Quick viewing(Text Mode)

Corneal Ectasia Following Laser in Situ Keratomileusis for Myopia

Corneal Ectasia Following Laser in Situ Keratomileusis for Myopia

CASE REPORTS AND SMALL CASE SERIES

high resulting from poste- results might be related to the pre- Photorefractive Keratectomy rior lenticonus. Postsurgical refrac- existing corneal stromal abnormali- for Correction of tion was stable for 8 years, then a ties in their , which were not Regression rapid myopic regression of the epi- observed in our group. Thus, PRK keratophakic was observed can effectively be used to treat epi- photorefractive kera- the following year (Table). In- keratophakic regressed lenses in a se- tectomy (PRK) is widely used for the stead of removing the failed epikera- lected group of patients in whom correction of myopia, , tophakic lenses, we performed PRK both the epikeratograft and the sur- and hyperopia.1,2 It has also been on the . rounding are clear. This used for correction of astigmatism method eliminates the need for re- after penetrating keratoplasty.3 Results. Two and a half years after moval of the epikeratograft and ex- Epikeratophakia has been used PRK, the in all 4 eyes is posing the to the risks of suc- in the treatment of nontolerant con- stable and the epigrafts are clear. The cessive penetrating keratoplasty. tact keratoconous patients.4,5 Table presents the refraction and vi- The epigrafts were made from ma- sual acuity results for the eyes be- Hirsh Ami, MD chined corneal tissue that was found fore PRK and at 3 months, 1 year, Solberg Yoram, MD, PhD unsuitable for penetrating kerato- and 21⁄2 years after PRK. No haze has Cahana Michael, MD plasty. Long-term follow-up of pe- developed during this period. In all Avni Isaac, MD diatric patients who underwent epi- 4 eyes, a thin brown deposit ring was Tel-Hashomer, Israel keratoplasty for optical correction of formed on the edge of the treated op- and were corrected for em- tical zone. Corresponding author: Yoram Sol- metropia revealed that later in life berg, MD, PhD, The Goldschleger there is delayed myopic regression Comment. We describe herein our Institute, Sheba Medical Center, of the treated eye, which required successful experience with PRK for Tel-Hashomer 52621, Israel (e-mail: further correction.6,7 In their pa- regressed epikeratophakic lenses. Af- [email protected]). 8 1 tients, Colin et al failed to correct ter a follow-up of 2 ⁄2 years, the re- 1. Seiler T, McDonnell P. Excimer laser photore- this myopic regression with PRK. sults were stable and the epigrafts fractive keratectomy: major review. Surv Oph- thalmol. 1995;40:89-110. We describe our experience with were clear. The eyes were also stable 2. Wu HK, Remers PZ. Photorefractive keratectomy PRK for correction of delayed my- with regard to the best-corrected vi- for myopia. Ophthalmic Surg Lasers. 1996;27: opic regression of epikeratophakia sual acuity. 29-44. 8 3. Campos M, Hertzog L, Garbus J, Lee M, McDon- in 4 eyes. Colin et al reported on 5 eyes nell P. Photorefractive keratectomy for severe kera- with delayed refractive regression toplasty astigmatism. Am J Ophthalmol. 1992;114: Design. All procedures were per- following myopic epikeratoplasty 429-436. 4. Kaufman HE, Werblin TP. Epikeratophakia for formed in the cornea and refractive that were treated with PRK. Al- the treatment of keratoconous. Am J Ophthal- unit of The Goldschleger Eye though the eyes were successfully mol. 1982;93:342-347. 5. McDonald MB, Koling SB, Sabir A, Kaufman HE. Institute, Sheba Medical Center, Tel- corrected for , all of them On-lay lamellar keratoplasty for the treatment of Hashomer, Israel. developed substantial subepithe- keratoconous. Br J Ophthalmol. 1983;67:615-618. Four eyes of 2 twin sisters un- lial haze with poor , and 6. Arffa RC, Marelli TL. Long term follow-up of refractive and keratometric results of pediatric derwent epikeratoplasty at the ages the epikeratophakic lenses had to be epikeratophakia. Arch Ophthalmol. 1986;104: of 8 and 9 years old because of very removed. It is possible that their poor 668-670.

Refraction and Visual Acuity Results

Refraction, Degrees (Visual Acuity)

Patient 1 Patient 2

Time Right Eye Left Eye Right Eye Left Eye Before epikeratophakia −12.50 −11.00 −16.00 −15.00 5 y after epikeratophakia −3.5 −2.0 ϫ 30 (6/15p) −3.5 −1.0 ϫ 100 (6/20) −5.0 −1.0 ϫ 100 (6/15) −5.0 −3.0 ϫ 180 (6/12) Before PRK −17.00 (6/12) −11.00 (6/12p) −17.00 (6/12p) −16.00 (6/12p) 3 mo after PRK −0.75 −2.0 ϫ 15 (6/12+) −3.0 −3.5 ϫ 160 (6/15) −3.75 −2.0 ϫ 15 (6/12) −4.0 −3.0 ϫ 170 (6/15) 1 y after PRK −1.0 −2.0 ϫ 45 (6/12+) −2.0 −3.0 ϫ 135 (6/12) −4.0 −2.0 ϫ 25 (6/12p) −5.0 −3.0 ϫ 170 (6/12+) 21⁄2 y after PRK −3.00 (6/15+) −2.5 −2.5 ϫ 135 (6/12) −3.00 (6/12p) −4.0 −2.0 ϫ 180 (6/12p)

*PRK indicates photorefractive keratectomy; p, partial. Boldface items indicate best-corrected visual acuity.

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 281

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 7. Neumann AC, McCarty G, Sanders DV. Delayed regression of effect in myopic keratophakia ver- sus myopic for high myopia. Re- 101.5 3.3 101.5 3.3 fract Corneal Surg. 1989;5:161-166. 96.5 3.5 96.5 3.5 8. Colin J, Sangiuolo R, Malet F, Volant A. Photore- fractive keratectomy following undercorrected 91.5 3.7 91.5 3.7 myopic epikeratoplasties. J Fr Ophthalmol. 1992; 86.5 3.9 86.5 3.9 15:384-388. 81.5 4.1 81.5 4.1 76.5 4.4 76.5 4.4 71.5 4.7 71.5 4.7 Iatrogenic : 66.5 5.1 66.5 5.1 Corneal Ectasia Following 61.5 5.5 61.5 5.5 Laser In Situ Keratomileusis 56.5 6.0 56.5 6.0 50.5 6.7 50.5 6.7 for Myopia 49.0 6.9 49.0 6.9 47.5 7.1 47.5 7.1 Laser in situ keratomileusis (LASIK) 46.0 7.3 46.0 7.3 to correct myopia is performed by par- 44.5 7.6 44.5 7.6 tially resecting a prescribed thick- ness of stroma, removing corneal tis- 43.0 7.8 43.0 7.8 sue from the exposed stromal bed 41.5 8.1 41.5 8.1 using the excimer laser, and then re- 40.0 8.4 40.0 8.4 placing the resected stromal tissue. 38.5 8.8 38.5 8.8 This results in a substantial reduc- 37.0 9.1 37.0 9.1 tion of the biomechanically effective 35.5 9.5 35.5 9.5 stress-bearing thickness of cornea pro- 29.0 11.6 29.0 11.6 vided by the residual stromal bed. 24.0 14.1 24.0 14.1 There is concern that at some point, 19.0 17.8 19.0 17.8 the tensile strength of the cornea 14.0 24.1 14.0 24.1 might be reduced to the degree that 9.0 37.5 9.0 37.5 progressive ectasia ensues, thereby Diop mm Diop mm resulting in steepening of the cor- nea, irregular astigmatism, and pro- Figure 1. Top, Preoperative corneal topographic map of the right eye demonstrating focal inferonasal steepening. Bottom, Preoperative corneal topographic map of the left eye demonstrating a gressive myopia. This becomes a par- homogeneously regular central corneal contour. Drop indicates diopters. ticularly contentious issue when, in the absence of classic clinical evi- gery demonstrated mild inferona- Technologies, Waltham, Mass) in dence of keratoconus, inferior steep- sal steepening with a maximum the stromal beds of the right and left ening of the cornea seen on corneal power of 44.5 diopters (D), simu- eyes, respectively, estimated to leave topographic scan suggests the possi- lated keratometry readings of residual stromal beds of 260 µm OD bility of subclinical keratoconus. 43.0 ϫ 134/41.5 ϫ 44, and a mini- and 290 µm OS. On the first post- Herein, we report such a case of pro- mum keratometry reading of operative day, the patient’s uncor- gressive ectasia following LASIK. 41.3 ϫ 37 (Figure 1, top). Kera- rected visual acuity was 20/40 OD toconus screening by Rabinowitz/ and 20/60 OS, but at the next ex- Report of a Case. A 23-year-old His- McDonnell criteria suggested the amination 6 weeks later, it had de- panic man sought presence of keratoconus, while creased to 20/400 OU, and his cor- to correct myopia. Prior to surgery, screening by Klyce/Maeda criteria rected visual acuity was 20/400 OD he relied on spectacles to correct his identified a 15% similarity to kera- and 20/30 OS with a refraction of vision. He reported infrequent toconus. Corneal topographic scans –5.50 sphere OU. A bilateral simul- changes in his prescription and good of the left eye showed a homoge- taneous enhancement procedure was visual acuity in the years prior to con- neously regular central corneal performed under the flaps to fully sultation. His optometrist’s records contour with a central corneal correct the estimated residual myo- prior to surgery documented a re- power of approximately 41.28 D pia, and on the next examination 2 fraction of –12.75 – 2.25 ϫ 65 OD (Figure 1, bottom). Keratoconus weeks later, the patient’s uncor- and –8.50 – 1.50 ϫ 79 OS, yielding screening by both Rabinowitz/ rected visual acuity was 20/400 OD a visual acuity of 20/30 OU. Find- McDonnell and Klyce/Maeda crite- and 20/40 OS. Six weeks later, his ings from slitlamp examination ria failed to detect any similarity to best spectacle-corrected visual acu- (N.C.C.) revealed no characteristic keratoconus. ity was 20/25 OU with a refraction corneal findings of keratoconus in ei- The patient underwent bilat- of –1.50 – 3.00 ϫ 77 OD and –2.50 – ther eye, including Vogt’s striae or a eral simultaneous LASIK (N.C.C.). 0.75 ϫ 170 OS. . Ultrasound pachym- A 130-µm flap was created using a During the next 10 months, the etry measurements prior to surgery manual microkeratome, and abla- of the patient’s right were 555 µm OD and 560 µm OS. tions 165 µm and 140 µm in depth eyeprogressed,necessitatingfrequent Corneal topographic scans of were performed using an excimer la- spectacle changes, from –3.25 – the right eye performed prior to sur- ser (Summit Apex Plus; Summit 4.00 ϫ 66 to –11.00 – 4.75 ϫ 71.

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 282

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 ring) who were followed for more 0mm 7mm 55.000 than 3 years after undergoing sur- 54.000 face PRK that while most experi- 53.000 enced improvement in unaided vi- 52.000 sion, 1 suffered progression of 0mm 51.000 keratoconus. Conversely, Doyle et 50.000 al,3 arguing that topographic evi- 49.000 dence of inferior corneal steepen- 48.000 ing in the absence of clinical signs 47.000 consistent with keratoconus is of- 46.000 ten artifactitious, performed PRK in 45.000 4 such eyes and found the results 44.000 comparable to those expected in nor- 43.000 mal eyes with myopic astigmatism. 42.000

Temporal Therefore, the risk of a poor vi- 41.000 sual outcome and progressive ecta- 40.000 sia after performing surface PRK in 39.000 eyes with isolated topographic ab- 38.000 normalities suggestive of keratoco- 37.000 7mm nus is unclear. Although there is evi- 36.000 dence to suggest that performing 35.000 surface PRK might be successful in 34.000 Inferior select cases, it cannot be assumed 33.000 that this experience is applicable to 32.000 patients undergoing LASIK proce- 31.000 dures in which is per- 30.000 formed in a partial-thickness cor- neal bed, producing a relatively Figure 2. Corneal topographic map of the right eye demonstrating marked focal inferonasal steepening with an apical power greater than 50 diopters. thinner effective stress-bearing cor- nea. Our patient provides an instruc- His best spectacle-corrected visual sion of ectasia is likely to be has- tive case-control study of the risk of acuity decreased to 20/200 OD. Sub- tened by the removal of central cor- ectasia following LASIK in eyes with jectively, the left eye remained rela- neal tissue. Some authors have no observable signs of keratoconus tivelystable.Onexaminationapproxi- suggested that the risk is greatly ex- but in which corneal topographic mately 1 year following the initial pro- aggerated and have reported no evi- scan findings are suspect. While nei- cedures, his best spectacle-corrected dent acceleration of ectasia from 6 ther eye demonstrated frank kera- visual acuity was 20/100 OD and to 46 months after performing sur- toconus, progressive ectasia oc- 20/30 OS with a refraction of face photorefractive keratectomy curred exclusively in the eye with a –10.50 − 4.00 ϫ 67 OD and –3.75 (PRK) on patients with a clinical di- suspect . Previ- sphere OS. Findings from slitlamp ex- agnosis of keratoconus.1 While ex- ous reports are few. Seiler and amination revealed a hinged ker- perience is limited and follow-up Quurke4 have also described pro- atomileusis flap in both and brief, these results have prompted gressive corneal ectasia that oc- ectasia of the right cornea with a steep others to perform PRK in eyes that curred in a patient with an asym- central protrusion. Ultrasound pa- might be classified as forme fruste metric bow-tie pattern that they chymetry measurements were 386 keratoconus; ie, those eyes demon- interpreted as forme fruste kerato- µm OD and 485 µm OS. A computed strating topographic changes sug- conus. In our case, in addition to an corneal topographic map of the right gestive of keratoconus but without asymmetric bow-tie pattern, other eye showed profound inferonasal notable thinning, ectasia, or scar- features suggested subclinical kera- steepening in a keratoconus pattern ring. It might be argued that such toconus, including markedly asym- with an apical corneal power in ex- corneas might share biomechani- metric topographic scan findings be- cess of 50 D (Figure 2), while a map cal properties with those that dem- tween the 2 eyes and poor initial best of the left eye showed a well-centered onstrate true keratoconus, and so spectacle-corrected visual acuity. excimer laser ablation with marked demonstrate an increased ten- It might be postulated that the flattening. The patient’s right eye was dency toward progression of thin- risk of ectasia following LASIK might fitted with a rigid , with ning, myopia, and astigmatism af- be higher than that following PRK which he achieved satisfactory visual ter surgery. Indeed, Kremer et al2 because of the relatively thinner ef- acuity. have demonstrated in a study of 8 fective stress-bearing corneal stroma, eyes in 6 patients with mild kerato- but the residual stromal-bed thick- Comment. Keratoconus is gener- conus (compound myopic astigma- ness required to avoid progressive ally considered a contraindication for tism and topographic features con- corneal ectasia in either topographi- excimer laser refractive surgery, sistent with keratoconus without cally normal or abnormal eyes that since it is expected that the progres- notable ectasia, thinning, or scar- undergo LASIK is unknown. Based

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 283

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 on personal experience, Barraquer5 led to overestimation of the re- 10. Binder PS, Moore M, Lambert RW, Seagrist DM. Comparison of two microkeratome systems. has suggested a 300-µm thickness of sidual stromal-bed thickness. J Refract Surg. 1997;13:142-153. stress-bearing cornea. By compar- While these cases of corneal ec- 11. Chayet AS, Assil KK, Montes M, Espinosa- ing the biomechanical properties of tasia following LASIK have demon- Lagana M, Castellanos A, Tsioulias G. Regres- sion and its mechanisms after laser in situ ker- keratoconic corneas with normal strated fairly rapid progression, we atomileusis in moderate and high myopia. corneas, Andreassen et al6 have es- are concerned that others may de- . 1998;105:1194-1199. timated that for the normal cornea, velop more slowly. In a study of loss a residual stromal-bed thickness of of refractive effect in the first year less than 250 µm might produce a following LASIK, Chayet et al11 re- cornea with a tangential elastic port that regression seemed to be Treatment of Conjunctival modulus comparable to that of a caused by an increase in corneal keratoconic cornea. thickness rather than ectasia. How- Mucosa-Associated Lyle and Jin7 have reported a ever, longer-term studies are neces- Lymphoid Tissue Lymphoma high incidence (26%) of progres- sary. We strongly advocate that un- With Intralesional sive corneal ectasia that they termed til we are better able to identify of Interferon Alfa-2b iatrogenic keratoconus following hy- patients at risk for ectasia follow- peropic automated lamellar kerato- ing LASIK, and the variables defin- Conjunctival mucosa-associated plasty. The depth of the lamellar cut ing the biomechanical properties of lymphoid tissue (MALT) lympho- in this patient group ranged from the operated cornea are better de- mas are typically localized, low- 52% to 70%. However, their cases in- scribed, LASIK should not be per- grade tumors that differ histologi- cluded corneas that had undergone formed when findings from the ex- cally from other forms of primary prior and were amination or corneal topography extranodal non-Hodgkin lympho- therefore structurally weakened to suggest subclinical keratoconus. mas. Patients with conjunctival begin with. Hyperopic lamellar sur- MALT lymphoma have been cared gery frequently produces residual Stephen D. McLeod, MD for with radiation, chemotherapy, stromal beds less than 250-µm thick, San Francisco, Calif surgical excision, cryotherapy, and but when performed in cases of pri- Timothy A. Kisla, DO even observation alone. To our mary hyperopia rather than con- Nicholas C. Caro, MD knowledge, this is only the third secutive hyperopia following radial Timothy T. McMahon, OD report of conjunctival MALT lym- keratotomy, progressive ectasia is Chicago, Ill phoma treated with local injection relatively rare.8 In 3 cases of pro- of interferon alfa-2b (IFN-␣-2b)1,2 gressive corneal ectasia and kerato- and the first to appear in the North conus-like steepening developing in Corresponding author: Stephen D. American ophthalmic literature. topographically normal-appearing McLeod, MD, Department of Ophthal- eyes that underwent LASIK, Seiler mology, University of California– Report of a Case. A 21-year-old male et al9 estimated that the residual stro- San Francisco, 10 Kirkham St, K-301, student had a 2-month history of a mal-bed thickness was less than 200 San Francisco, CA 94143, (e-mail: painless, progressively enlarging µm in 1 patient and between 200 µm [email protected]). mass on his right eye. Findings from and 250 µm in the others. Based on anterior segment examination re- 1. Mortensen J, Carlsson K, Ohrstrom A. Ex- this experience and on the theoreti- cimer laser surgery for keratoconus. J Cata- vealed a large salmon-colored le- cal calculations of Andreassen et al,6 ract Refract Surg. 1998;24:893-898. sion involving the right nasal con- 2. Kremer I, Shochot Y, Kaplan A, Blumenthal M. these authors advocated a minimal Three year results of photoastigmatic refrac- junctiva, caruncle, and superior and residual stromal-bed thickness of tive keratectomy for mild and atypical kerato- inferior fornices (Figure 1). His un- 250 µm. conus. J Refract Surg. 1998;24:1581- corrected visual acuity was 20/20 OU 1588. In the case we report, the esti- 3. Doyle SJ, Hynes E, Naroo S, Shah S. PRK in and findings from the remainder mated residual stromal-bed thick- patients with a keratoconic topographic pic- of the ocular examination were un- ness prior to enhancement was 260 ture: the concept of a physiological “displaced remarkable. The patient was tenta- apex syndrome.” Br J Ophthalmol. 1996;80: µm OD. This suggests that for cer- 25-28. tively diagnosed as having a conjunc- tain corneas, such as those demon- 4. Seiler T, Quurke AW. Iatrogenic keratectasia tival lymphoid neoplasm, pending after LASIK in a case of forme fruste keratoco- strating features of keratoconus on nus. J Cataract Refract Surg. 1998;24:1007- biopsy and orbital imaging. He ac- topography, even 250 µm may not 1009. knowledged a 40-lb weight loss dur- be an adequate stromal-bed thick- 5. Barraquer JI. Querato Mileusis y Queratofa- ing a 2-year period and moderate fa- quia. Bogota, Columbia: Instituto Barraquer de ness to prevent progressive ectasia. America; 1980:342. tigue of recent onset. He denied Alternatively, the actual thickness of 6. Andreassen T, Simonsen TH, Oxlund H. Bio- having fever, chills, night sweats, the flap created by the microkera- mechanical properties of keratoconus and nor- other known masses, and risk fac- mal corneas. Exp Eye Res. 1980;31:435-441. tome might differ substantially from 7. Lyle WA, Jin GJ. Hyperopic automated lamel- tors associated with human immu- the expected, which might pro- lar keratoplasty: complications and visual re- nodeficiency virus infection. He was sults. Arch Ophthalmol. 1998;116:425-428. duce a thinner than anticipated 8. Burnstein Y, Robin AL. Pressure or progres- otherwise healthy with no family his- 10 bed. Also, reading error in pachym- sion? [letter]. Arch Ophthalmol. 1999;117:417- tory of hematological malignancy. etry measurements obtained, for ex- 419. Findings from physical examina- 9. Seiler T, Koufala K, Richter G. Iatrogenic kera- ample, from thicker paracentral re- tectasia after laser in situ keratomileusis. J Re- tion demonstrated a thin man with- gions of the cornea might also have fract Surg. 1998;14:312-317. out evidence of lymphadenopathy or

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 284

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 port no recurrence and a low rate of ocular morbidity in 115 patients re- ceiving 30 or 40 Gy during 3 or 4 weeks; 8 (7%) developed radiation- induced , and 5 (4%) ex- perienced disorders of ocular lubri- cation, despite shielding of the cornea and lens. Interferon alfa-2b is a glycoprotein produced by leu- kocytes possessing antiviral, immu- noregulatory, and antitumor activ- ity. The mechanisms responsible for Figure 1. Right conjunctival lymphoma before (left) and 3 months after initiating treatment with local its antitumor effects may include interferon alfa-2b (right). modulation of oncogenes and up- regulation of tumor cell surface an- tigens. As a single agent, its role seems limited to the treatment of low-grade lymphomas.4 Adverse ef- fects include fevers, chills, and my- algias. Fatigue, anorexia, and weight loss may occur with protracted use. Complete remission was achieved in all 5 previously described patients with conjunctival lymphoma treated with local injection of IFN-␣-2b.1,2 Adverse effects, if any, were not dis- cussed. Our patient experienced transient and nausea fol- lowing the first 3 injections. The in- jection itself caused subconjuncti- val hemorrhage twice. The absence of vision-threatening complica- tions and demonstrated efficacy, in this case, may warrant further con- sideration of the role of intrale- sional IFN-␣-2b injection for treat- Figure 2. Heavy infiltration of by small cleaved lymphocytes. The arrow indicates . ing conjunctival MALT lymphoma. Inset, higher magnification (immunoperoxidase). Long-term follow-up of this and other cases is needed.

hepatosplenomegaly. A conjuncti- tomographic scan of his head did not Kevin R. Lachapelle, MD val biopsy was performed. Sections show orbital extension or intracra- Rajinder Rathee, MD of the conjunctival biopsy specimen nial abnormality. A computed tomo- Vladimir Kratky, MD showed a diffuse, small, cleaved lym- graphic scan of the chest, abdomen, David F. Dexter, MD phocytic infiltrate with occasional and pelvis showed no abnormali- Kingston, Ontario cells percolating into the epithelial ties. We elected to treat this patient layer (Figure 2). Immunoperoxi- with an intralesional injection of Corresponding author: Kevin dase stains were positive for CD20 (a 1 000 000 U of IFN-␣-2b, adminis- Lachapelle, MD, Department of Oph- B-cell marker) and negative for CD3 tered 3 times weekly for a total of 12 thalmology, Queen’s University, King- (a T-cell marker). Findings from flow doses. One month later, at the con- ston, Ontario, Canada K7L 3N6. cytometry demonstrated ␬ light chain clusion of treatment, the mass was restriction (80%) and were positive considerably smaller. By 3 months, 1. Cellini M, Possati GL, Puddu P, Caramazza R. Interferon alpha in the therapy of conjunctival for CD19 (a B-cell marker) (86%). A despite no additional therapeutic in- lymphoma in an HIV+ patient. Eur J Ophthal- clonal rearrangement of the heavy tervention, the conjunctival mass was mol. 1996;6:475-477. chain, indicative of a clonal lympho- undetectable (Figure 1). There is no 2. Zinzani PL, Magagnoli M, Ascani S, et al. Non- gastrointestinal mucosa-associated lymphoid tis- proliferative cell population, was evidence of recurrence after 3 addi- sue (MALT) lymphomas: clinical and therapeu- demonstrated by polymerase chain tional months of follow-up, and the tic features of 24 localized patients. Ann Oncol. 1997;8:883-886. reaction. No evidence of circulating patient is otherwise healthy. 3. Bessell EM, Henk JM, Whitelocke AF, Wright JE. lymphoma cells was found in either Ocular morbidity after radiotherapy of orbital and the blood or bone marrow by mor- Comment. Lymphoid neoplasms of conjunctival lymphoma. Eye. 1987;1:90-96. 4. McLaughlin P. The role of interferon in the phology, flow cytometry, or poly- the conjunctiva are usually treated therapy of low grade lymphoma. Leuk Lym- merase chain reaction. A computed with radiotherapy. Bessell et al3 re- phoma. 1993;10(suppl):17-20.

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 285

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Spontaneous Resolution Nasal Temporal of Vitreomacular Traction 750 µm Documented by Optical Coherence Tomography

Optical coherence tomography 250 (OCT) is an imaging technology that µm can clearly define the vitreoretinal interface. The preoperative and post- operative features of vitreomacular 44 dB traction (VMT) have been described in a recent case report.1 In the pres- ent case, serial OCT images docu- mented the natural history of VMT A Log Reflection secondary to intermediate . This is the first report demonstrat- Nasal Temporal ing spontaneous resolution of VMT with OCT.

Report of a Case. The patient is a 1020 µm 60-year-old white woman with a 11⁄2- year history of stable, bilateral in- 180 µm 250 termediate uveitis treated with pred- µm nisolone acetate drops twice daily in both eyes. Five months before her initial visit to us, her visual acuity dropped to 20/60 OS from 20/25 OS. 48 dB Six weeks before referral to us, vi- sual acuity dropped further to 20/80 OS and a sub-Tenon triamcinolone B Log Reflection acetonide injection was adminis- tered in the left eye. The patient was Inferotemporal referred to our service for Superonasal evaluation of continuing decreased vision in her left eye. 210 µm Best-corrected Snellen visual acuity was 20/30 OD and 20/70 OS. Applanation tonometry revealed in- 250 traocular pressures of 19 mm Hg in µm the right eye and 27 mm Hg in the left, likely secondary to the cortico- steroid injection. Slitlamp examina- 45 dB tion revealed trace pigmented cells in the anterior chambers of both eyes. Dilated examination re- vealed a normal retina in the right C Log Reflection eye and multiple foveal cysts in the A, On the patient’s initial visit, the optical coherence tomographic image demonstrates a partially detached left eye. Fluoroscein angiography did posterior hyaloid located 750 µm anterior to the retina, which is characteristic of vitreomacular traction. There not reveal cystoid macular . are cystic intraretinal spaces produced by vitreous traction in the fovea. The foveal contour is irregular. B, One month later the posterior hyaloid is more elevated and located 1020 µm anterior to the retina but the area of The OCT image of the left eye re- vitreoretinal attachment is decreased. Central foveal thickness is decreased and measures 180 µm. C, Four vealed a partially detached poste- months after the initial visit, there is complete release of posterior hyaloid attachment to the retina with rior hyaloid exerting traction on residual intraretinal edema nasal to the fovea. Central foveal thickness is 210 µm. the fovea and secondary intrareti- nal thickening with large cystic spaces (Figure, A). Treatment was One month later the patient fovea although retinal thickening observation at this time with pars reported spontaneous improve- appeared to be decreased. The plana to be considered ment in vision. Best-corrected OCT image of the left eye revealed to relieve the traction if visual loss visual acuity improved to 20/40 that the posterior hyaloid had persisted in the setting of quiescent OS. Retinal examination revealed largely detached from the retina uveitis. persistent vitreous traction at the although a focal area of adhesion

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 286

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 remained temporal to the fovea reous anatomy may be considered procedure that allows visualization (Figure, B). prior to surgical intervention. of the spinal cord and epidural space Four months after the initial in patients with chronic back . visit the patient reported no visual Destry J. Sulkes complaints and visual acuity im- Michael S. Ip, MD Report of a Case. An 80-year-old proved to 20/30 OS. Retinal exami- Caroline R. Baumal, MD woman whose was nation revealed resolution of the cen- Helen K. Wu, MD significant only for chronic back pain tral retinal thickening and a taut Carmen A. Puliafito, MD and bilateral lower extremity weak- posterior hyaloid anterior to the fo- Boston, Mass ness unresponsive to medical man- vea. The OCT image confirmed com- agement underwent diagnostic epi- plete detachment of the posterior Corresponding author: Caroline R. duroscopy. The patient was brought hyaloid, diminished retinal thick- Baumal, MD, New England Eye Cen- to the operating room and placed in ening, and restoration of the nor- ter, 750 Washington St, Box 450, Bos- the prone position. Needle place- mal foveal contour (Figure, C). ton, MA 02111. ment in the epidural space was con- firmed with fluoroscopic guidance Comment. Vitreomacular traction 1. Munuera JM, Garcia-Layana A, Maldonado MJ, in 3 views. A guidewire was in- et al. Optical coherence tomography in success- is a result of detachment of the ful surgery of vitreomacular traction syn- serted and the scope apparatus was posterior vitreous with persistent drome. Arch Ophthalmol. 1998;116:1388-1389. introduced. Visualization of the epi- vitreomacular adhesions that pro- 2. Smiddy WE, Michels RG, Glaser BM, deBustros dural space was allowed via S. Vitrectomy for macular traction caused by in- duce retinal thickening and cystic complete vitreous separation. Arch Ophthal- instillation at a pressure not exceed- changes.2 In this case the cause ap- mol. 1988;106:624-628. ing 60 mm Hg for a period of less pears to have been intermediate 3. Schepens CL, Avila MP, Jalkh AE, Trempe CL. than 5 minutes. Epiduroscopy was Role of the vitreous in cystoid . uveitis because the pattern of adhe- Surv Ophthalmol. 1984;28(suppl):499-504. performed without un- sion and the patient’s course after 4. Puliafito CA, Hee MR, Schuman JS, Fujimoto JG. der intravenous sedation; the pa- Optical Coherence Tomography of Ocular Dis- vitreomacular separation are con- eases. Thorofare, NJ: Slack Inc; 1996. tient’s vital signs remained stable sistent with previous descriptions throughout the procedure. Imme- by Schepens et al.3 diately following the procedure, the Optical coherence tomogra- patient noted in phy is a diagnostic technique that both eyes. may be useful in distinguishing the Acute Bilateral Visual Loss Ocular examination at that time morphologic characteristics of a wide Associated With Retinal revealed a best-corrected visual acu- variety of retinal abnormalities.4 This Hemorrhages Following ity of 5/200 OD and 3/200 OS. In- case illustrates the utility of OCT in Epiduroscopy traocular pressures and were diagnosis and follow-up of patients within normal limits. Anterior seg- with VMT. Over a 4-month period, Acute bilateral visual loss associ- ment examination was notable for our patient’s condition spontane- ated with retinal hemorrhages fol- well-positioned intraocular lenses. ously improved, both subjectively lowing epidural steroid injection or Dilated funduscopic examination and on the OCT images, and this is gas myelography has been de- was remarkable for multiple, round, attributed to detachment of the pos- scribed.1-3 To our knowledge, we re- preretinal, retinal, and subretinal terior hyaloid from the macular sur- port the first case of acute bilateral hemorrhages involving the poste- face. As spontaneous resolution may visual loss associated with prereti- rior pole and midperiphery in both occur in some eyes with VMT, a pe- nal, retinal, and subretinal hemor- eyes (Figure 1). The disc margins riod of observation with sequential rhages occurring after epiduros- were sharp and the retinal vessels OCT evaluations to assess the vit- copy, a diagnostic and therapeutic were of normal caliber. Fluores-

Figure 1. Color fundus photographs of the right (left) and left (right) eyes demonstrate multiple retinal hemorrhages involving the posterior pole.

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 287

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Figure 2. Fluorescein angiograms of the right (left) and left (right) eyes reveal that the hemorrhages are located both deep to and superficial to the retinal circulation.

Figure 3. Color fundus photographs of the right (left) and left (right) eyes 6 months after epiduroscopy demonstrate resolution of the retinal hemorrhages.

cein angiography revealed areas of the epidural space with injection of ering the likelihood of this ocular blocked fluorescence correspond- saline for visualization of the spi- complication.4 Patient education and ing to the hemorrhages seen clini- nal cord and subarachnoid space. informed consent are paramount cally (Figure 2). Increased cerebrospinal fluid when recommending this proce- Six months later, best-cor- (CSF) pressure during epiduros- dure, especially for patients with rected visual acuity had improved to copy may lead to retinal hemor- bleeding tendencies or compro- 20/100 OD and 20/80 OS. The reti- rhages by 2 possible mechanisms. mised retinal circulation. nal hemorrhages had resolved spon- First, increased CSF pressure may be taneously in both eyes (Figure 3); transmitted directly through the op- Arezo Amirikia, MD vision was limited secondary to non- tic nerve sheaths to the retinal ve- Ingrid U. Scott, MD, MPH exudative age-related macular de- nous circulation.2 Alternatively, in- Timothy G. Murray, MD generation. creased CSF pressure may result in Miami, Fla decreased cerebral blood flow, which Lawrence S. Halperin, MD Comment. The occurrence of reti- in turn stimulates a reflex increase Ft Lauderdale, Fla nal hemorrhages in one or both eyes in ophthalmic artery pressure with immediately after the injection of resultant venous collapse and rup- Reprints: Timothy G. Murray, MD, oxygen into the subarachnoid space ture of capillaries.1 Bascom Palmer Eye Institute, 900 NW during myelography or following Acute visual loss associated 17th St, Miami, FL 33136. epidural injection of corticoste- with retinal hemorrhages is an un- 1. Oberman J, Cohn H, Grand G. Retinal compli- roids has been described previ- common but significant complica- cations of gas myelography. Arch Ophthalmol. 1-3 1979;97:1905-1906. ously. To our knowledge, this is tion of epiduroscopy. Experimen- 2. Kushner FH, Olson JC. Retinal hemorrhage as the first report of bilateral retinal tal evidence suggests that elevation a consequence of epidural steroid injection. Arch hemorrhages occurring after epidu- of CSF pressure may be modulated Ophthalmol. 1995;113:309-313. 3. Ling C, Atkinson PL, Munton CGF. Bilateral reti- roscopy, a procedure involving by decreasing the rate and volume nal haemorrhages following epidural injection. placement of a fiberoptic scope into of epidural injection, potentially low- Br J Ophthalmol. 1993;77:316-317.

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 288

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 4. Usubiaga JE, Usubiaga LE, Brea LM, Goyena R. Effect of saline injections on epidural and sub- arachnoid space pressure and relation to post- spinal anesthesia . Anesth Analg. 1967; 46:293-296.

Capillary Hemangioma of the Head and Juxtapapillary Retina

A 24-year-old man was noted to have decreased vision in the left eye on a routine . Fundus- copy revealed a mass overlying the optic nerve head and adjacent su- perotemporal retina in the left eye, as well as a large amount of exu- date in the macula. Fluorescein an- giography demonstrated early hy- Figure 1. Fundus photograph of the left eye showing a discrete, elevated, reddish lesion obscuring the perfluorescence of the lesion and late optic nerve head. Note the surrounding subretinal lipid exudate. pooling of the dye, outlining the reti- nal detachment. A diagnosis of cap- tumor frequently enlarges, and, over Based on the clinical appear- illary hemangioma of the optic nerve time, leads to progressive subreti- ance of the mass and the support- head and juxtapapillary retina was nal and intraretinal exudation and ing diagnostic studies, a diagnosis of made. The lesion was treated with . We present a capillary hemangioma of the optic argon laser photocoagulation. Sub- case of a capillary hemangioma of the nerve head and juxtapapillary retina sequent progression of the tumor led optic nerve head and juxtapapil- was made. The tumor was treated to a total retinal detachment and ru- lary retina in a 24-year-old man. with argon laser photocoagulation beosis iridis and resulted in enucle- on 2 occasions. Following this treat- ation. Histopathologic examina- Report of a Case. A 24-year-old ment, the patient was lost to fol- tion of the lesion revealed a mass white man was noted to have de- low-up for 8 months. Upon his re- composed of fine capillaries, lined creased visual acuity in the left eye turn to care, he had developed a total with endothelium, and filled with on a routine eye examination. He retinal detachment and rubeosis iri- red blood cells. Capillary heman- had not been aware of the visual loss. dis, and the eye was enucleated. Al- gioma of the optic nerve head and His medical history was unremark- though the patient had no family his- juxtapapillary retina is rare, and only able. Visual acuity was 20/20 OD and tory or symptoms suggesting von a few reports of its histopathologic counting fingers at 1.2 m (4 ft) in the Hippel-Lindau syndrome, he was ad- characteristics exist. The associa- left eye. On dilation of the , a vised to undergo testing to rule out tion of the tumor with von Hippel- discrete, elevated, reddish mass ob- cerebellar hemangioblastoma or other Lindau syndrome should be consid- scuring the optic nerve head was visceral tumors, but he failed to com- ered by ophthalmologists. noted in the left eye (Figure 1). The ply with that recommendation. Retinal capillary hemangioma elevated portion of the mass was ap- The gross section of the enucle- is a rare, benign vascular tumor of proximately 4.5 mm in diameter. ated eye revealed a funnel-shaped the retina. Two variants of the tu- Contact B-scan ultrasonography retinal detachment with xanthochro- mor are recognized: peripheral reti- demonstrated the mass measured 5.4 mic subretinal fluid containing re- nal capillary hemangioma and the mm thick and 11.5 mm ϫ 9.4 mm fractile particles and a white mass less frequent capillary heman- in diameter at the base. Dilated, tor- lesion (Figure 3). The mass, mea- gioma of the optic nerve head and/or tuous feeder vessels, arising from the suring 4.0 mm wide and 3.0 mm juxtapapillary retina. Either vari- , were seen on the surface thick, arose mainly from the optic ant can be associated with von Hip- of the lesion. Subretinal lipid exu- nerve head and involved the inner pel-Lindau syndrome. Retinal cap- date surrounded the lesion, and an retina superotemporally. Histologi- illary hemangioma usually occurs in exudative retinal detachment involv- cal examination demonstrated a to- the second or third decade of life ing the macula was present. No other tal retinal detachment with a mas- with equal frequency in both sexes hemangiomatous lesions were pres- sive amount of subretinal fluid with and no racial predilection. Heredi- ent in the left eye. Results of the fun- numerous cholesterol clefts, corre- tary and nonhereditary forms exist. dus examination of the right eye sponding to the refractile particles Initial symptoms include de- were normal. Fluorescein angiogra- seen on a gross section (Figure 4). creased visual acuity and phy demonstrated early hyperfluo- A lobulated mass composed of fine loss. The lesion may be asymptom- rescence of the vascular lesion and capillaries lined with normal endo- atic and discovered on a routine oph- late pooling of the dye, outlining the thelium and filled with red blood thalmic examination. An untreated retinal detachment (Figure 2). cells was seen. The vascular chan-

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 289

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Figure 2. Fluorescein angiogram demonstrating hyperfluorescence of the Figure 3. Photograph of gross section of the enucleated eye showing a lesion in the late venous stage. funnel-shaped retinal detachment with xanthochromic subretinal fluid and mass overlying the optic nerve head.

Figure 4. Low-magnification photomicrograph demonstrating retinal Figure 5. Photomicrograph showing fine capillaries lined with normal detachment with a massive amount of subretinal fluid with numerous endothelium and filled with red blood cells (hematolyxin-eosin, ϫ400). cholesterol clefts (hematoxylin-eosin, ϫ2). Note the elevated lesion overlying the optic nerve head.

nels were separated by vacuolated in- gray nodule in the peripheral retina by normal endothelium, separated by terstitial cells (Figure 5). In the an- supplied by enlarged, tortuous feeder plump, vacuolated interstitial cells, terior segment of the eye, rubeosis vessels. The well-circumscribed en- containing lipidlike material.3 Juxta- iridis and anterior displacement of dophytic capillary hemangioma aris- papillary capillary angioma has been the and lens were apparent. ing from the optic nerve head can shown histologically to have a cho- also be recognized clinically with- roidal as well as a retinal blood sup- Comment. Capillary hemangioma of out difficulty. When sessile or exo- ply.3 With time, the capillaries within the retina most likely represents a phytic, capillary hemangioma of the the hemangioma become incompe- vascular hamartoma and was first de- juxtapapillary retina and optic nerve tent, which presumably leads to pro- scribed by von Hippel in 1904. head may be misdiagnosed as uni- gressive subretinal and intraretinal Lindau reported the association be- lateral , papillitis, cho- exudation in the macula, often re- tween retinal capillary hemangio- roiditis, choroidal neovasculariza- sulting in a total retinal detachment. mas and central nervous system tion, or choroidal hemangioma.2 Spontaneous regression of a retinal tumors. The term “von Hippel- Stereoscopic fluorescein angi- hemangioma has been described, but Lindau syndrome” is applied to an ography is often helpful in estab- this is a rare occurrence.4 autosomal dominant disorder, re- lishing the diagnosis of retinal an- Eyes with untreated retinal an- cently linked to abnormalities in the gioma.2 giomas tend to have a poor progno- short arm of 3 (locus shows hyperfluorescence of the vas- sis because of the associated macu- 3p25),1 characterized by cerebellar cular lesion in the middle and later lar exudate and exudative retinal and spinal cord hemangioblasto- retinal stages, often followed by con- detachment. Early treatment of pe- mas, renal cell carcinoma, pheochro- tinuous leakage from the lesion in ripheral capillary hemangioma le- mocytoma, and retinal angiomas. the later stages of the study. Di- sions with argon and xenon photo- Peripheral capillary heman- lated feeder vessels can be readily vi- coagulation is advocated and tends gioma of the retina is often multi- sualized with angiography. to be more successful with tumor focal and bilateral and has the char- Histologically, the tumor is com- size of 1 disc diameter or smaller.5 acteristic appearance of a reddish or posed of numerous capillaries lined Multiple treatment sessions with

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 290

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 moderate-intensity, prolonged du- capillary angiomas of the juxtapapillary retina consistent with an ischemic optic and optic nerve head. Arch Ophthalmol. 1980; ration photocoagulation are recom- 98:1790-1797. neuropathy (Figure 2). The right mended. Other treatment modali- 3. Nicholson DH, Green WR, Kenyon KR. Light and eye had a normal optic nerve with a ties such as cryotherapy, diathermy, electron microscopic study of early lesions in an- 0.1 cup-disc ratio. Findings from the giomatosis retinae. Am J Ophthalmol. 1976;82: and proton beam irradiation have 193-204. remainder of the ophthalmic exami- been used as well. Photocoagula- 4. Whitson JT, Welch RB, Green WR. von Hippel nation were normal; results of a thor- Lindau disease: case report of a patient with spon- tion of juxtapapillary lesions is as- taneous regression of a retinal angioma. Retina. ough laboratory evaluation were un- 6 sociated with poor outcomes, since 1986;6:253-259. revealing. Ophthalmic examination destruction of nerve fiber layer in the 5. Gass JDM. Treatment of retinal vascular anoma- 9 months later showed pallor supe- lies. Trans Am Acad Ophthalmol Otolaryngol. macula during photocoagulation 1977;83:432-442. riorly in the left eye. may contribute to the visual loss. 6. Schindler RF, Sarin LK, MacDonald PR. He- Therefore, treatment of juxtapapil- mangiomas of the optic disc. Can J Ophthalmol. Comment. Transient changes in per- 1975;10:305-318. lary angiomas is generally not rec- ception of color hue or brightness ommended until macular exuda- have been reported with sildenafil.1 tion threatens the central vision.2 It Electrophysiological reductions on is also unlikely for photocoagula- Sildenafil (Viagra) electroretinograms have also been de- tion to penetrate a large endo- Associated Anterior scribed although all patients were phytic lesion, such as the one de- Ischemic clinically asymptomatic.2 The exact scribed in this case report. mechanism for these changes is un- Retinal capillary hemangioma Sildenafil citrate (Viagra) has been clear. However, the retina has a higher is often associated with von Hippel- shown to be effective for erectile dys- concentration of phosphodiesterase Lindau syndrome, but the actual in- function and is well tolerated.1 Head- type 6 and the visual phenomena may cidence is not precisely known and ache and flushing are common side be due to the ability of sildenafil to may have been underestimated in effects.1 We describe the novel find- weakly block this .2 previous studies. Schindler et al6 ing of ischemic optic neuropathy in The only neuro-ophthalmo- found a 24% incidence of von Hip- a patient concomitant with his in- logic complication reported thus far pel-Lindau syndrome or a positive gesting sildenafil. is a third nerve palsy.3 This patient family history in 55 patients with developed 36 hours after in- capillary angioma of the optic disc.6 Report of a Case. A 52-year-old gestion of 50 mg of sildenafil citrate. Thus, it is important to obtain a fam- healthy man with no known vascu- The patient smoked and took ami- ily history and refer the affected lopathic risk factors suffered from triptyline therapy. It was unknown individual for the appropriate sys- erectile dysfunction after transure- if the patient developed an erection temic studies, which include mag- thral resection for prostate cancer. or successfully completed inter- netic resonance imaging of the brain He took his first dose of 50 mg of course. Donahue and Taylor3 hypoth- and spinal cord to rule out heman- sildenafil citrate in the evening and, esized that sildenafil caused a hypo- gioblastomas, and abdominal com- within 1 hour, sweating and a se- tensive ischemic event to the third puted tomography to rule out renal vere generalized headache devel- nerve. This is supported by the abil- and other visceral cysts. Urinary oped. He saw blue “lightning bolts” ity of sildenafil to potentiate the hy- studies for pheochromocytoma have and reported blurry vision in both potensive effects of nitrates.4 Their also been advocated. The patient’s eyes. This lasted 30 minutes, but the patient, however, developed his relatives should undergo a dilated vision in the left eye remained symptoms after a significant period. fundus examination to exclude reti- blurred inferiorly. No erection oc- We believe this raises some doubt nal angiomas. curred and he did not have sex. He concerning the causation of the ocu- tried the medication the next night lomotor palsy by sildenafil therapy. Monika A. Malecha, MD with recurrence of the same symp- In , our patient developed Barrett G. Haik, MD toms. The blurry vision of the left eye symptoms within 1 hour of ingest- William R. Morris, MD did not change. He had Crohn dis- ing sildenafil. It is unlikely that our Memphis, Tenn ease and took methylphenidate hy- patient’s event was complicated by drochloride for attention deficit dis- order in the morning and at noon. This study was supported in part by 120 105 90 75 60 He was not a headache sufferer. 135 70 45 grants from the St Giles Foundation, 60 Ophthalmic examination 5 150 50 30 New York, NY, and by Research to 40 I-4-e days later showed corrected visual 30 Prevent Blindness, New York, NY. 165I-2-e 15 acuities of 20/20 OU and normal 20 Corresponding author: Barrett G. 10 color vision () in both 18090 80 70 60 50 40 30 20 10 10 20 30 40 50 60 70 80 90 0 Haik, MD, Department of Ophthalmol- 10 V-4-e eyes. There was no relative afferent 20 195 345 ogy, University of Tennessee, 956 Court 30 pupillary defect. Kinetic perimetry Ave, Suite D228, Memphis, TN 38163. 40 revealed an inferior altitudinal vi- 210 50 330 60 1. Seizinger BR, Rouleau GA, Ozelius LJ. von Hip- sual field depression in the left eye 225 70 315 240255 270 300 pel-Lindau disease maps to the region of chro- (Figure 1). Dilated funduscopy 285 mosome 3 associated with renal cell carci- noma. Nature. 1988;332:268-269. showed superior swelling of the left Figure 1. Inferior altitudinal visual field 2. Gass JDM, Braunstein R. Sessile and exophytic optic nerve head and a normal retina depression in the left eye.

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 291

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 chemic optic neuropathy in an ana- tomically susceptible optic nerve head, specifically hypotension. Al- though it cannot be explicitly proven that sildenafil therapy was the cause of this patient’s optic neuropathy, the close temporal relationship strongly suggests this.

Robert Egan, MD Howard Pomeranz, MD, PhD Boston, Mass

Corresponding author: Robert Egan, MD, Casey Eye Institute, 3375 SW Terwilliger Blvd, Portand, OR 97201 (e-mail: [email protected]).

1. Goldstein I, Lue TF, Padma-Nathan H, Rosen RC, Steers WD, Wicker PA. Oral sildenafil in the treat- ment of erectile dysfunction: Sildenafil Study Figure 2. Left optic nerve head with superior edema. Group [published correction appears in N Engl J Med. 1998;59]. N Engl J Med. 1998;338:1397- 1404. 2. Vobig MA, Klotz T, Staak M, Bartz-Schmidt KU, methylphenidate therapy because he be postulated as the cause of the prob- Engelmann U, Walter P. Retinal side-effects of was not taking the long-acting, sus- able hypotensive event to his optic sildenafil [letter]. Lancet. 1999;353:375. 3. Donahue SP, Taylor RJ. Pupil-sparing third nerve tained-release formulation and silde- nerve. palsy associated with sildenafil citrate (Viagra). nafil was ingested many hours later. Our patient had a very small Am J Ophthalmol. 1998;126:476-477. He also did not develop an erection cup-disc ratio. Sildenafil therapy may 4. FDA Talk Paper. Pfizer updates Viagra labeling. Food and Drug Administration Web site. Avail- or participate in any sexual activity have potentiated the same mecha- able at: http://www.fda.gov/bbs/topics/ANSWERS/ and thus no steal phenomenon can nisms that theoretically induce is- ANS00926.html. Accessed May 10, 1999.

ARCH OPHTHALMOL / VOL 118, FEB 2000 WWW.ARCHOPHTHALMOL.COM 292

©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021

Top View