmetic iris implants in 14 eyes.” J Cataract Refract Surg. 2012 Mar;38(3):387-­‐93. Epub 2012 Jan 11. 3. Mamalis N. “Cosmetic Iris Implants” J Cataract Refract Surg. 2012 Mar;38(3):383. 4. Garcia-­‐Pous M., et. al., “Acute endothelial failure after cosmetic iris implants (NewIris®).” Clin Ophthalmol. 2011;5:721-­‐3. Epub 2011 May 26. 5. Snyder ME, Osterholzer E., “Capsular tension segments in repositioning capsular bag complex containing an intraocular lens and iris prosthesis” J Cataract Refract Surg. 2012 Mar;38(3):551-­‐2. 6. George MK, Tsai JC, Loewen NA., “Bilateral irreversible severe vision loss from cosmetic iris implants.” Am J Ophthalmol. 2011 May;151(5):872-­‐875.e1. Epub 2011 Feb 18. 7. Castanera F, Fuentes-­‐Páez G, Ten P, Pinalla B, Guevara O., “Scanning electron microscopy of explanted cosmetic iris implants.” Clin Experiment Ophthalmol. 2010 Aug;38(6):648-­‐51. Epub 2010 Apr 29. 8. Arthur SN, Wright MM, Kramarevsky N, Kaufman SC, Grajewski AL, “Uveitis-­‐glaucoma-­‐
hyphema syndrome and corneal decompensation in association with cosmetic iris implants.” Am J Ophthalmol. 2009 Nov;148(5):790-­‐3. Epub 2009 Aug 5. 9. Kohnen S., “Reconstructive iris surgery: implants and techniques” Ophthalmologe. 2011 Aug;108(8):709. 10. Anderson JE, Grippo TM, Sbeity Z, Ritch R, “Serious complications of cosmetic NewColorIris implantation.” Acta Ophthalmol. 2010 Sep;88(6):700-­‐4. doi: 10.1111/j.1755-­‐
3768.2008.01499.x. 11. Hull S, Jayaram H, Mearza AA, “Complications and management of cosmetic anterior chamber iris implants.” Cont Lens Anterior Eye. 2010 Oct;33(5):235-­‐8. Epub 2010 Apr 9. 12. Menezo JL, Martínez-­‐Costa R, Cisneros A, Desco MC., “Implantation of iris devices in congenital and traumatic aniridias: surgery solutions and complications.” Eur J Ophthalmol. 2005 Jul-­‐Aug;15(4):451-­‐7. 13. Ozturk F, Osher RH, Osher JM. “Secondary prosthetic iris implantation following traumatic total aniridia and pseudophakia.” J Cataract Refract Surg. 2006 Nov;32(11):1968-­‐
70. 14. Burk SE, Da Mata AP, Snyder ME, Cionni RJ, Cohen JS, Osher RH, “Prosthetic iris implantation for congenital, traumatic, or functional iris deficiencies.” J Cataract Refract Surg. 2001 Nov;27(11):1732-­‐40. 15. Thiagalingam S, Tarongoy P, Hamrah P, Lobo AM, et. al., “Complications of cosmetic iris implants.” J Cataract Refract Surg. 2008 Jul;34(7):1222-­‐4. 16. Ngoei, Enette., “A suitable Iris”, October 2010 http://www.eyeworld.org/article.php?sid=5602&strict=&morphologic=&query=iris%20impl
ant 17. Lipner, Maxine, “Improving aniridia with iris matching”, The Eye World August 2009 http://www.eyeworld.org/article.php?sid=5059&strict=&morphologic=&query=iris%20impl
ant 18. George, Mathew K., Tsai, James C., Loewen, Nils A., “Bilateral Irreversible Severe Vision Loss From Cosmetic Iris Implants” Department of Ophthalmology and Visual Science, Yale University School of Medicine, New Haven, Connecticut. Accepted 11 November 2010. Available online 18 February 2011. 19. Arthurs, Stella N., Wright, Martha M., “Uveitis-­‐Glaucoma-­‐Hymphema Syndrome and Corneal Decompensation in Association with Cosmetic Iris Implants., Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota. Department of Ophthalmology, Bascom Palmer Eye Institute, Miami, Florida. Accepted 1 June 2009. Available online 5 August 2009. VI. 20. C.Y.W. Khng and M.E. Snyder., “LE216 functional and cosmetic outcomes after artificial, iris implantation” The Eye Institute, Tan Tock Seng Hospital, Singapore. Cincinnati Eye Institute, Cincinnati, Ohio, USA 21. Blackmon, Douglas M. and Lambert, Scott R., “Congenital iris coloboma repair using a modified McCannel suture technique” Emory Eye Center, Emory University, Atlanta, Georgia., USA. Accepted 12 November 2002. Available online 21 April 2003. 22. Arundhati, Anshu, et. al, “Iris Reconstruction in Penetrating Keratoplasty—Surgical Techniques and a Case-­‐control Study to Evaluate Effect on Graft Survival”, Singapore National Eye Centre, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore Eye Research Institute, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Department of Ophthalmology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Accepted 2 October 2007. Available online 25 January 2008. 23. Gillette, Bill, “Syrian Medical Authorities Skeptical About Iris Implant”, Cosmetic Surgery Times, Feb 10, 2010. 24. Thiagalingam, S, Tarongoy P, Hamrah P, Lobo AM, Nagao K, Barsam C, Bellows R, Pineda. Complications of Cosmetic Iris Implants. Journal of Cataract and Refractive Surgery. 2008 July; 34(7):1222-­‐1224. 25. Hoguet A, Ritterband D, Koplin R, Wu E, Raviv T, Aljian J, Seedor J. Serious ocular complications of cosmetic iris implants in 14 eyes. Journal of Cataract and Refractive Surgery. 2012 March; 38(3):387-­‐93. Epub 2012 Jan 11 Conclusion A. Although iris prosthetic devices do provide benefits, they can and do have destructive results. Close and regular follow up is important to avoid such complications. As with any medical procedure, risks and benefits should be properly assessed. Depending on the reason for treatment, iris implant risks may outweigh benefits, especially in cases for cosmetic purposes given the serious complications of the implant and removal of implant. However, in contrast, a patient with severe symptoms secondary to congenital or acquired defects the benefits may outweigh the risks. This case portrays a young patient who had healthy eyes that rapidly changed to diseased eyes as result of opting for cosmetic iris implants. Patient education continues to be essential on proper treatment and management. AAO 2013 Choroidal Retinal Lesion in a Patient with History of Prostate Cancer (A New Meaning to Flashes) Annie Wan Case Report A 73 year old Caucasian male presents for a routine glaucoma examination, but reports symptoms of a persistent flash in his left eye. Clinical exam reveals a choroidal lesion suspicious for melanoma or metastasis. Case History 8/2010 70yo WM presents in August 2010 for initial examination with no ocular or visual complaints. His last reported dilation was more than 40 years ago at which time he was told there was a spot in the back of his eye. He denies any family history of ocular conditions, history of trauma, or ocular surgery. Hypertension since 2002: Hydrochlorothiazide, Lisinopril (132/70, rate 76) Diabetes Mellitus: Metoprolol Hypercholesterolemia: Simvastatin Low compound hyperopic prescription with best corrected vision of 20/20 OD, OS. Pupils, ocular motilities, confrontation fields, and Amsler grid tests were unremarkable. Anterior segment evaluation showed slight capped meibomian glands and mild cataracts OU. Intraocular pressures were measured to be 16mmHg OD and 17mmHg OS. Dilated exam revealed moderate cupping of the discs 0.65 OD and 0.60 OS, moderately attenuated blood vessels with few crossing changes, and a 3.5 disc diameter flat choroidal nevus located superonasal OD. All other findings were unremarkable. Patient was diagnosed with blepharitis, mild hypertensive retinopathy, moderate optic nerve cupping, early cataracts, and choroidal nevus OD. The patient to return in 3 months for baseline visual fields. 9/2011 71yo: CC itchy/tearing eyes and symptomatic relief with Visine Recently diagnosed and undergoing radiation treatment for prostate cancer since July 2011. Pain: Acetaminophen, Ibuprofen Gout: Allopurinol HTN: Amlodipine, Hydrochlorothiazide, Lisinopril (116/78, rate 64) GERD: Omeprazole CHL: Simvastatin Prostate: Terazosin, Warfarin Inactive: Bicalutamide, Colon elec lavage, fentanyl citrate injection, midazolam IV injection Intradermal injection: Enoxaparin, Goserelin Intraocular pressures were 19/18 with cupping being noted as 0.70 OD, 0.75 OS. Choroidal nevus OD was stable. Return in 3 months for visual fields, glaucoma work up. 12/2011 72yo WM Humphrey visual field 12/2011 was reliable and full OD, reliable with superior arcuate defect OS not noted from 2010. Gonioscopy revealed open angles. Corneal thickness was average (544/540). Diagnosed with primary open angle glaucoma, started with travatan qPM OU, and return in 3 months for repeated fields. Last measured IOP 14/14. Choroidal Retinal Lesion in a Patient with History of Prostate Cancer (A New Meaning to Flashes) Annie Wan AAO 2013 2 8/2012 72yo WM, Ran out of travatan x 3 weeks. Last visual fields (5/2012) was stable OU. Pain: Acetaminophen, Ibuprofen Gout: Allopurinol HTN: Amlodipine, Hydrochlorothiazide, Lisinopril, metoprolol (130/78, rate 66) GERD: Omeprazole CHL: Simvastatin Prostate: Terazosin, Warfarin, Tamsulosin Inactive: Bicalutamide, Colon elec lavage, fentanyl citrate injection, midazolam IV injection Intradermal injection: Enoxaparin, Goserelin IOP 15/16 Choroidal nevus OD is slightly elevated on clinical observation. Return in 3 months for pressures. 12/2012 73yo WM, POAG returning for pressure check, travatan qPM OU with good compliance Reports “light bulb” inferiorly OS for 1 month. Denies floaters/curtains. IOP 18/19, C/D 0.70 thin sup OD, 0.75 thin inf OS OD: Choroidal nevus flat sup OS: 4DD choroidal lesion (+)drusen, elevated, pigmentary changes B-­‐scan shows small elevation with minimal internal reflectivity FA confirms early hyperfluorescense that does not change in the late stages. (+)allergy to fluorescein Referral to outside ocular oncologist immediately. IMAGES 2012: OD NEVUS OS CHOROIDAL LESION Differential Diagnosis -­‐ Elaborate on condition o Choroidal melanoma is most common primary intraocular malignant neoplasm in adults § 88% choroidal, 12% ciliary body/iris o Cancer that arises from melanocytes § Melanoma 4% skin cancers, 79% skin cancer related deaths Choroidal Retinal Lesion in a Patient with History of Prostate Cancer (A New Meaning to Flashes) Annie Wan AAO 2013 3 o Secondary cancers: 5yr ~7.7% rate, most common prostate (23%), breast (17%) o 6% prostate cancers metastasize to the choroid o 50% mortality rate § 33% from melanoma metastasis, 29% malignant tumor other than metastatic, 11% malignancy of unknown origin § Median survival for stage 4 ~9mos, and 3yr survival <15% o Poor prognosis 70-­‐79yo, males>women 3:2 >80yo o Diagnosis/Staging scales § LUMPO § TNM – AJCC and UICC § Kaplan-­‐Meier analysis o Early diagnosis = surgical resection (80% effective for thin lesions) o Other presentations: Subfoveal melanoma à Tx plaque brachytherapy à
-­‐ Expound on unique features o How does cancer develop? Cell proliferation à Lack of response to normal apoptosis à genetic alterations à translocations/mutations à loss/gain of function o Risk of metastasis depends on several factors: gene expression, basal tumor diameter, tumor thickness, ciliary body/extraocular muscle involvement, melanoma cytomorphology, etc. -­‐ Diagnostic tests o Transcleral fine needle aspiration biopsy § Cell culture: cell lines, blood DNA o B scan Ultrasonography o Fluorescein Angiography o Indocyanine Green Angiography Treatment/Management -­‐ Treatment o Enucleation o External proton beam radiation o Transcleral local resection o Endoresection o Transpupillary thermotherapy o Plaque radiation therapy o Iodine Brachytherapy o Androgen deprivation therapy: Gonadotropin-­‐releasing hormone agonist (Leuprolide acetate) o Intralesional injection BCG/interferon/interleukin 2 (stage 3) § Dacarbazine, Temozolomide, high dose IL2, and paclitaxel (stage 4) o Alternative Photodynamic therapy (PDT): induce direct tumor cell photodamage, destruction of tumor vasculature and activation of an immune response § Photosensitizing agent + illumination of the tumor with visible light § Apoptosis =controlled, energy consuming process of suicidal cell death ·∙ Mitochondria mediated/intrinsic pathway ·∙ Death receptor-­‐mediated/extrinsic pathway § Necrosis Choroidal Retinal Lesion in a Patient with History of Prostate Cancer (A New Meaning to Flashes) Annie Wan AAO 2013 4 § Protective mechanisms against therapy: pigmentation and increased oxidative stress defense -­‐ Response to treatment o Visual symptoms improve significantly depending on location Conclusion -­‐ Clinical Pearls o Lesions present when you least expect them o Prompt referral important o Ancillary tests like OCT serves a valuable tool o Discussion about prognosis important o Most aggressive treatment may not be the best o Other malignancies may occur Bibliography 1. Burgess BL, Rao NP, Eskin A, Nelson SF, and McCannel TA. Characterization of three cell lines derived from fine needle biopsy of choroidal melanoma with metastatic outcome. Molecular Vision 2011;17:607-­‐15. 2. Baldea I and Filip AG. Photodynamic therapy in melanoma – an update. Journal of Physiology and Pharmacology 2012;63(2):109-­‐18. 3. Ameri H, Araujo JC, and Gombos DS. Leuprolide monotherapy for choroidal metastasis from prostate adenocarcinoma. Arch Ophthalmol 2012;130(9):1225-­‐6. 4. Sallet G, Amoaku WMK, Lafaut BA, Branbant P, and De Laey JJ. Indocyanine green angiography of choroidal tumors. Graefe’s Arch Clin Exp Ophthalmol 1995;233:677-­‐89. 5. Torres VLL, Brugnoni N, Kaiser PK, and Singh AD. Optical coherence tomography enhanced depth imaging of choroidal tumors. Am J Ophtalmol 2011;151:586-­‐93. 6. Keenan TDL, Yeates D, and Goldacre MJ. Uveal melanoma in England: trends over time and geographical variation. Br J Ophthalmol 2012;96:1415-­‐9. 7. Damato B, Eleuteri A, Taktak AFG, and Coupland SE. Estimating prognosis for survival after treatment of choroidal melanoma. Progress in Retinal and Eye Research 2011;30:285-­‐95. 8. Collaborative Ocular Melanoma Study Group (COMS). Second primary cancers after enrollment in the COMS trials for treatment of choroidal melanoma. COMS report no. 25. Arch Ophthalmol. 2005;123:601-­‐4.