In this case report, careful consideration of the differential careful consideration of the differential In this case report, answer. right diagnoses led to the ALICE WONG, DO; BY JONATHAN GO, DO; LITINSKY, MD WILLIAM B. TRATTLER, MD; AND STEVEN Figure 2. A comparison of corneal topographies in the patient’s left eye showed progressive inferior steepening with Figure 2. A comparison of corneal topographies in the patient’s left eye showed progressive inferior steepening with an increased I-S ratio over a period of 2 years, suggesting keratoconus. Figure 1. Corneal topography in 2011 demonstrated mild inferior steepening (black arrows), greater in the patient’s left steepening (black arrows), greater in the patient’s left Figure 1. Corneal topography in 2011 demonstrated mild inferior eye than in the right. | AUGUST 2020| AUGUST
topography, in particular,in topography, areful preoperative evaluationpreoperative areful issurgery cataract to prior improveddelivering to integral Cornealoutcomes. refractive Two years later, the patient returnedpatient the later, years Two A 62-year-old man presented forpresented man 62-year-old A Furthermore, repeat biometry showedbiometry repeat Furthermore, IOLpreferred the in reduction D 1.50 a todue D, 18.00 to 19.50 from power, theover power corneal in increase an surgerycataract first his since years 2 3). (Figure procedure of keratoconus—was measured atmeasured keratoconus—was of progressiveconfirmed This 2.67. wheneye left the in steepening inferior topography initial the to compared I-San when before, years 2 from measured.was 2) (Figure 2.24 of ratio postoperative course.postoperative incataract significant visually a with wastopography Corneal eye. left his time,that At 2). (Figure repeated ratio—a(I-S) inferior-superior the severitythe evaluate to used metric cataract evaluation in his right eye.right his in evaluation cataract topographycorneal Preoperative steepeninginferior mild identified Cataract 1). (Figure eye each in thein performed was surgery excellent an with eye right patient’s has become an invaluable tool intool invaluable an become has thisWith planning. preoperative followingthe present we mind, in report. case PRIOR TO CATARACT SURGERY? CATARACT TO PRIOR LATE-ONSET KERATOCONUS KERATOCONUS LATE-ONSET C CATARACT & REFRACTIVE SURGERY TODAY
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Figure 4. Slit-lamp examination of the cornea with fluorescein dye revealed an inferior epithelial ridge with negative staining (black arrow) suggestive of EBMD.
Figure 3. Biometry in the patient’s left eye showed a significant change in keratometry between 2011 and 2013 that The area of negative staining contributed to a 1.50 D reduction in IOL power. was consistent with a diagnosis of map-dot-fingerprint dystrophy At this juncture, the differential 1.50 D compared with IOL calculations or EBMD. Coincidentally, the area diagnoses of unilateral irregular from 2 years prior, in order to of EBMD observed on slit-lamp astigmatism were considered. These compensate for the increased corneal examination corresponded to differentials included ocular surface curvature and to attain the patient’s the worsening inferior steepening disease (OSD), epithelial basement desired postoperative outcome of demonstrated on corneal topography. membrane dystrophy (EBMD), slight myopia to emmetropia. The A diagnosis of EBMD masquerading development of a Salzmann nodule, lens power choice highlights the as a late-onset unilateral keratoconus corneal scarring, and keratoconus. importance of repeating biometry was made. Because the slit-lamp The patient’s history was negative if there exists any doubt as to the examination of the fellow eye was for most of the aforementioned accuracy of initial measurements or if entirely within normal limits, without diagnoses, and the anterior and there was an extended period between evidence of EBMD, we also considered posterior segment examinations were measurement and surgery, as was the that the findings in the patient’s left entirely normal, with the exception of case here. eye might simply represent localized decreased tear breakup time and trace It is of note that the choice of a epithelial hyperplasia. inferior punctate epithelial erosions, monofocal IOL was made given the consistent with OSD. irregular astigmatism in that eye and EBMD IDENTIFICATION AND The diagnosis of progressive the concern for the possibility of TREATMENT OPTIONS unilateral keratoconus was other underlying corneal pathology. EBMD is present in 6% to 18% of entertained, given the worsening Placing a multifocal IOL in such cases the population, predominantly in inferior steepening and significant increases the risk of decreased patient women older than 50 years of age.1 I-S ratio demonstrated on topography. satisfaction and reduced quality The typical presentation may include Although progressive keratoconus is of vision. blurry vision, monocular diplopia, uncommon in patients in their 60s, The patient’s BCVA immediately ghost images, and recurrent corneal keratoconus itself occurs with relative after cataract extraction was erosions. There have also been case frequency. Risk factors for progressive 20/25 OS. Two months later, the reports of EBMD masquerading as keratoconus include vigorous eye patient returned complaining of keratoconus. In a retrospective review rubbing and a significant family worsening blurry vision OS with BCVA of 1,000 cases of presumed corneal history; however, this patient attested of 20/30. Anterior and posterior ectasia (keratoconus, keratoglobus, to neither of these. segment exams appeared normal; pellucid marginal degeneration) After treatment for OSD, the however, following instillation referred for possible CXL, PRK, or patient successfully underwent of fluorescein dye, the patient intrastromal corneal ring segment cataract surgery in his left eye with demonstrated a small area of negative placement, 26 eyes of 20 patients had implantation of a monofocal IOL. The staining inferiorly (Figure 4), which had been falsely diagnosed with an ectatic choice of IOL power was decreased by not been evident preoperatively. disorder.2 Of the misdiagnosed cases,
AUGUST 2020 | CATARACT & REFRACTIVE SURGERY TODAY 21 - . . n J Refract Surg Am J Ophthalmol 2017;11:15-22. Clin Ophthalmol. . 2019;54(3):374-381. Executive Advisory Board CRST Can J Ophthalmol This case brings to light a uniquea light to brings case This Member, Larkin Community Hospital, Miami [email protected] Financial disclosure: None Clinical Affiliate Assistant Professor of Surgery, [email protected] Financial disclosure: None Director of Cornea, Center for Excellence in Eye [email protected] Financial disclosure: Consultant (Johnson & Larkin Community Hospital, Miami [email protected] Financial disclosure: None Johnson Vision) Charles E. Schmidt College of Medicine, Florida Charles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, Florida Care, Miami
2. Stein R, Salim G. False corneal ectasia in patients referred for corneal cross linking, topography-guided photorefractive keratectomy, and intrastromal corneal rings. therefore underwent a piggyback IOL IOL piggyback a underwent therefore distance his improve to procedure vision. present,may EBMD which in manner surgicaland medical the as well as presentation.this of implications demonstratescase the Specifically, thorougha of importance the usethe with examination slit-lamp and pre- repeated dye, fluorescein of ensureto testing postoperative keratometry,of assessments accurate inIOLs monofocal of implantation and cornealunderlying other which in eyes present. be might pathology 1. Weisenthal RW. External Disease and Cornea. In: Basic and Clinical Science Course. San Francisco: American Academy of Ophthalmology; 2017. n n n ALICE WONG, DO n n n 3. Reinstein DZ, Archer TJ, Gobbe M. Stability of LASIK in topographically suspect keratoconus confirmed non-keratoconic by Artemis VHF digital ultrasound epithelial thickness mapping: 1-year follow-up. 2009;25(7):569-577. 4. Itty S, Hamilton SS, Baratz KH, Diehl NN, Maguire LJ. Outcomes of epithelial debridement for anterior basement membrane dystrophy. 2007;144(2):217-221. 5. Lee WS, Lam CK, Manche EE. Phototherapeutic keratectomy for epithelial basement membrane dystrophy. JONATHAN GO, DO n n n STEVEN LITINSKY, MD, MBA n n n WILLIAM B. TRATTLER, MD n
In thatIn 4 After PTK, 58 eyes of eyes 58 PTK, After 5 After discussing the options, the options, the discussing After In a study by Itty et al, 74 eyes74 al, et Itty by study a In the examine to sought al et Lee the patient’s BCVA was 20/20, he 20/20, was BCVA patient’s the he and spectacle-free, be desiredto WHAT WE DID WHAT WE DID in PTK undergo to decided patient procedure, the Following eye. left his 5) (Figure normalized topography the The 20/20. to improved BCVA and returned eye that in keratometry original the in seen value the to at performed readings biometry presentation initial his of time the flattened The earlier. months 26 hyperopic a in resulted keratometry Although 180°. x +1.00 +1.50 to shift 51 patients who had not responded not had who patients 51 for management conservative to BCVA average an experienced EBMD 20/25. to 20/32 from improvement erosions, recurrent with eyes 29 In was improvement BCVA average the authors These 20/20. to 20/25 from and safe was PTK that concluded with term long the over effective other of those to comparable results modalities. surgical of EBMD. of of 55 patients underwent simpleunderwent patients 55 of resultingdebridement, mechanical improvementBCVA average in 20/33. to 20/44 from theof 18 in recurred EBMD study, persistentor infections No eyes. 74 butreported, were defects epithelial inpresent was haze subepithelial eyes.19 management the in PTK of efficacy | AUGUST 2020| AUGUST
3 Treatment of EBMD is notis EBMD of Treatment simple epithelial debridement, with orwith debridement, epithelial simple andpolishing, burr diamond without (PTK).keratectomy phototherapeutic necessary if a patient is asymptomatic;is patient a if necessary hypertoniclubricants, however, maylenses contact and solutions, conservativeinitial as trialed be treatmentsSurgical management. includeEBMD significant visually for with VHF ultrasound to confirmto ultrasound VHF with that eyes in pathology nonkeratoconic classified been have normally would cornealon based keratoconic as topography. included superficial punctate keratitispunctate superficial included 3),= (n astigmatism high 7), = (n 2),= (n keratopathy amiodarone scarscorneal 2), = (n warping corneal 2).= (n error measurement and 1), = (n foundal et Reinstein study, another In profilingthickness epithelial in utility CONSERVATIVE MANAGEMENT.” MANAGEMENT.” CONSERVATIVE inpathology underlying true the etiologiesOther EBMD. was eyes nine A PATIENT IS ASYMPTOMATIC; HOWEVER, ASYMPTOMATIC; HOWEVER, A PATIENT IS AND HYPERTONIC SOLUTIONS, LUBRICANTS, INITIAL MAY BE TRIALED AS CONTACT LENSES “TREATMENT OF EBMD IS NOT NECESSARY IF IF IS NOT NECESSARY OF EBMD “TREATMENT Figure 5. Topography of the patient’s left eye from Figure 5. Topography of the patient’s left eye from January 2014 after PTK in December 2013 demonstrates the bow-tie pattern of regular astigmatism. CATARACT & REFRACTIVE SURGERY TODAY
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