CONSULTATION SECTION
more useful to us than we were to them. In other words, these almost artistic ring opacities might just be a rare coincidental finding not requiring medical attention.1,A,B (The reduced visual acuity in the left eye in this case is probably explained by the macular hole.) That being said, this case and earlier similar cases could still be of great scientific interest for ophthalmology. Such rings could be the result of an anatomic stromal defect or of refractive surgery; however, these possibilities are less likely because the corneal tissue did not show defects and all patients denied a history of corneal surgery or trauma. It would stand to reason that the rings relate to some type of gradient across the cornea that entails symmetry (thus producing perfect circles) as well as demarcation properties from the limbus to apex (cornea clear both centrally and peripherally from the depositions). If so, this observation could inform our understanding of the corneal physiology and its associated disorders. Gerrit R.J. Melles, MD, PhD Rotterdam, the Netherlands Financial Disclosure: Dr. Melles is a consultant to D.O.R.C. International BV/Dutch Ophthalmic USA and Surgicube International.
some thoughts regarding the description, etiology, additional testing, and management. There is a perfectly circular ring opacity with a small linear opacity extending superotemporally and a mild, diffuse, anterior corneal haze central and peripheral to the ring. Because of the perfectly circular shape and the apparent normal examination previously, it is extremely unlikely that the ring opacity is caused by an inherent or purely biological condition. One could look at family members to explore this possibility further. The ring's shape suggests some type of lightor laser-induced opacity; however, the uniformity of the opacity over its entire depth argues against this suggestion. Another possibility is that it is a lesion caused in some way by the tonometer tip. A final option is that it is a self-inflicted lesion. Additional testing could help in understanding the nature of these rings. In performing corneal topography and tomography, one should see central steepening if the lesion is thermal. Specular micrography could determine whether there is evidence of injury to the endothelium that could cause these opacities. In managing the case, I would observe and then perform a follow-up examination in 1 to 2 months. Douglas D. Koch, MD Houston, Texas, USA
REFERENCES ra JP, Eggink CA, Cruysberg JRM, Binder PS. 1. Melles GRJ, de Se Bilateral, anterior stromal ring opacity of the cornea. Br J Ophthalmol 1998; 82:522–525. Available at: http://www.ncbi.nlm.nih.gov/pmc/ articles/PMC1722592/pdf/v082p00522.pdf. Accessed February 23, 2015 2. Bron AJ. Peripheral ring opacity of the cornea. Br J Ophthalmol 1969; 53:270–273. Available at: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC1207311/pdf/brjopthal00328-0056.pdf. Accessed February 23, 2015 3. Caroline PJ, Melles GRJ. Two cases of bilateral, stromal ring opacity of the cornea. Cornea 2001; 20:237–238
OTHER CITED MATERIAL A. Winnick J, “Bilateral Corneal Ring Opacity Found in Long-Term Contact Lens Wearer,” poster presented at the annual meeting of the American Academy of Optometry, San Francisco, California, USA, December 1998. Abstract available at: http://www.aaopt. org/bilateral-corneal-ring-opacity-found-long-term-contact-lenswearer. Accessed February 23, 2015 B. Barnes D, Volkert T, Dunphy R, Selvin G, Lambert N, “Bilateral Ring-Shaped Corneal Opacities,” poster presented at the annual meeting of the American Academy of Optometry, San Diego, California, USA, December 2002. Abstract available at: http:// www.aaopt.org/bilateral-ring-shaped-corneal-opacities. Accessed February 23, 2015
- First, I want to acknowledge that I am fairly clueless. I have never seen anything like this (nor have 4 of my colleagues at Baylor University). Here are
897
- This 63-year-old woman presented with decreased vision in the left eye, which was referred for evaluation of bilateral corneal opacities noted by a retinal surgeon. The slitlamp photographs show bilateral white rings that are remarkably thin, circular, and symmetric between the 2 eyes and approximately 4.0 mm in diameter. The left eye otherwise appears uninflamed and to have a smooth contour and a clear central cornea. The thin-beam slitlamp and AS-OCT images confirm the full-thickness stromal location and the absence of significant corneal thickening or previous surgical scars. The rings likely have minimum clinical significance because they are symmetric and the vision in the right eye is correctable to 20/20, confirming to a retinal surgeon that the origin of the vision limitation is largely retinal, not corneal. The differential diagnosis can be broadly any disorder that can lead to opacification; however these thin, symmetric, midperipheral, circular lesions are characteristic of a presumably idiopathic corneal dystrophy that has been described periodically since it was first reported in 1964.1 Melles et al.2 reviewed a collection of 6 cases that shared the same appearance but varied from patient to patient in terms of size and age at presentation (20s to elderly). Over the years, these opacities have presented as an incidental
J CATARACT REFRACT SURG - VOL 41, APRIL 2015
898
CONSULTATION SECTION
finding, like with the patient presently being discussed, without visual alterations and with no progression when followed for many years. To my knowledge, they have not been related to any systemic disorders and are of no clinically significant consequence. Although the appearance is quite characteristic, other sources of corneal opacity should be considered and tested for if there are related symptoms suggestive of an alternative diagnosis. None of the drugs currently listed is commonly associated with this type of stromal opacity. Signs of inflammation should raise the possibility of a herpetic or even parasitic etiology with a Wessely-type ring immunologic reaction. A history of previous corneal surgery, especially LASIK or stromal implants, seems unlikely with an otherwise normal-appearing AS-OCT. Schnyder crystalline corneal dystrophy should always be considered in roughly ring-like, white opacities and should prompt a detailed lipid profile and genetic testing. Immunoglobulin deposition as part of a multiple myeloma should prompt a serum protein electrophoresis and urinalysis. Barring the elicitation of other history, signs, or symptoms of systemic disease besides high blood pressure and normal hyperlipidemia, further testing is probably not necessary, which also obviates the need for medical intervention at this time. Elmer Tu, MD Chicago, Illinois, USA
REFERENCES € hnliche Hornhautringe [Unusual corneal 1. Ascher KW. Ungewo ring]. Dtsch Ophthalmol Ges 1964; 65:44–46 ra JP, Eggink CA, Cruysberg JRM, Binder PS. 2. Melles GRJ, de Se Bilateral, anterior stromal ring opacity of the cornea. Br J Ophthalmol 1998; 82:522–525. Available at: http://www.ncbi.nlm.nih. gov/pmc/articles/PMC1722592/pdf/v082p00522.pdf. Accessed February 25, 2015
- This 63-year-old woman was incidentally found to have bilateral, sharply outlined, midperipheral corneal opacities that are almost perfectly circular. Several cases of ring-shaped opacities in the midperipheral corneal stroma have been reported in the literature. This condition was first described by Ascher et al. in 1964 as a bilateral, gray–white, sharply demarcated ring in the midperipheral cornea of a 39-year-old man with history of unilateral iritis.1 In that case, the condition was located in the superficial stroma and was 7.0 mm in diameter. In 1969, Bron et al. described a 59-year-old man and a 66-year-old woman with similar opacities in the midperiphery of the cornea that were wedge-shaped, dense, and based in the Descemet membrane.2 Other reported
bilateral ring opacities were 4.0 to 5.0 mm in diameter and extended halfway down the stroma.3,4 Melles et al.5 described a 25-year-old man who had been followed since age 11 and had developed the bilateral opacities over time. Caroline and Melles6 described 2 cases of similar bilateral opacities unassociated with medications and systemic disorders. Other cases appeared similar, with imaging that showed fullthickness stromal involvement.7 In patients with no history of ocular inflammation or systemic autoimmune disease, it has been speculated that these acquired corneal opacities originated from depositions of unknown material. Proposed causes for these depositions include topical or systemic drugs, metabolic disorders, and corneal dystrophies. In this patient, the opacities likely have no visual impact because the decreased vision in the left eye can be explained by the full-thickness macular hole and the corneal opacities are outside the visual axis. My diagnosis would be Ascher intrastromal corneal ring opacity, which has no reported association with systemic medications or disorders. However, I also would consider a workup for hyperlipidemia and mucopolysaccharidosis, and I would examine the patient's family members for the possibility of a new dystrophy diagnosis. Although some earlier cases did not find affected family members, this condition is rare and much still is not known about it. My examination would also include corneal sensitivity, corneal pachymetry, topography, gonioscopy, and serial examinations. I have followed a patient with similar bilateral corneal stromal rings; over time, that patient's eyes developed corneal endothelial decompensation and edema. I would examine the patient in the present case initially every 3 months and then every 6 months. Natalie Afshari, MD San Diego, California, USA
REFERENCES €hnliche Hornhautringe [Unusual corneal 1. Ascher KW. Ungewo ring]. Dtsch Ophthalmol Ges 1964; 65:44–46 2. Bron AJ. Peripheral ring opacity of the cornea. Br J Ophthalmol 1969; 53:270–273. Available at: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC1207311/pdf/brjopthal00328-0056.pdf. Accessed February 23, 2015 €rmige Hornhauttru € bung [Paracen3. Laqua H. Parazentrale ringfo tral ring-like corneal opacity]. Dtsch Ophthalmol Ges 1972; 71:583–585 €rmige 4. Bopp S, Laqua H. Kornealer Ascher-Ring - Eine ringfo € bung [Corneal Ascher ring - a ring-shaped stromale Hornhauttru stromal corneal opacity]. Klin Monatsbl Augenheilkd 1991; 198:201–204 ra JP, Eggink CA, Cruysberg JRM, Binder PS. 5. Melles GRJ, de Se Bilateral, anterior stromal ring opacity of the cornea. Br
J CATARACT REFRACT SURG - VOL 41, APRIL 2015
more useful to us than we were to them. In other words, these almost artistic ring opacities might just be a rare coincidental finding not requiring medical attention.1,A,B (The reduced visual acuity in the left eye in this case is probably explained by the macular hole.) That being said, this case and earlier similar cases could still be of great scientific interest for ophthalmology. Such rings could be the result of an anatomic stromal defect or of refractive surgery; however, these possibilities are less likely because the corneal tissue did not show defects and all patients denied a history of corneal surgery or trauma. It would stand to reason that the rings relate to some type of gradient across the cornea that entails symmetry (thus producing perfect circles) as well as demarcation properties from the limbus to apex (cornea clear both centrally and peripherally from the depositions). If so, this observation could inform our understanding of the corneal physiology and its associated disorders. Gerrit R.J. Melles, MD, PhD Rotterdam, the Netherlands Financial Disclosure: Dr. Melles is a consultant to D.O.R.C. International BV/Dutch Ophthalmic USA and Surgicube International.
some thoughts regarding the description, etiology, additional testing, and management. There is a perfectly circular ring opacity with a small linear opacity extending superotemporally and a mild, diffuse, anterior corneal haze central and peripheral to the ring. Because of the perfectly circular shape and the apparent normal examination previously, it is extremely unlikely that the ring opacity is caused by an inherent or purely biological condition. One could look at family members to explore this possibility further. The ring's shape suggests some type of lightor laser-induced opacity; however, the uniformity of the opacity over its entire depth argues against this suggestion. Another possibility is that it is a lesion caused in some way by the tonometer tip. A final option is that it is a self-inflicted lesion. Additional testing could help in understanding the nature of these rings. In performing corneal topography and tomography, one should see central steepening if the lesion is thermal. Specular micrography could determine whether there is evidence of injury to the endothelium that could cause these opacities. In managing the case, I would observe and then perform a follow-up examination in 1 to 2 months. Douglas D. Koch, MD Houston, Texas, USA
REFERENCES ra JP, Eggink CA, Cruysberg JRM, Binder PS. 1. Melles GRJ, de Se Bilateral, anterior stromal ring opacity of the cornea. Br J Ophthalmol 1998; 82:522–525. Available at: http://www.ncbi.nlm.nih.gov/pmc/ articles/PMC1722592/pdf/v082p00522.pdf. Accessed February 23, 2015 2. Bron AJ. Peripheral ring opacity of the cornea. Br J Ophthalmol 1969; 53:270–273. Available at: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC1207311/pdf/brjopthal00328-0056.pdf. Accessed February 23, 2015 3. Caroline PJ, Melles GRJ. Two cases of bilateral, stromal ring opacity of the cornea. Cornea 2001; 20:237–238
OTHER CITED MATERIAL A. Winnick J, “Bilateral Corneal Ring Opacity Found in Long-Term Contact Lens Wearer,” poster presented at the annual meeting of the American Academy of Optometry, San Francisco, California, USA, December 1998. Abstract available at: http://www.aaopt. org/bilateral-corneal-ring-opacity-found-long-term-contact-lenswearer. Accessed February 23, 2015 B. Barnes D, Volkert T, Dunphy R, Selvin G, Lambert N, “Bilateral Ring-Shaped Corneal Opacities,” poster presented at the annual meeting of the American Academy of Optometry, San Diego, California, USA, December 2002. Abstract available at: http:// www.aaopt.org/bilateral-ring-shaped-corneal-opacities. Accessed February 23, 2015
- First, I want to acknowledge that I am fairly clueless. I have never seen anything like this (nor have 4 of my colleagues at Baylor University). Here are
897
- This 63-year-old woman presented with decreased vision in the left eye, which was referred for evaluation of bilateral corneal opacities noted by a retinal surgeon. The slitlamp photographs show bilateral white rings that are remarkably thin, circular, and symmetric between the 2 eyes and approximately 4.0 mm in diameter. The left eye otherwise appears uninflamed and to have a smooth contour and a clear central cornea. The thin-beam slitlamp and AS-OCT images confirm the full-thickness stromal location and the absence of significant corneal thickening or previous surgical scars. The rings likely have minimum clinical significance because they are symmetric and the vision in the right eye is correctable to 20/20, confirming to a retinal surgeon that the origin of the vision limitation is largely retinal, not corneal. The differential diagnosis can be broadly any disorder that can lead to opacification; however these thin, symmetric, midperipheral, circular lesions are characteristic of a presumably idiopathic corneal dystrophy that has been described periodically since it was first reported in 1964.1 Melles et al.2 reviewed a collection of 6 cases that shared the same appearance but varied from patient to patient in terms of size and age at presentation (20s to elderly). Over the years, these opacities have presented as an incidental
J CATARACT REFRACT SURG - VOL 41, APRIL 2015
898
CONSULTATION SECTION
finding, like with the patient presently being discussed, without visual alterations and with no progression when followed for many years. To my knowledge, they have not been related to any systemic disorders and are of no clinically significant consequence. Although the appearance is quite characteristic, other sources of corneal opacity should be considered and tested for if there are related symptoms suggestive of an alternative diagnosis. None of the drugs currently listed is commonly associated with this type of stromal opacity. Signs of inflammation should raise the possibility of a herpetic or even parasitic etiology with a Wessely-type ring immunologic reaction. A history of previous corneal surgery, especially LASIK or stromal implants, seems unlikely with an otherwise normal-appearing AS-OCT. Schnyder crystalline corneal dystrophy should always be considered in roughly ring-like, white opacities and should prompt a detailed lipid profile and genetic testing. Immunoglobulin deposition as part of a multiple myeloma should prompt a serum protein electrophoresis and urinalysis. Barring the elicitation of other history, signs, or symptoms of systemic disease besides high blood pressure and normal hyperlipidemia, further testing is probably not necessary, which also obviates the need for medical intervention at this time. Elmer Tu, MD Chicago, Illinois, USA
REFERENCES € hnliche Hornhautringe [Unusual corneal 1. Ascher KW. Ungewo ring]. Dtsch Ophthalmol Ges 1964; 65:44–46 ra JP, Eggink CA, Cruysberg JRM, Binder PS. 2. Melles GRJ, de Se Bilateral, anterior stromal ring opacity of the cornea. Br J Ophthalmol 1998; 82:522–525. Available at: http://www.ncbi.nlm.nih. gov/pmc/articles/PMC1722592/pdf/v082p00522.pdf. Accessed February 25, 2015
- This 63-year-old woman was incidentally found to have bilateral, sharply outlined, midperipheral corneal opacities that are almost perfectly circular. Several cases of ring-shaped opacities in the midperipheral corneal stroma have been reported in the literature. This condition was first described by Ascher et al. in 1964 as a bilateral, gray–white, sharply demarcated ring in the midperipheral cornea of a 39-year-old man with history of unilateral iritis.1 In that case, the condition was located in the superficial stroma and was 7.0 mm in diameter. In 1969, Bron et al. described a 59-year-old man and a 66-year-old woman with similar opacities in the midperiphery of the cornea that were wedge-shaped, dense, and based in the Descemet membrane.2 Other reported
bilateral ring opacities were 4.0 to 5.0 mm in diameter and extended halfway down the stroma.3,4 Melles et al.5 described a 25-year-old man who had been followed since age 11 and had developed the bilateral opacities over time. Caroline and Melles6 described 2 cases of similar bilateral opacities unassociated with medications and systemic disorders. Other cases appeared similar, with imaging that showed fullthickness stromal involvement.7 In patients with no history of ocular inflammation or systemic autoimmune disease, it has been speculated that these acquired corneal opacities originated from depositions of unknown material. Proposed causes for these depositions include topical or systemic drugs, metabolic disorders, and corneal dystrophies. In this patient, the opacities likely have no visual impact because the decreased vision in the left eye can be explained by the full-thickness macular hole and the corneal opacities are outside the visual axis. My diagnosis would be Ascher intrastromal corneal ring opacity, which has no reported association with systemic medications or disorders. However, I also would consider a workup for hyperlipidemia and mucopolysaccharidosis, and I would examine the patient's family members for the possibility of a new dystrophy diagnosis. Although some earlier cases did not find affected family members, this condition is rare and much still is not known about it. My examination would also include corneal sensitivity, corneal pachymetry, topography, gonioscopy, and serial examinations. I have followed a patient with similar bilateral corneal stromal rings; over time, that patient's eyes developed corneal endothelial decompensation and edema. I would examine the patient in the present case initially every 3 months and then every 6 months. Natalie Afshari, MD San Diego, California, USA
REFERENCES €hnliche Hornhautringe [Unusual corneal 1. Ascher KW. Ungewo ring]. Dtsch Ophthalmol Ges 1964; 65:44–46 2. Bron AJ. Peripheral ring opacity of the cornea. Br J Ophthalmol 1969; 53:270–273. Available at: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC1207311/pdf/brjopthal00328-0056.pdf. Accessed February 23, 2015 €rmige Hornhauttru € bung [Paracen3. Laqua H. Parazentrale ringfo tral ring-like corneal opacity]. Dtsch Ophthalmol Ges 1972; 71:583–585 €rmige 4. Bopp S, Laqua H. Kornealer Ascher-Ring - Eine ringfo € bung [Corneal Ascher ring - a ring-shaped stromale Hornhauttru stromal corneal opacity]. Klin Monatsbl Augenheilkd 1991; 198:201–204 ra JP, Eggink CA, Cruysberg JRM, Binder PS. 5. Melles GRJ, de Se Bilateral, anterior stromal ring opacity of the cornea. Br
J CATARACT REFRACT SURG - VOL 41, APRIL 2015
