AIMS/BACKGROUND: To describe a bilateral, mid peripheral, ring-shaped
          corneal opacity, not resembling any known corneal degeneration, dystrophy,
          or other disorder, and occurring without ocular or systemic disease.
          METHODS: Ophthalmic examination, haematological screening, and ultrasound
          biomicroscopy. RESULTS: A 25 year old man showed grey-white, granular
          opacities in both corneas, with an 8 mm diameter ring configuration, and a
          V-shaped distribution in the anterior stroma. The surrounding corneal
          stroma was clear, and the tear film, the epithelium and its basement
          membrane, Descemet's membrane, and the endothelium were normal. Evidence
          of systemic disease was not found. Family members did not show corneal
          abnormalities. CONCLUSION: A bilateral corneal ring opacity may occur in
          healthy, asymptomatic, young people. These corneal rings may result from
          depositions of unknown origin, or possibly a rare corneal dystrophy

Binder, P.S.

Cruysberg, J.R.M. (Johan)

Eggink, C.A. (Catharina)

Melles, G.R.J. (Gerrit)

Sera, J.P. de

Melles, G.R.J.

Eggink, C.A.

Cruysberg, J.R.M.

Radboud Repository

Bilateral, anterior stromal ring opacity of the cornea

NARCIS 

Erasmus University Digital Repository

 1998;82;522-525 Br. J. Ophthalmol.  Perry S Binder Gerrit R J Melles, Johan P de Séra, Cathrien A Eggink, Johan R M Cruysberg and   Bilateral, anterior stromal ring opacity of the cornea http://bjo.bmj.com/cgi/content/full/82/5/522Updated information and services can be found at:  These include: References http://bjo.bmj.com/cgi/content/full/82/5/522#otherarticles1 online articles that cite this article can be accessed at:   Rapid responses http://bjo.bmj.com/cgi/eletter-submit/82/5/522You can respond to this article at:  serviceEmail alertingtop right corner of the article Receive free email alerts when new articles cite this article - sign up in the box at the Notes    http://www.bmjjournals.com/cgi/reprintformTo order reprints of this article go to:  http://www.bmjjournals.com/subscriptions/ go to: British Journal of OphthalmologyTo subscribe to  on 11 December 2006 bjo.bmj.comDownloaded from Bilateral, anterior stromal ring opacity of thecorneaGerrit R J Melles, Johan P de Séra, Cathrien A Eggink, Johan R M Cruysberg,Perry S BinderAbstractAims/background—To describe a bilat-eral, mid peripheral, ring-shaped cornealopacity, not resembling any known cor-neal degeneration, dystrophy, or otherdisorder, and occurring without ocular orsystemic disease.Methods—Ophthalmic examination, hae-matological screening, and ultrasoundbiomicroscopy.Results—A 25 year old man showed grey-white, granular opacities in both corneas,with an 8 mm diameter ring configura-tion, and a V-shaped distribution in theanterior stroma. The surrounding cornealstroma was clear, and the tear film, theepithelium and its basement membrane,Descemet’s membrane, and the endothe-lium were normal. Evidence of systemicdisease was not found. Family membersdid not show corneal abnormalities.Conclusion—A bilateral corneal ring opac-ity may occur in healthy, asymptomatic,young people. These corneal rings mayresult from depositions of unknown origin,or possibly a rare corneal dystrophy.(Br J Ophthalmol 1998;82:522–525)In the past three decades, a ring-shaped opac-ity in the mid peripheral corneal stroma hasbeen described in a total of six patients.1–5 In1963, Ascher reported1 the occurrence of abilateral, grey-white ring in the mid peripheralcornea of a 39 year old man with recurrent,unilateral iritis of unknown cause. The ring wassharply demarcated, about 7 mm in diameter,and located in the superficial corneal stroma.Bron described2 a similar corneal ring opacityin both eyes of a 59 year old white male and a66 year old white female, who presented withunilateral marginal infiltrates. In both patients,the opacities were wedge-shaped with a densebase over Descemet’s membrane and a faintapex underneath Bowman’s layer, and locatedin the mid or far corneal periphery. Riegerdocumented3 a unilateral, annular opacity inthe right cornea of a 65 year old man. A perfectring was located in the mid periphery andextended posteriorly to about two thirds of cor-neal thickness. Bopp and Laqua described4 5 abilateral, ring-shaped opacity in a 65 year oldman and an 80 year old woman. The rings were4–5 mm and 7–8 mm in diameter, and extend-ed half way down the stroma.Depending on concomitant ocular findings,the occurrence of this corneal ring opacity hasbeen speculated to result from an immunologi-cal reaction after ocular inflammation, ordegenerative changes with advancing age. Inthe current report, we describe the presence ofa bilateral, ring-shaped, mid peripheral cornealopacification as an isolated finding in a young,healthy patient, who did not have a history ofocular inflammation.Patient and methodsA 25 year old man with a bilateral, ring-shapedcorneal opacity of unknown origin underwentcomplete ophthalmic examination, as well ashaematological screening for known metabolicdisorders, and ultrasound biomicroscopy at 50MHz (Ultrasound Biomicroscope, Humphrey)of his corneas.ResultsIn 1981, at the age of 11, a Mediterranean manwas first examined by us, after a bullet from asmall bore rifle had hit his right eye. Ahyphaema and a vitreous haemorrhage haddeveloped, which cleared in 3 weeks. At thattime, funduscopy revealed the presence of fiveattached retinal breaks in the temporal quad-rant, which were treated with argon laser pho-tocoagulation.From 1981 to 1993, he was seen at yearlyintervals by his own ophthalmologist. Duringthis period, corneal abnormalities were notobserved. In 1993, he was examined by us forcomplaints of floaters, which were attributed toa posterior vitreous detachment in the righteye. In 1995, he was referred to us becauseboth corneas showed a faint, grey-white,granular deposition in a perfect continuouscircle, with a diameter of approximately 8.0mm and a width of about 1/3 mm (Fig 1A-D).From anterior to posterior, the opacity had aV-shaped configuration; the lower end of the Vextended into the mid stroma, sharply demar-cated from the surrounding, clear cornealstroma (Fig 1E). The tear film, the epithelium,and the endothelial layer were unremarkable;the ocular surface did not stain with fluores-cein, the epithelium had normal thickness,basement membrane abnormalities were notseen, and specular microscopy showed anormal endothelial layer. The stroma did notshow central thinning or other abnormalities.Both eyes were quiet. The other media wereclear and, except for the areas treated with laserphotocoagulation, the fundi were normal.The uncorrected visual acuity was 1.0 inboth eyes. Regular, smooth keratometry read-ings were 44.0 · 43.5 D in the right eye, and43.0 · 43.0 D in the left eye. Corneal sensitiv-ity measured with a cotton tip was normal.Gonioscopy showed no abnormalities of theBr J Ophthalmol 1998;82:522–525522Rotterdam EyeHospital, Rotterdam,NetherlandsG R J MellesSlingeland Hospital,Doetinchem,NetherlandsJ P de SéraInstitute ofOphthalmology,University ofNijmegen, NetherlandsC A EgginkJ R M CruysbergNational VisionResearch Institute,San Diego, USAP S BinderCorrespondence to:Gerrit R J Melles, MD, PhD,The Rotterdam EyeHospital, Schiedamse vest180, 3011 BH Rotterdam,Netherlands.Accepted for publication19 November 1997 on 11 December 2006 bjo.bmj.comDownloaded from anterior chamber angle. Our patient deniedany previous ocular, orbital, or eyelid surgery,and, in particular, any type of excimer laserphotoablation treatment. Except for the oculartrauma 14 years before, his medical history wasunremarkable. He denied any symptoms ofphotophobia, pain, cold sores, or fever blisters.He had a profession in the paint producingindustry, and worked with liquid trichlorethyl-ene. He denied oral intake of vitamin D,antihyperglycaemic, antiprotozoa (quinacine),antiarrhythmic, antirheumatic (gold, indo-methacin), antiviral medication, iron, migraine(ergotamine), silver containing antibacterial,antipsychotic, antiangina (amiodarone), orantimalarial (chloroquine) drugs.The haematological examination, serumcopper and lipoprotein spectrum (VLDL,LDL, HDL, triglycerides, cholesterol, apolipo-protein I and II) were normal. Serological testsfor syphilis (VDRL and TPHA), Lyme disease,and sarcoid (angiotensin converting enzymeand lysozyme) were negative. Urine analysis formucopolysaccharides, amino acids, and or-ganic acids (homogentisic acid) in the urineshowed no abnormalities. With ultrasoundbiomicroscopy the corneal deposition couldnot be detected.The patient’s father was found to have a mildarcus senilis in both corneas, and his motherhad bilateral crocodile shagreen. The 4 yearold son of our patient showed no cornealabnormalities, and his 1 year old daughter didnot permit ocular examination. The patient’sbrother was not available for examination.DiscussionA mid peripheral ring opacity has beendescribed in six elderly patients (Table 1).1–5Three of these patients presented with unilat-eral marginal infiltrate or iritis.1 2 It wassuggested that the rings resulted from anon-specific degeneration at an advanced age,or from a bilateral immunological reactionassociated with unilateral ocular inflammation(Wessely ring). We observed a bilateral, midperipheral ring opacity in a healthy patient as acoincidental finding during routine ocularexamination. Because our patient was relativelyyoung and had never suVered from ocularinflammation, the aetiology of the ring-shapedcorneal opacity is currently undetermined.In the cornea, six patterns of pathologicalresponses thought to be attributed to cornealopacity have been described: immune re-sponse, deposition, proliferation, defects, fibro-sis with vascularisation, and oedema withcysts.6 In our patient, there was no evidence ofan addition or a loss of corneal tissue, oedema,or vascularisation so the corneal ring opacitymay be classified best as an “immune re-sponse” or a “deposition”.The most common immunological reactionthat leaves a stromal, ring-shaped opacity is theWessely ring after herpetic keratitis.7 Such astromal scar most often follows (recurrentattacks of) stromal oedema associated withherpetic keratitis. Autoimmune disorders mayoften be limited to the peripheral cornea.7 8Humoral and cellular factors from the limbalarea may react to agents present within thecentral cornea, which may result in accumula-tion of immune complexes and associatedinflammation in the peripheral cornea. How-ever, since our patient did not have a history ofkeratouveitis or any evidence of systemicvasculitis, an interstitial or autoimmune kerati-tis may be an unlikely explanation for thedevelopment of the observed corneal ringopacity.Figure 1 Slit lamp photographs of the right (A and C) and left eye (B and D). Bothcorneas show a granular deposition with a configuration of a perfect ring, 8.0 mm indiameter—that is, sharply demarcated from the clear surrounding cornea. At highermagnification (E) the opacity appears to consist of a granular deposition with a V-shapeddistribution in the anterior stroma.Bilateral, anterior stromal ring opacity of the cornea 523 on 11 December 2006 bjo.bmj.comDownloaded from Corneal depositions have been described tooriginate from three sources—topical or sys-temic drugs, ocular or systemic metabolic disor-ders, and corneal dystrophies or degenerations.6Corneal opacities induced by topical or systemicdrugs usually involve both the central andperipheral cornea.9 To our knowledge, accumu-lation of a drug in a ring-shaped fashion in theanterior stroma has not been described. In hisprofession, our patient was exposed for aprolonged period of time to vaporised trichlor-ethylene, but corneal opacification with systemicuse of this drug has not been described.9A wide variety of ocular and systemicdisorders have been associated with depositionsthroughout the cornea.7 10 Ring-shaped opaci-ties limited to the peripheral cornea includesubepithelial pigment globules in alkaptonuria,11gold deposition within the peripheral cornealepithelium in ocular chrysiasis,10 11 and copperor silver deposition at the level of Descemet’smembrane in, respectively, Wilson’s disease(Kayser–Fleischer ring) and ocular argyrosis,10 11epithelial crystals in Waldenström’smacroglobulinaemia,12 and peripheral cornealdepositions in Scheie syndrome.13 A peripheralcorneal arcus may result from a deficientlipoprotein metabolism with fat deposition overthe entire corneal thickness, and is characterisedby a distinct peripheral and an indistinct centralborder.14 In our patient, the ring opacity couldbe distinguished from a typical arcus by its moregranular appearance, involvement of only theanterior stroma, the sharp borders, and themore central location of the ring—that is, a wideband of clear cornea between the opacity andlimbus. The serum copper and lipoprotein spec-trum were normal, incompletely degradedmucopolysaccharides and homogentisic acidwere absent in the urine, and our patients neverused gold or silver containing drugs. Therefore,the ring opacity could not be related to knownmetabolic disorders.Corneal dystrophies associated with anteriorstromal opacities usually show involvement ofthe central cornea.10 15 16 In our patient, thegranular deposition was finer and more regu-larly distributed than in a granular or centralcrystalline corneal dystrophy, but more distinctthan in a central cloudy15 16 or macular cornealdystrophy.15 16 The overall appearance of thering opacity did not fit in with any of the knowncorneal dystrophies.15–17Ring-shaped degenerations of the corneawith advancing age include mosaic crocodileshagreen, Vogt’s white limbal girdle, single ordouble anterior embryotoxon (arcus senilis),and posterior embryotoxon (prominent line ofSchwalbe).10 16 Furrow degeneration in theabsence of an arcus senilis, may present as a cir-cumferential, peripheral corneal opacificationwith an irregular outline.16 Early spheroiddegeneration may present with gold coloured,peripheral deposits.7 16 In early Terrien’s mar-ginal degeneration, peripheral opacificationwith vascularisation may be found, beforecorneal ectasia develops.7 16 Iron depositionwith a Hudson Stähli or Stocker’s line inkeratoconus, may have a semicircular configu-ration and a colourless appearance.10 16 Semicir-cular rings in the inferior corneal halves havealso been found in aplasia cutis congenitacircumscripta.18 Central corneal ring opacitieswith a reddish brown appearance have beendescribed in hereditary spherocytosis.19 ACoats’ white ring may be observed after localcorneal tissue damage by an iron foreign body.16Again, the corneal ring in our patient could bediVerentiated from all of the peripheral cornealdegenerations above, by the perfect round con-figuration, the fine, colourless granular appear-ance, the anterior stromal distribution, and themore central location of the opacity.From our study we conclude that an asymp-tomatic, mid peripheral corneal ring opacityhas been observed in seven healthy, adultpatients (Table 1). Except for two cases, thecorneal ring manifested bilaterally, as a sharplyoutlined, grey-white opacity in the anteriorstroma. The opacity resulted from a depositionof an unknown material, that showed anacoustic reflectivity similar to corneal tissuewith ultrabiomicroscopy, so that deposition ofhighly reflective substances like, for example,calcium or lipids is unlikely. The distribution ofthe opacities in a perfect ring—that is, at anequal distance from the corneal centre, maysuggest that the material was deposited afterdiVusion over a gradient between the periph-eral and central cornea or vice versa. Theoccurrence of the corneal ring at a young adultage may suggest that the deposition resultedTable 1 Patient characteristics in reported cases with a mid peripheral corneal ring opacityReported casesPatient ageat time ofdiagnosis,and sexUni- orbilateralDiameter(and width)of rings(mm)DistributionopacitiesAppearanceopacitiesProgressionopacities atdiagnosisOcularpathologyOcularhistoryMedicalhistoryBlood pictureand lipoproteinspectrumFamilyexamination1 Ascher1 39 M bilateral 7–8 (1/3) anteriorstromagrey-white,non-granularstable for10 yearsiritis recurrent iritis unremarkable normal two sonsnormalBron22 Case 1 59 M bilateral 10 posteriorwedge stromawhite NR unilateral,marginal,infiltrateunremarkable NR normal relatives notavailable3 Case 2 66 F bilateral 8 posteriorwedge stromawhite,granularNR unilateral,marginal,infiltrateNR angina andcoronarythrombosisNR NR4 Rieger3 65 M unilateral 7–8 anterior2/3 stromagrey-white,yellowNR none unremarkable unremarkable elevated serumcholesterolNRBopp/Laqua4,55 Case 1 80 F bilateral 7–8 anterior1/2 stromagrey, granular stable for9 yearscataract NR NR normal relativesnormal6 Case 2 65 M bilateral 4–5 (1/3) anterior2/3 stromagrey-white stable for6 yearscataract cataract NR normal siblingsnormal7 Present study 25 M bilateral 8 (1/3) anterior1/2 stromawhite, granular stable for2 yearsnone unilateralocular traumaunremarkable normal relativesnormal524 Melles, de Séra, Eggink, et al on 11 December 2006 bjo.bmj.comDownloaded from from a corneal dystrophy rather than a degen-eration, although no evidence of an inheriteddisorder was found in our patient or in any ofthe cases described (Table 1).1–5 Like others, wefound that the opacity showed no detectableprogression, which may suggest that the ringresulted from exposition to an exogenous orendogenous factor for a limited period oftime—for example, a drug to which an idiosyn-cratic reaction developed.We thank A L Aandekerk for the slit lamp photography, andA M Verbeek for performing ultrasound biomicroscopy.1 Ascher KW. Ungewöhnliche Hornhautringe. Ber Dtsch Oph-thal Ges 1963;65:44–6.2 Bron AJ. Peripheral ring opacity of the cornea. Br JOphthalmol 1969;53:270–3.3 Rieger G. Primäre, einseitige, ringförmige Trübung derHornhaut. Fortschr Ophthalmol 1987;84:242–4.4 Laqua H. Parazentrale ringförmige Hornhauttrübung. BerDtsch Ophthal Ges 1971;71:583–5.5 Bopp S, Laqua H. Kornealer Ascher-Ring. Klin MonatsblAugenheilkd 1991;198:201–4.6 Waring GO, Rodrigues MM. Patterns of pathologicresponse in the cornea. Surv Ophthalmol 1987;31:262–6.7 Robin JB, Schanzlin DJ, Verity SM, et al. Peripheral cornealdisorders. Surv Ophthalmol 1986;31:1–36.8 Mondino DJ. Inflammatory diseases of the peripheralcornea. Ophthalmology 1988;95:463–72.9 Fraunfelder FT, Meyer SM. Ocular toxicology. In: TasmanW, Jaeger EA, eds. Duane’s clinical ophthalmology. Volume 5,Chapter 37, revised edition. Philadelphia: Lippincott-Raven, 1990:1–21.10 Vogt A. Die normale Hornhaut, ihre senilen und patholo-gischen Veränderungen. In:Lehrbuch und Atlas der Spaltlam-penmikroskopie des lebenden Auges. Berlin: Springer, 1930:47–269.11 Smith RE, Lee JS. The cornea in systemic disease. In:Tasman W, Jaeger EA, eds. Duane’s clinical ophthalmology.Volume 4, Chapter 15, revised edition. Philadelphia:Lippincott-Raven, 1990:1–44.12 Orellana J, Friedman AH. Dysproteinemias. In: Gold DH,Weingeist TA, eds. The eye in systemic disease. Philadelphia:Lippincott, 1990:134–7.13 Bajart AM, Pavilack MA. Corneal manifestations ofmetabolic disease. In: Albert DM, Jackobiec FA, eds. Prin-ciples and practice of ophthalmology. Philadelphia: Saunders,1994:294–319.14 Barchiesi BJ, Eckel RH, Ellis PP. The cornea and disordersof lipid metabolism. Surv Ophthalmol 1991;36:1–22.15 Waring GO, Rodrigues MM, Laibson PR. Corneal dystro-phies. I Dystrophies of the epithelium, Bowman’s layer andstroma. Surv Ophthalmol 1978;23:71–122.16 Kenyon KR, Hersh PS, Starck T, et al. Corneal dysgeneses,dystrophies and degenerations. In: Tasman W, Jaeger EA,eds. Duane’s clinical ophthalmology. Volume 4, Chapter 16,revised edition. Philadelphia: Lippincott-Raven, 1992:1–53.17 Bron AJ, Williams HP, Carruthers ME. Hereditary crystal-line stromal dystrophy of Schnyder. I Clinical features of afamily with hyperlipoporoteinaemia. Br J Ophthalmol1972;56:383–99.18 Vogel M, Kiessling W. Weisse Hornhautringe in Verbindungmit aplasia cutis congenita circumscripta des behaartenKopfes. Ber Dtsch Ophthal Ges 1964;66:361–4.19 Dalgleish R. Ring-like corneal deposits in a case of congeni-tal spherocytosis. Br J Ophthalmol 1965;49:40–2.Bilateral, anterior stromal ring opacity of the cornea 525 on 11 December 2006 bjo.bmj.comDownloaded from 

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